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    Severity of Functional Mitral Regurgitation on Admission for Acute Decompensated Heart Failure Predicts Long-Term Risk of Rehospitalization and Death. Journal of the American Heart Association Background Functional mitral regurgitation (FMR) has emerged as a therapeutic target in patients with chronic heart failure and left ventricular systolic dysfunction. The significance of FMR in acute decompensated heart failure remains obscure. We systematically investigated the prevalence and clinical significance of FMR on admission in patients admitted with acute decompensated heart failure and left ventricular systolic dysfunction. Methods and Results The study was a single-center, retrospective review of patients admitted with acute decompensated heart failure and left ventricular systolic dysfunction between 2012 and 2017. Patients were divided into 3 groups of FMR: none/mild, moderate, and moderate-to-severe/severe FMR. The primary outcome was 1-year post-discharge all-cause mortality. We also compared these groups for 6-month heart failure hospitalization rates. Of 2303 patients, 39% (896) were women. Median left ventricular ejection fraction was 25%. Four hundred and fifty-three (20%) patients had moderate-to-severe/severe FMR, which was independently associated with 1-year all-cause mortality. Moderate or worse FMR was found in 1210 (53%) patients and was independently associated with 6-month heart failure hospitalization. Female sex was independently associated with higher severity of FMR. Conclusions More than half of patients hospitalized with acute decompensated heart failure and left ventricular systolic dysfunction had at least moderate FMR, which was associated with increased readmission rates and mortality. Intensified post-discharge follow-up should be undertaken to eliminate FMR amenable to pharmacological therapy and enable timely and appropriate intervention for persistent FMR. Further studies are needed to examine sex-related disparities in FMR. 10.1161/JAHA.121.022908
    Left atrial global function in chronic heart failure patients with functional mitral regurgitation after MitraClip. Öztürk Can,Fasell Tamana,Sinning Jan-Malte,Werner Nikos,Nickenig Georg,Hammerstingl Christoph,Schueler Robert Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions BACKGROUND:Left atrial (LA) volumes and function are believed to improve following interventional reduction of mitral regurgitation (MR) with MitraClip. However, exact LA alterations after MitraClip in patients with functional MR and functional mitral regurgitation (FMR) are unknown. OBJECTIVES:We aimed to evaluate the effect of MitraClip on LA volumes and global function in patients with FMR and its importance for patients' prognosis. METHODS:All patients underwent three-dimensionally transthoracic echocardiography with an offline evaluation of LA geometry and strain analysis at baseline and follow-up (FU). FU examinations were planned for 6 and 12 months after MitraClip. RESULTS:We prospectively included 50 consecutive surgical high-risk (logistic EuroSCORE: 17.2 ± 13.9%) patients (77 ± 9 years, 22% female) with symptomatic moderate-to-severe to severe functional MR without atrial fibrillation. Echocardiographic evaluation showed that the E/E' ratio was significantly higher at FU (15.6 ± 7.3, 24.1 ± 13.2, p = .05) without relevant changes in systolic left ventricle (LV) function (p = .5). LA volumes (end-diastolic volume [LA-EDV] and end-systolic volume [LA-ESV]) (LA-EDV: 83.1 ± 39.5 ml, 115.1 ± 55.3 ml, p = .012; LA-ESV: 58.4 ± 33.4 ml, 80.1 ± 43.9 ml, p = .031), muscular mass (105.1 ± 49.3 g, 145.4 ± 70.6 g, p = .013), as well as LA stroke volume (24.6 ± 12.5 ml, 34.9 ± 19.1 ml, p = .016) significantly increased after the procedure. LA ejection fraction (LA-EF: 31.7 ± 12.8%, 31.1 ± 12.3%, p = .8) and atrial global strain (aGS: -10.8 ± 5.4%, -9.7 ± 4.45%, p = .4) showed no significant changes at FU. Despite no relevant changes during FU, the baseline aGS was found to be the strongest predictor for mortality and adverse interventional outcome. CONCLUSION:MitraClip increases atrial stroke volume, atrial volumes, and muscular mass in patients with FMR. We found that the baseline aGS the strongest predictor for mortality, rehospitalization, and higher residual MR at FU. 10.1002/ccd.28775
    Global longitudinal strain is a hallmark of cardiac damage in mitral regurgitation: the Italian arm of the European Registry of mitral regurgitation (EuMiClip). Santoro Ciro,Galderisi Maurizio,Esposito Roberta,Buonauro Agostino,Monteagudo Juan Manuel,Sorrentino Regina,Lembo Maria,Fernandez-Golfin Covadonga,Trimarco Bruno,Zamorano Josè Luis Cardiovascular ultrasound BACKGROUND:The search for reliable cardiac functional parameters is crucial in patients with mitral regurgitation (MR). In the Italian arm of the European Registry of MR, we compared the ability of global longitudinal strain (GLS) and left ventricular (LV) ejection fraction (LVEF) to detect cardiac damage in MR. METHODS:Five hundred four consecutive patients with MR underwent a complete echo-Doppler exam. A total of 431, 53 and 20 patients had degenerative, secondary and mixed MR, respectively. The main echocardiographic parameters, including LV and left atrial (LA) size measurements, pulmonary artery systolic pressure (PASP) and GLS were compared between patients with mild MR (n = 392) vs. moderate to severe MR (n = 112). RESULTS:LVEF and GLS were related one another in the pooled population, and separately in patients with mild and moderate/severe MR (all p < 0.0001). However, a certain number of patients were above the upper or below the lower limits of the 95% confidence interval (CI) of the normal relation in the pooled population and in patients with mild MR. Only 2 patients were below the 95% CI in moderate to severe MR. After adjusting for confounders by separate multivariate models, LVEF and GLS were independently associated with LV and left atrial size in the pooled population and in mild and moderate/severe MR. GLS, but not LVEF, was also independently associated with PASP in patients with mild and moderate to severe MR. CONCLUSIONS:Both LVEF and GLS are independently associated with LV and LA size, but only GLS is related to pulmonary arterial pressure. GLS is a powerful hallmark of cardiac damage in MR. 10.1186/s12947-019-0178-7
    Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Luedde Mark,Ehlermann Philipp,Weichenhan Dieter,Will Rainer,Zeller Raphael,Rupp Stefan,Müller Andreas,Steen Henning,Ivandic Boris T,Ulmer Herbert E,Kern Michael,Katus Hugo A,Frey Norbert Cardiovascular research AIMS:Left ventricular non-compaction (LVNC) is caused by mutations in multiple genes. It is still unclear whether LVNC is the primary determinant of cardiomyopathy or rather a secondary phenomenon with intrinsic cardiomyocyte dysfunction being the actual cause of the disease. Here, we describe a family with LVNC due to a novel missense mutation, pE96K, in the cardiac troponin T gene (TNNT2). METHODS AND RESULTS:The novel mutation was identified in the index patient and all affected relatives, but not in 430 healthy control individuals. Mutations in known LVNC-associated genes were excluded. To investigate the pathophysiological implications of the mutation, we generated transgenic mice expressing human wild-type cTNT (hcTNT) or a human troponin T harbouring the pE96K mutation (mut cTNT). Animals were characterized by echocardiography, histology, and gene expression analysis. Mut cTNT mice displayed an impaired left ventricular function and induction of marker genes of heart failure. Remarkably, left ventricular non-compaction was not observed. CONCLUSION:Familial co-segregation and the cardiomyopathy phenotype of mut cTNT mice strongly support a causal relationship of the pE96K mutation and disease in our index patient. In addition, our data suggest that a non-compaction phenotype is not required for the development of cardiomyopathy in this specific TNNT2 mutation leading to LVNC. 10.1093/cvr/cvq009
    Echocardiographic quantification of regional deformation helps to distinguish isolated left ventricular non-compaction from dilated cardiomyopathy. Niemann Markus,Liu Dan,Hu Kai,Cikes Maja,Beer Meinrad,Herrmann Sebastian,Gaudron Philipp Daniel,Hillenbrand Hanns,Voelker Wolfram,Ertl Georg,Weidemann Frank European journal of heart failure AIMS:Pronounced trabeculation is presented in both left ventricular non-compaction (LVNC) and dilated cardiomyopathy (DCM), which sometimes makes the differentiation difficult. We hypothesized that echocardiographic deformation analysis would help to differentiate these two cardiomyopathies. METHODS AND RESULTS:We investigated 15 patients with LVNC (9 males; 42 ± 9 years), 15 age- and gender-matched DCM patients, and 15 healthy controls. The echocardiographic diagnosis of LVNC was confirmed by magnetic resonance imaging. In all subjects standard echocardiography and tissue Doppler imaging (TDI) to study regional LV deformation were carried out. No statistical difference was observed in standard echocardiographic parameters between LVNC and DCM patients. Compared with controls, both patient groups showed significantly reduced annular displacements (septal: controls 14 ± 2 mm vs. DCM 6 ± 3 mm vs. LVNC 7 ± 3 mm) and reduced strain values of the LV segments. A characteristic deformation pattern with significantly higher values in the LV base compared with the apex was observed in patients with LVNC by deformation measurements with TDI. This gradient was found particularly in the lateral and inferior wall but spared the anteroseptal wall; non-compaction was not found in basal segments throughout the ventricle and also spared the anteroseptal midventricular wall. In DCM the strain and strain rate values were homogeneously reduced in all LV segments. CONCLUSION:A special regional deformation pattern (preserved deformation in basal segments of LVNC) seems to be of major diagnostic help for the definite differential diagnosis of LVNC and DCM. 10.1093/eurjhf/hfr164
    Hypertrophic cardiomyopathy and left ventricular non-compaction: Different manifestations of the same cardiomyopathy spectrum? Lorca Rebeca,Martín María,Gómez Juan,Santamarta Elena,Morís César,Reguero José Julián R,Coto Eliecer International journal of cardiology 10.1016/j.ijcard.2015.04.138
    Prominent left ventricular trabeculations in competitive athletes: A proposal for risk stratification and management. Caselli Stefano,Ferreira Diana,Kanawati Eyad,Di Paolo Fernando,Pisicchio Cataldo,Attenhofer Jost Christine,Spataro Antonio,Jenni Rolf,Pelliccia Antonio International journal of cardiology BACKGROUND:Recently, an unexpectedly large prevalence of Left Ventricular Non Compaction (LVNC) has been reported in athletes, raising the question of the appropriateness of current diagnostic criteria. We sought to describe prevalence and clinical characteristics of athletes with suspected LVNC in a large cohort of Olympic athletes. METHODS:Over 29months, 2501 consecutive athletes underwent a cardiac evaluation including physical examination, ECG, exercise test and echocardiography. Additional investigations (Cardiac Magnetic Resonance and/or genetic testing) were selectively performed in athletes with abnormal ECGs, ventricular arrhythmias, borderline LV dysfunction or positive family history. RESULTS:Of the 2501 athletes, 36 (1.4%) showed prominent trabeculations suggestive for LVNC. Of these, 3 (0.1%) were considered to be affected by LVNC, based on presence of LV dysfunction (ejection fraction<50%) and/or positive family history and genetic testing; these athletes were cautiously restricted from competitions and entered a clinical follow-up program. The remaining 33 athletes, in the absence of LV impairment or familial cardiac diseases, were considered normal (n=24) or unlikely affected (n=9), regardless of the extent of the trabeculations. CONCLUSIONS:In a large athlete population, a marked LV trabecular pattern was seen in 1.4%. Only a small subset of these athletes (0.1%) showed familial, clinical and morphologic changes supporting the diagnosis of LVNC. In the vast majority of the athletes, the increased trabeculations were not associated with LV dysfunction and/or positive family history, likely representing a morphologic LV variant, deprived of clinical significance. 10.1016/j.ijcard.2016.08.272
    Decreased glycolytic metabolism in non-compaction cardiomyopathy by 18F-fluoro-2-deoxyglucose positron emission tomography: new insights into pathophysiological mechanisms and clinical implications. Tavares de Melo Marcelo Dantas,Giorgi Maria Clementina Pinto,Assuncao Antonildes Nascimento,Dantas Roberto Nery,Araujo Filho Jose de Arimateia,Parga Filho Jose Rodrigues,Bierrenbach Ana Luiza de Souza,de Lima Camila Rocon,Soares José,Meneguetti José Claudio,Mady Charles,Hajjar Ludhmila Abrahão,Kalil Filho Roberto,Bocchi Edimar Alcides,Salemi Vera Maria Cury European heart journal. Cardiovascular Imaging AIMS:The pathophysiological mechanisms of left ventricular non-compaction cardiomyopathy (LVNC) remain controversial. This study performed combined 18F-fluoro-2-deoxyglucose dynamic positron emission tomography (FDG-PET) and 99mTc-sestamibi single-photon emission computed tomography (SPECT) studies to evaluate myocardial glucose metabolism and perfusion in patients with LVNC and their clinical implications. METHODS AND RESULTS:Thirty patients (41 ± 12 years, 53% male) with LVNC, diagnosed by cardiovascular magnetic resonance (CMR) criteria, and eight age-matched healthy controls (42 ± 12 years, 50% male) were prospectively recruited to undergo FDG-PET with measurement of the myocardial glucose uptake rate (MGU) and SPECT to investigate perfusion-metabolism patterns. Patients with LVNC had lower global MGU compared with that in controls (36.9 ± 8.8 vs. 44.6 ± 5.4 μmol/min/100 g, respectively, P = 0.02). Of 17 LV segments, MGU levels were significantly reduced in 8, and also a reduction was observed when compacted segments from LVNC were compared with the segments from control subjects (P < 0.001). Perfusion defects were also found in 15 (50%) patients (45 LV segments: 64.4% match, and 35.6% mismatch perfusion-metabolism pattern). Univariate and multivariate analyses showed that beta-blocker therapy was associated with increased MGU (beta coefficient = 10.1, P = 0.008). Moreover, a gradual increase occurred in MGU across the beta-blocker dose groups (P for trend = 0.01). CONCLUSION:The reduction of MGU documented by FDG-PET in LVNC supports the hypothesis that a cellular metabolic pathway may play a role in the pathophysiology of LVNC. The beneficial effect of beta-blocker mediating myocardial metabolism in the clinical course of LVNC requires further investigation. 10.1093/ehjci/jex036
    Images in cardiovascular medicine. Noncompaction of the ventricular myocardium. Lau Theodore K,Flamm Scott D,Stainback Raymond F Circulation 10.1161/hc0902.103431
    Left ventricular non-compaction in children and adolescents: clinical features, treatment and follow-up. Ergul Yakup,Nisli Kemal,Demirel Atalay,Varkal Muhammed Ali,Oner Naci,Dursun Memduh,Dindar Aygun,Aydogan Umrah,Omeroglu Rukiye Eker Cardiology journal BACKGROUND:Left ventricular non-compaction (LVNC) is a specific cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. This study presents clinical findings, diagnostic features, treatment and follow-up of pediatric patients diagnosed with LVNC. METHODS:Patients with LVNC who were followed from January 2006 to March 2010 were included in this study. Diagnosis was made with the use of characteristic findings of magnetic resonance imaging and echocardiography. Holter electrocardiography and metabolic screening tests were also performed in all patients. RESULTS:A total of 24 patients were studied (18 male, six female). Patient age at diagnosis was 50 ± 60 months (eight days to 15 years). Average follow-up period was 22 ± 12 months (four months to four years). Findings at diagnosis were as follows: eight (33%) patients had heart failure, five (20%) had rhythm abnormalities, five (20%) had cardiomegaly, two had murmurs, two had cyanosis, and two presented with fatigue. Ten (41%) patients had been followed previously with other diagnoses. In 21 (87.5%) patients, electrocardiographic abnormalities were noted, especially left ventricular hypertrophy and ST-T changes. Patients had an average ejection fraction of 46% (18-73%) and three of them had additional congenital heart disease (patent ductus arteriosus, aortopulmonary window and complex cyanotic heart disease). Scanning for metabolic diseases revealed fatty acid oxidation disorder in one patient, and mitochondrial disease in another. During follow-up, a permanent pacemaker was implanted in a patient with severe bradycardia and ventricular dysfunction, and three patients died. CONCLUSION:LVNC can be diagnosed at any age from newborn to adolescent and has a variable clinical course. Closer study of patients with cardiomegaly and heart failure can reduce delays in diagnosis of LVNC.
    Quantification of left ventricular trabeculae using fractal analysis. Captur Gabriella,Muthurangu Vivek,Cook Christopher,Flett Andrew S,Wilson Robert,Barison Andrea,Sado Daniel M,Anderson Sarah,McKenna William J,Mohun Timothy J,Elliott Perry M,Moon James C Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Left ventricular noncompaction (LVNC) is a myocardial disorder characterized by excessive left ventricular (LV) trabeculae. Current methods for quantification of LV trabeculae have limitations. The aim of this study is to describe a novel technique for quantifying LV trabeculation using cardiovascular magnetic resonance (CMR) and fractal geometry. Observing that trabeculae appear complex and irregular, we hypothesize that measuring the fractal dimension (FD) of the endocardial border provides a quantitative parameter that can be used to distinguish normal from abnormal trabecular patterns. METHODS:Fractal analysis is a method of quantifying complex geometric patterns in biological structures. The resulting FD is a unitless measure index of how completely the object fills space. FD increases with increased structural complexity. LV FD was measured using a box-counting method on CMR short-axis cine stacks. Three groups were studied: LVNC (defined by Jenni criteria), n=30(age 41±13; men, 16); healthy whites, n=75(age, 46±16; men, 36); healthy blacks, n=30(age, 40±11; men, 15). RESULTS:In healthy volunteers FD varied in a characteristic pattern from base to apex along the LV. This pattern was altered in LVNC where apical FD were abnormally elevated. In healthy volunteers, blacks had higher FD than whites in the apical third of the LV (maximal apical FD: 1.253±0.005 vs. 1.235±0.004, p<0.01) (mean±s.e.m.). Comparing LVNC with healthy volunteers, maximal apical FD was higher in LVNC (1.392±0.010, p<0.00001). The fractal method was more accurate and reproducible (ICC, 0.97 and 0.96 for intra and inter-observer readings) than two other CMR criteria for LVNC (Petersen and Jacquier). CONCLUSIONS:FD is higher in LVNC patients compared to healthy volunteers and is higher in healthy blacks than in whites. Fractal analysis provides a quantitative measure of trabeculation and has high reproducibility and accuracy for LVNC diagnosis when compared to current CMR criteria. 10.1186/1532-429X-15-36
    Isolated noncompaction of the myocardium. van der Loo Bernd,Jenni Rolf Circulation 10.1161/01.cir.0000055539.05437.f6
    Contrast-enhanced cardiac magnetic resonance in a patient with familial isolated ventricular non-compaction. Korcyk D,Edwards C C,Armstrong G,Christiansen J P,Howitt L,Sinclair T,Bargeois M,Hart H,Patel H,Scott T Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance Isolated ventricular non-compaction (IVNC) is an idiopathic form of cardiomyopathy. Recent clinical reports have suggested that this form of cardiomyopathy is more frequently associated with complications of congestive heart failure, thromboembolism and malignant ventricular arrhythmias. Contrast enhanced cardiac magnetic resonance imaging with its excellent spatial resolution, its large field of view and its ability to demonstrate thrombus and myocardial scar is an excellent modality to non-invasively assess patients with this form of cardiomyopathy. This paper presents a case of familial isolated ventricular non-compaction. We describe the echocardiographic, X-ray angiographic and cardiac MRI findings. Cine imaging using a steady-state free precession sequence (BFFE) was performed in axial and short axis planes. Left ventricular (LV) mass was estimated both with and without the incorporation of trabeculations from a contiguous stack of short axis images. Trabecular mass was expressed as a percentage of total left ventricular mass. We compared trabecular mass: total LV mass in 10 patients with dilated cardiomyopathy. The mean percentage trabecular mass: LV mass in dilated cardiomyopathy was 11.3% (range 1.5%-19%), and this differed significantly from the trabecular mass of the noncompaction patient (two-tailed Mann-Whitney test, p = 0.028). Trabecular mass of greater than 20% of total myocardial mass may be a useful index to suggest the diagnosis of IVNC. Gadolinium was administered (0.1 mmol/kg). Qualitative analysis of first pass perfusion suggested reduced trabecular perfusion. Early imaging with an inversion recovery sequence and a fixed long inversion time did not demonstrate LV thrombus. Late imaging with the same sequence (TI = 280-300 msec) did not demonstrate myocardial fibrosis. 10.1081/jcmr-120030586
    Different types of cardiomyopathy associated with isolated ventricular noncompaction. Biagini Elena,Ragni Luca,Ferlito Marinella,Pasquale Ferdinando,Lofiego Carla,Leone Ornella,Rocchi Guido,Perugini Enrica,Zagnoni Silvia,Branzi Angelo,Picchio Fernando M,Rapezzi Claudio The American journal of cardiology Although mainly described in the context of dilated and hypokinetic left ventricles, it is unclear whether isolated ventricular noncompaction (IVNC) is a distinct cardiomyopathy, a subtype of dilated cardiomyopathy, or a morphogenetic disorder. To investigate the spectrum of cardiomyopathies associated with IVNC, children and adults with stringent echocardiographic diagnoses of IVNC were reviewed. Seventy-three patients (12 children aged <15 years) seen since 1994 satisfied stringent echocardiographic criteria for IVNC. Sixty-five patients (89%; 11 children) had dilated cardiomyopathy, 2 adults had clear-cut hypertrophic cardiomyopathy, 1 adult had restrictive cardiomyopathy (to the investigators' knowledge, the first reported example of this particular association), and 5 patients (1 child) had normal left ventricular morphology and function. In conclusion, knowledge that IVNC can co-exist with restrictive and hypertrophic cardiomyopathy (in addition to the dilated form) supports the concept that IVNC is a morphologic trait rather than a distinct cardiomyopathy. This knowledge should be taken into account during echocardiographic examination and encourage the use of contrast echocardiography (and magnetic resonance) and could also orient molecular biology studies. 10.1016/j.amjcard.2006.04.021
    Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Grothoff Matthias,Pachowsky Milena,Hoffmann Janine,Posch Maximilian,Klaassen Sabine,Lehmkuhl Lukas,Gutberlet Matthias European radiology OBJECTIVES:To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls. METHODS:We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMI(compacted)), non-compacted (LV-MMI(non-compacted)), percentage LV-MM(non-compacted), ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett's test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed. RESULTS:Total LV-MMI(non-compacted) and percentage LV-MM(non-compacted) were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m(2) and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of ≥3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE. CONCLUSIONS:Absolute CMR quantification of the LV-MMI(non-compacted) or the percentage LV-MM(non-compacted) and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies. KEY POINTS:Cardiac magnetic resonance imaging can reliably diagnose left ventricular non-compaction cardiomyopathy. Differentiation of LVNC from other cardiomyopathies and normal hearts is possible. The best diagnostic performance can be achieved if combined MRI criteria for the diagnosis are used. 10.1007/s00330-012-2554-7
    Myocardial fibrosis in left ventricular non-compaction: is late gadolinium enhancement indeed indicative of fibrosis? Alter Peter,Rupp Heinz European journal of heart failure 10.1093/eurjhf/hfr033
    Clinical significance of right ventricular dysfunction in left ventricular non-compaction cardiomyopathy. Leung Steve W,Elayi Claude-Samy,Charnigo Richard J,Syed Mushabbar A The international journal of cardiovascular imaging Left ventricular non-compaction (LVNC) is described as the persistence of trabeculated myocardium in the left ventricle (LV) and is optimally assessed by cardiac magnetic resonance (CMR). Right ventricular (RV) involvement in LVNC remains poorly studied. Consecutive patients (N = 14) diagnosed with LVNC by CMR were studied. Their clinical data were analyzed. In addition, CMR assessment included quantification of LV and RV volumes, mass, ejection fraction (EF), LV wall motion score, LV non-compacted segments and non-compacted to compacted myocardium ratios. Average age of presentation was 33.1 ± 17.6 years old, with 9 males (64%). Of these patients, 7 (50%) presented with acute heart failure and 3 (21%) with syncope, including 1 documented ventricular tachycardia. RV EF < 35% was identified in 7 (50%) of these patients. Patients with RV EF < 35% presented at a higher median New York Heart Association class (1 [IQR 1-2] vs. 3 [IQR 2-4], P = 0.021) and had significantly lower LV EF (50.7% ± 15.4 vs. 21.8% ± 19.9, P = 0.029), higher LV end diastolic (100.9 ml/m(2) ± 22.3 vs. 159.1 ml/m(2) ± 36.0, P = 0.002) and systolic volume indices (52.0 ml/m(2) ± 25.8 vs. 129.1 ml/m(2) ± 48.4, P = 0.002), higher LV wall motion score index (1.3 ± 0.5 vs. 2.2 ± 0.6, P = 0.004) and higher ratio of LV non-compacted to compacted myocardium (3.3 ± 0.6 vs. 4.1 ± 0.8, P = 0.026). All 4 patients that had ventricular tachycardia also had RV dysfunction. RV dysfunction was present in half of patients with LVNC. Significant RV dysfunction seems to be a marker of advanced LVNC and may carry a worse prognosis. Further studies in a larger sample of patients are needed to confirm those observations. 10.1007/s10554-011-9925-z
    Abnormalities of myocardial perfusion and glucose metabolism in patients with isolated left ventricular non-compaction. Gao Xiao-Jin,Li Yan,Kang Lian-Ming,Zhang Jian,Lu Min-Jie,Wan Jun-Yi,Luo Xiao-Liang,He Zuo-Xiang,Zhao Shi-Hua,Yang Min-Fu,Yang Yue-Jin Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology BACKGROUND:The prevalence of myocardial perfusion and glucose metabolic abnormalities and their significance in patients with isolated left ventricular non-compaction (ILVNC) have not been well investigated. METHODS:Seventeen ILVNC patients who underwent cardiac magnetic resonance (CMR) and (99m)Tc-sestamibi SPECT/fluorine-18 deoxyglucose ((18)F-FDG) PET imaging were included. Left ventricular non-compaction, regional wall motion abnormalities, left ventricular ejection fraction (LVEF), and delayed enhancement (DE) were estimated using CMR. Myocardial perfusion and metabolism were evaluated with SPECT/PET. RESULTS:Ninety-five (32.9%) segments were considered non-compacted. DE was present in 52 (18.0%) segments and 10 (58.8%) patients. The rate of occurrence of DE was significantly higher in compacted segments than in non-compacted segments (22.7% vs 8.4%, P = .003). Myocardial perfusion abnormalities were present in 92 (31.8%) segments, of which 66 were perfusion/metabolism match and 26 were perfusion/metabolism mismatch. The rate of occurrence of perfusion abnormality was similar between compacted and non-compacted segments (32.0% vs 31.6%, P = .948), but it was significantly higher in segments with DE than in those without DE (51.9% vs 27.4%, P = .001). None of the imaging features alone (non-compaction, DE, perfusion abnormalities, match or mismatch) showed significant correlations with LVEF (all P > .05). CONCLUSION:In the current study, myocardial perfusion/metabolism mismatch and match were observed in both non-compacted and compacted myocardium in ILVNC patients. Further research is warranted to determine their pathologic and clinical significance. 10.1007/s12350-014-9890-8
    Myocardial deformation pattern in left ventricular non-compaction: Comparison with dilated cardiomyopathy. Huttin Olivier,Venner Clément,Frikha Zied,Voilliot Damien,Marie Pierre-Yves,Aliot Etienne,Sadoul Nicolas,Juillière Yves,Brembilla-Perrot Béatrice,Selton-Suty Christine International journal of cardiology. Heart & vasculature INTRODUCTION:Left ventricular (LV) systolic dysfunction is the most frequent initial presentation of patient with LV noncompaction (NC). Our objectives were to evaluate myocardial contraction properties in patients with LVNC and the relationship of non-compacted segments with the degree of global and regional systolic deformation. METHODS:We included 50 LVNC with an echocardiography and speckle imaging calculation of peak longitudinal strain (PLS). Each of the 16 LV myocardial segments was defined as NC (ratio NC/compacted layer > 2), borderline (NC/C 0-2) and compacted (NC/C = 0). Basal, median and apical strain values were calculated as the average of segmental strain values. For comparison a group of 50 patients with dilated cardiomyopathy (DCM) underwent the same measurements. RESULTS:There was no statistical difference between the 2 groups for any conventional LV systolic parameters. A characteristic deformation pattern was observed in LVNC with higher strain values in the LV apical segments (- 12.8 ± 5.9 vs - 10.7 ± 5.7) and an apical-basal ratio (1.52 ± 0.73 vs 1.12 ± 0.42; p < 0.001). There was no correlation between LV function and the degree of NC. Among 726 segments, compacta thickness was thinner in NC vs C segments (6.4 ± 1.4 vs 7.7 ± 1.8 mm; p < 0.05). There was no difference in WMS but regional strain values were significantly higher in NC compared to C segments (- 13.1 ± 6.1 vs - 10.2 ± 6.3; p < 0.05). CONCLUSIONS:Compared to DCM, LVNC presented with relatively preserved apical deformation as compared to basal segments. Lower regional deformation values in compacted segments confirm the concept that LVNC is a phenotypic marker of an underlying diffuse cardiomyopathy involving both C and NC myocardium. 10.1016/j.ijcha.2014.11.001
    The Desmin () Mutation p.A337P Is Associated with Left-Ventricular Non-Compaction Cardiomyopathy. Kulikova Olga,Brodehl Andreas,Kiseleva Anna,Myasnikov Roman,Meshkov Alexey,Stanasiuk Caroline,Gärtner Anna,Divashuk Mikhail,Sotnikova Evgeniia,Koretskiy Sergey,Kharlap Maria,Kozlova Viktoria,Mershina Elena,Pilus Polina,Sinitsyn Valentin,Milting Hendrik,Boytsov Sergey,Drapkina Oxana Genes Here, we present a small Russian family, where the index patient received a diagnosis of left-ventricular non-compaction cardiomyopathy (LVNC) in combination with a skeletal myopathy. Clinical follow-up analysis revealed a LVNC phenotype also in her son. Therefore, we applied a broad next-generation sequencing gene panel approach for the identification of the underlying mutation. Interestingly, -p.A337P was identified in the genomes of both patients, whereas only the index patient carried -p.L1348X. encodes the muscle-specific intermediate filament protein desmin and encodes desmoplakin, which is a cytolinker protein connecting desmosomes with the intermediate filaments. Because the majority of mutations cause severe filament assembly defects and because this mutation was found in both affected patients, we analyzed this mutation in vitro by cell transfection experiments in combination with confocal microscopy. Of note, desmin-p.A337P forms cytoplasmic aggregates in transfected SW-13 cells and in cardiomyocytes derived from induced pluripotent stem cells underlining its pathogenicity. In conclusion, we suggest including the gene in the genetic analysis for LVNC patients in the future, especially if clinical involvement of the skeletal muscle is present. 10.3390/genes12010121
    Images in cardiovascular medicine. Cardiac imaging in isolated noncompaction of ventricular myocardium. Baumhäkel Magnus,Janzen Ingrid,Kindermann Michael,Schneider Günther,Hennen Benno,Böhm Michael Circulation 10.1161/01.cir.0000024107.08695.bc
    Left ventricular trabeculation assessment with cardiac magnetic resonance. Fernández-Golfín Covadonga,Gómez José Zamorano Journal of cardiovascular medicine (Hagerstown, Md.) 10.2459/JCM.0b013e32833833bc
    Left ventricular noncompaction: analysis of a pediatric population. António Marta,Costa Carmen,Venâncio Margarida,Martins Paula,Dionísio Teresa,Pires António,Sousa Graça,Santos Isabel,Mota Ana,Duarte Ricardo,Costa Hélder,Ribeiro Lúcia,Saraiva Jorge,Castela Eduardo Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology INTRODUCTION:Left ventricular noncompaction (LVNC) is a rare and potentially progressive cardiomyopathy, characterized by the persistence of multiple trabeculations and deep intratrabecular recesses in the ventricular myocardium. Although two-dimensional and color Doppler echocardiography are the most useful diagnostic modalities, cardiac magnetic resonance imaging has proved to have high sensitivity and specificity in the diagnosis of this anomaly. OBJECTIVE:To characterize the clinical and imaging features of LVNC in a pediatric population and to assess their evolution. METHODS AND RESULTS:We performed a retrospective chart review of five pediatric patients with LVNC, followed at Coimbra Pediatric Hospital between January 1999 and December 2007. Median age at presentation was five months (ranging from one day to 13 years), and they were mainly male (1.5:1). Two of the children had a family history of sudden death. In one case the clinical presentation was cardiac arrest due to ventricular fibrillation and in three others, congestive cardiac failure. None of the five cases had associated congenital cardiac anomalies. Involvement of the ventricular apical region was found in all cases. Four children additionally had ventricular dysfunction which improved with diuretic and vasodilator therapy. Mean follow-up was 34 months, ranging from six months to seven years. In one case a change in the morphological phenotype was noted, from a dilated to a hypertrophic form. In this case and in the child's father a mutation in the MYBPC3 gene was identified, which is associated with hypertrophic cardiomyopathy. No thromboembolic phenomena or deaths occurred during the study period. CONCLUSION:In the pediatric population, congestive cardiac failure is the most common clinical presentation of LVNC, which can coexist with other cardiomyopathies, particularly dilated and hypertrophic forms. The sample presented in this analysis is statistically non-significant due to its limited size and the authors highlight the need for larger prospective studies in the pediatric population in order to clarify this disease and its diagnostic criteria.
    Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. Caselli Stefano,Autore Camillo,Serdoz Andrea,Santini Daria,Musumeci Maria Beatrice,Pelliccia Antonio,Agati Luciano Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography BACKGROUND:Despite several efforts using two-dimensional echocardiography and cardiac magnetic resonance in the diagnosis of left ventricular noncompaction (LVNC), there are no universally accepted diagnostic criteria. The aim of this study was to describe the extent of noncompacted myocardium using a new three-dimensional echocardiographic parameter. METHODS:Seventeen patients with diagnoses of LVNC on the basis of two-dimensional echocardiographic and clinical criteria, 26 Olympic rowing athletes, and 49 healthy volunteers underwent three-dimensional echocardiography. By offline analysis, left ventricular volumes, mass, ejection fraction, and sphericity index were calculated. Trabeculated left ventricular volume (TLV) was calculated as the difference between left ventricular end-diastolic volume obtained including and excluding the trabeculae in the cavity contour. TLV was also normalized by left ventricular end-diastolic volume (TLV%). RESULTS:TLV and TLV% were significantly higher in patients with LVNC (33.7 ± 10.9 mL and 24 ± 7%) as opposed to controls (7.1 ± 2.2 mL, P < .001, and 6 ± 2%, P < .001, respectively) and athletes (8.0 ± 3.0 mL, P < .001, and 5 ± 2%, P < .001, respectively). In detail, on receiver operating characteristic curve analysis, optimal cutoff values of 15.8 mL for TLV and 12.8% for TLV% were determined for the identification of LVNC (area under the curve, 1.00; P < .001). Mild positive correlations of TLV and TLV% were found with sphericity index (r = 0.294, P = .004, and r = 0.301, P = .004, respectively), and mild negative correlations were found with ejection fraction (r = -0.454, P < .001, and r = -0.217, P = .038, respectively). CONCLUSIONS:Because of high spatial resolution and accuracy in volumetric quantification, three-dimensional echocardiography allows accurate measurement of the extent of noncompacted myocardium and identification of patients with LVNC. 10.1016/j.echo.2011.11.012
    Left-ventricular non-compaction-comparison between different techniques of quantification of trabeculations: Should the diagnostic thresholds be modified? Donghi Valeria,Tradi Farouk,Carbone Andreina,Viala Marie,Gaubert Guillaume,Nguyen Karine,Reant Patricia,Donal Erwan,Eicher Jean-Christophe,Selton-Suty Christine,Huttin Olivier,Resseguier Noemie,Michel Nicolas,Guazzi Marco,Jacquier Alexis,Habib Gilbert Archives of cardiovascular diseases BACKGROUND:Diagnosis of left ventricular non-compaction (LVNC) is challenging, and different imaging techniques propose different criteria. AIM:To compare the value of two-dimensional transthoracic echocardiography (2D-TTE) and cardiac magnetic resonance (CMR) criteria in diagnosing LVNC, and to test a new trabecular quantification method obtained by 2D-TTE, exploring its relationship with CMR non-compacted mass quantification. METHODS:From a multicentre French study, we selected 48 patients with LVNC and 20 with dilated cardiomyopathy (DCM) who underwent 2D-TTE and CMR. Current 2D-TTE (Jenni et al.) and CMR criteria (Petersen et al., Jacquier et al.), were tested. A new 2D-TTE method of trabecular quantification (percentage of trabecular area) was also proposed, and compared with current criteria. RESULTS:The best cut-off values for the diagnosis of LVNC were a non-compacted/compacted ratio≥2.3 (Petersen et al.), a trabeculated left ventricular mass≥20% (Jacquier et al.) and a non-compacted/compacted ratio≥1.8 (Jenni et al.). Lowering the threshold for the criterion of Jenni et al. from>2 to ≥1.8 improved its sensitivity from 69% to 98%. The 2D-TTE percentage of trabecular area was 25.9±8% in the LVNC group vs. 9.9±4.4% in the DCM group (P<0.05), and was well correlated with CMR non-compacted mass (r=0.65; P<0.05). A 15.8% threshold value for 2D-TTE percentage of trabecular area predicted LVNC diagnosis with a specificity of 95% and a sensitivity of 92%; its sensitivity was better than that for the criteria of Jenni et al. (P<0.01) and Petersen et al. (P=0.03). CONCLUSIONS:Revision of the current threshold for the criterion of Jenni et al. from>2 to ≥1.8 is necessary to improve LVNC diagnosis in patients with left ventricular dysfunction. A new 2D-TTE trabecular quantification method improves TTE diagnosis of LVNC. 10.1016/j.acvd.2020.01.004
    A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults. Ross Samantha B,Jones Katherine,Blanch Bianca,Puranik Rajesh,McGeechan Kevin,Barratt Alexandra,Semsarian Christopher European heart journal AIMS:To assess the reported prevalence of left ventricular non-compaction (LVNC) in different adult cohorts, taking in to consideration the role of diagnostic criteria and imaging modalities used. METHODS AND RESULTS:A systematic review and meta-analysis of studies reporting LVNC prevalence in adults. Studies were sourced from Pre-Medline, Medline, and Embase and assessed for eligibility according to inclusion criteria. Eligible studies provided a prevalence of LVNC in adult populations (≥12 years). Studies were assessed, and data extracted by two independent reviewers. Fifty-nine eligible studies documenting LVNC in 67 unique cohorts were included. The majority of studies were assessed as moderate or high risk of bias. The pooled prevalence estimates for LVNC were consistently higher amongst cohorts diagnosed on cardiac magnetic resonance (CMR) imaging (14.79%, n = 26; I2 = 99.45%) compared with echocardiogram (1.28%, n = 36; I2 = 98.17%). This finding was unchanged when analysis was restricted to studies at low or moderate risk of bias. The prevalence of LVNC varied between disease and population representative cohorts. Athletic cohorts demonstrated high pooled prevalence estimates on echocardiogram (3.16%, n = 5; I2 = 97.37%) and CMR imaging (27.29%, n = 2). CONCLUSION:Left ventricular non-compaction in adult populations is a poorly defined entity which likely encompasses both physiological adaptation and pathological disease. There is a higher prevalence with the introduction of newer imaging technologies, specifically CMR imaging, which identify LVNC changes more readily. The clinical significance of these findings remains unclear; however, there is significant potential for overdiagnosis, overtreatment, and unnecessary follow-up. 10.1093/eurheartj/ehz317
    [Clinical and genetic features of left ventricular noncompaction: a continuum in cardiomyopathies]. Baldi Massimo,Sgalambro Aurelio,Nistri Stefano,Girolami Francesca,Baldini Katia,Fantini Silvia,Grifoni Camilla,Rega Luigi,Olivottol Iacopo,Cecchi Franco Giornale italiano di cardiologia (2006) Isolated left ventricular non-compaction (LVNC) is a rare genetic form of cardiomyopathy (CM) characterized by prominent left ventricular wall trabeculation and intertrabecular recesses communicating with the ventricular cavity. Clinical signs are variable, ranging from lack of symptoms to severe manifestations including heart failure, sustained ventricular arrhythmias, cardioembolism and sudden death. The diagnosis of LVNC is frequently missed, due to limited awareness in the medical community. Contemporary diagnostic sensitivity has been enhanced by the introduction of specific morphologic criteria by high resolution echocardiography and cardiac magnetic resonance. As a consequence, LVNC has been diagnosed more frequently in association with other disorders such as congenital heart disease or genetic CM. The clinical relevance of regional non-compaction in the context of other cardiac diseases is still uncertain. Recent evidence points to an overlapping genetic background encompassing LVNC, hypertrophic and dilated CM, suggesting a continuum of disease associated with sarcomere protein gene mutations. This concept may prove relevant to the understanding of common pathogenetic mechanisms of CM and offer novel research opportunities.
    [Comparison of clinical and MRI features between dilated cardiomyopathy and left ventricular noncompaction]. Yu Jin-chao,Zhao Shi-hua,Jiang Shi-liang,Wang Li-ming,Wang Zhen-fu,Lu Min-jie,Ling Jian,Zhang Yan,Yan Chao-wu,Liu Qiong,Cheng Huai-bing Zhonghua xin xue guan bing za zhi OBJECTIVE:To characterize the clinical and cardiac MRI features of dilated cardiomyopathy (DCM) and left ventricular noncompaction (LVNC). METHODS:Compared the clinical and MRI features between 25 patients with LVNC and 21 patients with DCM. The MRI derived diastolic left ventricular wall thickness and the number and degree of noncompaction (NC) were evaluated using the 17-segment model. RESULTS:Chest distress, shortness of breath and abnormal ECG were presented in all DCM patients, abnormal ECG was evidenced in 22 LVNC patients and 21 out of 25 LVNC patients presented similar clinical symptoms as DCM patients while the rest 4 LVNC patients were asymptomatic. Left atrial and ventricular dimensions were significantly smaller in LVNC patients compared to DCM patients. The degree of left ventricular (LV) spherical remodeling was significantly greater in patients with DCM (sphericity index, SI = 0.81 +/- 0.06) than in patients with LVNC (SI = 0.74 +/- 0.11, P < 0.05). The LV ejection fraction (LVEF) was significantly higher in patients with LVNC (32.7% +/- 14.2%) than that in patients with DCM (15.0% +/- 5.1%). The number of NC segments in LVNC patients (9 +/- 1) was significantly higher than the number of hypertrabeculation segment in DCM patients (5 +/- 2). The left ventricular apex (the 17th segment) was unexceptionally involved in all LVNC patients, while hypertrabeculation was absent in the 17th segment of DCM patients. The NC was more common in the apical and mid segments (16th, 12th and 11th segments) than in basal and mid septal segments (2nd, 3rd, 8th and 9th segments) in both LVNC and DCM patients. The thickness of compacted myocardium of the segments associated with noncompaction appeared thin in two groups. The wall thickness of noncompaction myocardium segments was thicker in LVNC patients than in DCM patients. The end-diastolic NC/C ratio was, on average, higher in patients with LVNC (3.3 +/- 0.6) than in patients with DCM (1.9 +/- 0.3). CONCLUSIONS:The clinical manifestation is similar while there are significant differences in the morphology and function of left atria and left ventricle between the LVNC and DCM patients. The different distribution and degree of NC were helpful to differentiate LVNC from DCM.
    Influence of observer-dependency on left ventricular hypertrabeculation mass measurement and its relationship with left ventricular volume and ejection fraction -  comparison between manual and semiautomatic CMR image analysis methods. Kubik Marcin,Dąbrowska-Kugacka Alicja,Dorniak Karolina,Kutniewska-Kubik Marta,Daniłowicz-Szymanowicz Ludmiła,Lewicka Ewa,Szurowska Edyta,Raczak Grzegorz PloS one BACKGROUND:Recent studies concerning left ventricular noncompaction (LVNC) suggest that the extent of left ventricular (LV) hypertrabeculation has no impact on prognosis. The variety of methods of LV noncompacted myocardial mass (NCM) assessment may influence the results. Hence, we compared two methods of NCM estimation: largely observer-independent Hautvast's(H) computed algorithm-based approach and commonly used Jacquier's(J) method, and their associations with LV end-diastolic volume (EDV) and ejection fraction (EF). METHODS:Cardiac magnetic resonance images of 77 persons (45±17yo) - 42 LVNC, 15 non-ischemic dilative cardiomyopathy, 20 control group were analyzed. LVNC patients were divided into the subgroup with normal (LVNCN) and high EDV (LVNCDCM). NCM and total left ventricular mass (LVM) were estimated by Hautvast's [excluding intertrabecular blood (ITB) and including papillary muscles (PMs) into NCM] and Jacquier's approach (including ITB and PMs, if unclearly distinguished, into NCM). RESULTS:The cut-off value of NCM for LVNC diagnosis was 22% (AUC 0.933) for NCMH/LVMH and 26% (AUC 0.883) for NCMJ/LVMJ. Inter- and intra-observer variability (estimated by coefficient of variation [CoV] and intraclass correlation coefficient [ICC]) of NCMH/LVMH appeared better than of NCMJ/LVMJ (CoV 4.3%, ICC 0.981 and CoV 4.9%, ICC 0.978; respectively for NCMH/LVMH, while for NCMJ/LVMJ: CoV 19.7%, ICC 0.15 and CoV 12.9%, ICC 0.504). In LVNCN subgroup, the correlation between EDV and NCMH was stronger than NCMJ (r = 0.677, p<0.001 vs. r = 0.480, p = 0.038; respectively). In LVNC the EDV correlated with NCMH/LVMH (r = 0.391, p<0.01), but not with NCMJ/LVMJ. In the overall group a relationship was present between EF and NCMH/LVMH (r = -0.449, p<0.001), but not NCMJ/LVMJ. Only NCMH/LVMH explained the variability of EDV (b 0.434, p<0.001). CONCLUSIONS:Choosing a method of NCM assessment that is less observer-dependent might increase the reliability of results. The impact of method selection on the LV parameters and cut-off values for hypertrabeculation should be further investigated. 10.1371/journal.pone.0230134
    Left ventricular non-compaction: clinical features and cardiovascular magnetic resonance imaging. Yousef Zaheer R,Foley Paul W X,Khadjooi Kayvan,Chalil Shajil,Sandman Harald,Mohammed Noor U H,Leyva Francisco BMC cardiovascular disorders BACKGROUND:It is apparent that despite lack of family history, patients with the morphological characteristics of left ventricular non-compaction develop arrhythmias, thrombo-embolism and left ventricular dysfunction. METHODS:Forty two patients, aged 48.7 +/- 2.3 yrs (mean +/- SEM) underwent cardiovascular magnetic resonance (CMR) for the quantification of left ventricular volumes and extent of non-compacted (NC) myocardium. The latter was quantified using planimetry on the two-chamber long axis LV view (NC area). The patients included those referred specifically for CMR to investigate suspected cardiomyopathy, and as such is represents a selected group of patients. RESULTS:At presentation, 50% had dyspnoea, 19% chest pain, 14% palpitations and 5% stroke. Pulmonary embolism had occurred in 7% and brachial artery embolism in 2%. The ECG was abnormal in 81% and atrial fibrillation occurred in 29%. Transthoracic echocardiograms showed features of NC in only 10%. On CMR, patients who presented with dyspnoea had greater left ventricular volumes (both p < 0.0001) and a lower left ventricular ejection fraction (LVEF) (p < 0.0001) than age-matched, healthy controls. In patients without dyspnoea (n = 21), NC area correlated positively with end-diastolic volume (r = 0.52, p = 0.0184) and end-systolic volume (r = 0.56, p = 0.0095), and negatively with EF (r = -0.72, p = 0.0001). CONCLUSION:Left ventricular non-compaction is associated with dysrrhythmias, thromboembolic events, chest pain and LV dysfunction. The inverse correlation between NC area and EF suggests that NC contributes to left ventricular dysfunction. 10.1186/1471-2261-9-37
    [Isolated left ventricular non-compaction associated with Ebstein's anomaly. Multimodality non-invasive imaging for the assessment of congenital heart disease]. Renilla Alfredo,Santamarta Elena,Corros Cecilia,Martín María,Barreiro Manuel,de la Hera Jesús Archivos de cardiologia de Mexico To establish the etiology of heart failure in patients with congenital heart disease can be challenging. Multiple concomitant anomalies that can be missed after an initial diagnosis could be seen in these patients. In patients with congenital heart disease, a more accurate evaluation of cardiac morphology and left ventricular systolic function could be evaluated by recent non-invasive cardiac imaging techniques. We present a rare case where multimodal cardiac imaging was useful to establish the final diagnosis of left ventricular non-compaction associated with Ebstein's anomaly. 10.1016/j.acmx.2013.03.003
    Left ventricular noncompaction cardiomyopathy: adult association with 1p36 deletion syndrome. Lee James,Rinehart Sarah,Polsani Venkateshewar Methodist DeBakey cardiovascular journal 10.14797/mdcj-10-4-258
    Myocardial T1 mapping and extracellular volume quantification in patients with left ventricular non-compaction cardiomyopathy. Araujo-Filho José A B,Assuncao Antonildes N,Tavares de Melo Marcelo D,Bière Loïc,Lima Camila R,Dantas Roberto N,Nomura Cesar H,Salemi Vera M C,Jerosch-Herold Michael,Parga Jose R European heart journal cardiovascular Imaging Aims:From pathophysiological mechanisms to risk stratification and management, much debate and discussion persist regarding left ventricular non-compaction cardiomyopathy (LVNC). This study aimed to characterize myocardial T1 mapping and extracellular volume (ECV) fraction by cardiovascular magnetic resonance (CMR), and investigate how these biomarkers relate to left ventricular ejection fraction (LVEF) and ventricular arrhythmias (VA) in LVNC. Methods and results:Patients with LVNC (n = 36) and healthy controls (n = 18) were enrolled to perform a CMR with T1 mapping. ECV was quantified in LV segments without late gadolinium enhancement (LGE) areas to investigate diffuse myocardial fibrosis. Patients with LVNC had slightly higher native T1 (1024 ± 43 ms vs. 995 ± 22 ms, P = 0.01) and substantially expanded ECV (28.0 ± 4.5% vs. 23.5 ± 2.2%, P < 0.001) compared to controls. The ECV was independently associated with LVEF (β = -1.3, P = 0.001). Among patients without LGE, VAs were associated with higher ECV (27.7% with VA vs. 25.8% without VA, P = 0.002). Conclusion:In LVNC, tissue characterization by T1 mapping suggests an extracellular expansion by diffuse fibrosis in myocardium without LGE, which was associated with myocardial dysfunction and VA, but not with the amount of non-compacted myocardium. 10.1093/ehjci/jey022
    Speckle myocardial imaging modalities for early detection of myocardial impairment in isolated left ventricular non-compaction. Bellavia Diego,Michelena Hector I,Martinez Matthew,Pellikka Patricia A,Bruce Charles J,Connolly Heidi M,Villarraga Hector R,Veress Gabriella,Oh Jae K,Miller Fletcher A Heart (British Cardiac Society) OBJECTIVE:To examine the hypothesis that speckle myocardial imaging (SMI) modalities, including longitudinal, radial and circumferential systolic (s) and diastolic (d) myocardial velocity imaging, displacement (D), strain rate (SR) and strain (S), as well as left ventricular (LV) rotation/torsion are sensitive for detecting early myocardial dysfunction in isolated LV non-compaction (iLVNC). DESIGN AND RESULTS:Twenty patients with iLVNC diagnosed by cardiac magnetic resonance (15) or echocardiography (5) were included. Patients were divided into two groups: ejection fraction (EF)>50% (n=10) and EF<or=50% (n=10). Standard measures of systolic and diastolic function including pulsed wave tissue Doppler Imaging (PWTDI) were obtained. Longitudinal, radial and circumferential SMI, and LV rotation/torsion were compared with values for 20 age/sex-matched controls. EF, PWTDI E', E/E' and all of the SMI modalities were significantly abnormal for patients with EF<or=50% compared with controls. In contrast, EF and PWTDI E', E/E' were not significantly different between controls and patients with iLVNC (EF>50%). However, SMI-derived longitudinal sS, sSR, sD and radial sS, as well as LV rotation/torsion values, were all reduced in iLVNC (EF>50%) compared with controls. Measurements with the highest discriminating power between iLVNC (EF>50%) and controls were longitudinal sS mean of the six apical segments (area under the curve (AUC)=0.94), sS global average (AUC=0.94), LV rotation apical mean (AUC=0.94); LV torsion (AUC=0.93) LV torsion rate (AUC=0.94). CONCLUSIONS:LV SMI values are reduced in patients with iLVNC, even those with normal EF and PWTDI. The most accurate SMI modalities to discriminate between patients and controls are longitudinal sS mean of the six apical segments, LV apical rotation or LV torsion rate. 10.1136/hrt.2009.182170
    Cardiac magnetic resonance of left ventricular trabeculation: the new normal. McNally Elizabeth M,Patel Amit R Circulation. Cardiovascular imaging 10.1161/CIRCIMAGING.110.962472
    Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction. Uribe Sergio,Cadavid Lina,Hussain Tarique,Parra Rodrigo,Urcelay Gonzalo,Heusser Felipe,Andía Marcelo,Tejos Cristian,Irarrazaval Pablo Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Isolated left ventricular non-compaction (LVNC) is an uncommon disorder characterized by the presence of increased trabeculations and deep intertrabecular recesses. In adults, it has been found that ejection fraction (EF) decreases significantly as non-compaction severity increases. In children however, there are a few data describing the relation between anatomical characteristics of LVNC and ventricular function. We aimed to find correlations between morphological features and ventricular performance in children and young adolescents with LVNC using cardiovascular magnetic resonance (CMR). METHODS:15 children with LVNC (10 males, mean age 9.7 y.o., range 0.6-17 y.o.), underwent a CMR scan. Different morphological measures such as the compacted myocardial mass (CMM), non-compaction (NC) to the compaction (C) distance ratio, compacted myocardial area (CMA) and non-compacted myocardial area (NCMA), distribution of NC, and the assessment of ventricular wall motion abnormalities were performed to investigate correlations with ventricular performance. EF was considered normal over 53%. RESULTS:The distribution of non-compaction in children was similar to published adult data with a predilection for apical, mid-inferior and mid-lateral segments. Five patients had systolic dysfunction with decreased EF. The number of affected segments was the strongest predictor of systolic dysfunction, all five patients had greater than 9 affected segments. Basal segments were less commonly affected but they were affected only in these five severe cases. CONCLUSION:The segmental pattern of involvement of non-compaction in children is similar to that seen in adults. Systolic dysfunction in children is closely related to the number of affected segments. 10.1186/1532-429X-14-9
    Frequency of asymptomatic disease among family members with noncompaction cardiomyopathy. Caliskan Kadir,Michels Michelle,Geleijnse Marcel L,van Domburg Ron T,van der Boon Robert,Balk Aggie H M M,Simoons Maarten L The American journal of cardiology Noncompaction cardiomyopathy (NCC) is a primary cardiomyopathy characterized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricular walls. Most cases are inherited, with a dominant inheritance pattern. The aim of the present study was to determine the prevalence and clinical characteristics of cardiomyopathies in the close relatives of patients with NCC. We evaluated 156, mostly first-degree, family members of 44 adult patients with NCC who agreed to familial screening. A family history of cardiac disease was reported by 16 (36%) of the 44 patients, including premature sudden death in 8 families (18%). NCC (n = 32) or dilated cardiomyopathy (n = 9) was diagnosed in 41 relatives (26%) by echocardiography (n = 25), contrast echocardiography (n = 6), or magnetic resonance imaging (n = 10). Of these family members, 13 already had known cardiac symptoms and signs, but most (28 of 41) were asymptomatic. Most subjects with NCC had mild to moderate left ventricular dysfunction (n = 29, 71%). After a median follow-up of 55 months (interquartile range 43 to 93), most remained asymptomatic. Four family members were treated with prophylactic implantable cardioverter-defibrillator placement and 23 of those with NCC were treated with drugs, including angiotensin-converting enzyme inhibitors (41%), β blockers (34%), and anticoagulants (17%). In conclusion, there is a high prevalence, mostly asymptomatic, of cardiac disease (26%) among first- and second-degree family members of patients with NCC. This warrants screening and offers an opportunity for early intervention. 10.1016/j.amjcard.2012.07.009
    [Isolated noncompaction cardiomyopathy with special emphasis on arrhythmia complications]. Gerecke B,Engberding R Herzschrittmachertherapie & Elektrophysiologie Isolated noncompaction cardiomyopathy (NCCM) is a rare genetically determined myocardial disease caused by abnormal fetal development of the myocardium resulting in a thin compacted and a thicker noncompacted layer of the affected left ventricular (LV) wall. The genetic basis of NCCM is heterogenous. Diagnosis can be made using echocardiography or magnetic resonance imaging. The diagnostic criteria for NCCM are still under discussion. Afflicted patients may present with various symptoms caused by arrhythmias, heart failure and cardioembolic events. Severely reduced LV function as well as left bundle branch block and atrial fibrillation were shown to be linked to worse outcomes. Treatment in patients with NCCM should be targeted at individual symptoms and clinical findings. Therapy includes pharmacological treatment, and in individual cases ablation or device therapy, as well as consideration for heart transplantation in selected cases. Aside from regular clinical follow-up of patients with NCCM screening of first degree family members with assessment of medical history, physical examination, ECG recording, and echocardiography are recommended. 10.1007/s00399-012-0226-6
    Left ventricular noncompaction in patients with bicuspid aortic valve. Agarwal Anushree,Khandheria Bijoy K,Paterick Timothy E,Treiber Shannon C,Bush Michelle,Tajik A Jamil Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography BACKGROUND:Left ventricular noncompaction (LVNC) is commonly associated with complex congenital anomalies. The association of LVNC with less complex but more frequent anomalies, such as bicuspid aortic valve (BAV), is not well described in the literature. The aims of this study were to (1) determine the incidence of association of LVNC with the most common congenital anomaly, BAV, in an echocardiographic database and (2) describe clinical and imaging characteristics of these patients. METHODS:An echocardiography database was retrospectively interrogated to identify 109 patients who fulfilled the echocardiographic criteria for BAV from July 1, 2011, to March 31, 2013. Echocardiograms were carefully evaluated to identify patients with concomitant LVNC. RESULTS:Twelve patients (11.0%) with BAV fulfilled the criteria for LVNC. The mean age at diagnosis was 33 ± 16.9 years; nine of 12 were men. Eight patients (66.7%) had symptoms during initial presentation. The most common BAV morphology was fusion of the right and left coronary cusps. Nine patients had mild or moderate aortic valve dysfunction (aortic regurgitation and/or stenosis), and eight had associated aortopathy. LVNC was located at the apex in all patients except one. Mean systolic global longitudinal strain was -16.9 ± 2.7%. CONCLUSIONS:In this series of patients, concomitant BAV and LVNC were observed in 11% of a BAV population. Further studies are needed to understand the genetic and pathophysiologic basis of this association. 10.1016/j.echo.2013.08.003
    Auxiliary diagnostic potential of ventricle geometry and late gadolinium enhancement in left ventricular non-compaction; non-randomized case control study. Boban Marko,Pesa Vladimir,Gabric Ivo Darko,Manola Sime,Persic Viktor,Antic-Kauzlaric Helena,Zulj Marinko,Vcev Aleksandar BMC cardiovascular disorders BACKGROUND:There are still ambiguities existing in regard to left ventricular non-compaction (LVNC) diagnostic imaging. The aim of our study was to analyze diagnostic potential of late gadolinium enhancement (LGE) and ventricle geometry in patients with LVNC and controls. METHODS:Data on cardiac magnetic resonance imaging (CMR) studies for LVNC were reassessed from the hospital's database (3.75 years; n=1975 exams). Matching sample of controls included cases with no structural heart disease, hypertrophic or dilative cardiomyopathy, arrhythmogenic right ventricular dysplasia or subacute myocarditis. Eccentricity of the left ventricle was measured at end diastole in the region with pronounced NC and maximal to minimal ratio (MaxMinEDDR) was calculated. RESULTS:Study included 255 patients referred for CMR, 100 (39.2%) with LVNC (prevalence in the studied period 5.01%) and 155 (60.8%) controls. Existing LGE had sensitivity of 52.5% (95%-CI:42.3-62.5), specificity of 80.4% (95%-CI:73.2-86.5) for LVNC, area under curve (AUC) 0.664 (95%-CI:0.603-0.722);p<0.001. MaxMinEDDR>1.10 had sensitivity of 95.0% (95%-CI:88.7-98.4), specificity of 82.6% (95%-CI: 75.7-88.2) for LVNC, AUC 0.917 (95%-CI:0.876-0.948); p<0.001. LGE correlated with Max-Min-EDD-R (Rho=0.130; p=0.038) and there was significant difference in ROC analysis ΔAUC0.244 (95%-CI:0.175-0.314); p<0.001. LGE also correlated negatively with stroke volume and systolic function (both p<0.05, respectively). CONCLUSIONS:LGE was found to be frequently expressed in patients with LVNC, but without sufficient power to be used as a discriminative diagnostic parameter. Both LGE and eccentricity of the left ventricle were found to be relatively solid diagnostic landmarks of complex infrastructural and functional changes within the failing heart. 10.1186/s12872-017-0721-0
    Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. Dodd Jonathan D,Holmvang Godtfred,Hoffmann Udo,Ferencik Maros,Abbara Suhny,Brady Thomas J,Cury Ricardo C AJR. American journal of roentgenology OBJECTIVE:The purpose of this study was to investigate whether MRI can quantify the severity and extent of left ventricular noncompaction and detect trabecular delayed hyperenhancement and whether doing so can show a relationship with clinical stage of disease. MATERIALS AND METHODS:In a retrospective blinded study, nine patients with left ventricular noncompaction and 10 control subjects had cardiac MRI studies evaluated for the severity and extent of left ventricular noncompaction and the amount and degree of trabecular delayed hyperenhancement on a myocardial segment basis (16-segment model). Findings were correlated with parameters of clinical stage of disease. RESULTS:Fifty-seven (39%) myocardial segments showed left ventricular noncompaction whereas 22 (17%) showed trabecular delayed hyperenhancement. Significant differences among clinical severity groups were noted in the severity and extent of left ventricular noncompaction at the mid (p < 0.05 and p < 0.005, respectively) and apical levels (p < 0.003 and p < 0.001, respectively), severity of trabecular delayed hyperenhancement at the mid (p < 0.04) and apical levels (p < 0.02), and amount of trabecular delayed hyperenhancement at the apical level (p < 0.006). The extent of left ventricular noncompaction and the amount and degree of trabecular delayed hyperenhancement correlated significantly with ejection fraction (EF) (r = -0.47, -0.53, -0.53, respectively, p < 0.05). The degree of trabecular delayed hyperenhancement was an independent predictor of EF (R2 = 0.30, p < 0.0001). Significant differences in the severity of trabecular delayed hyperenhancement were detected among patients with mild and those with moderate and severe clinical stage of disease (p < 0.0001). CONCLUSION:Cardiac MRI shows trabecular delayed hyperenhancement in left ventricular noncompaction. Evaluating the extent and severity of left ventricular noncompaction and trabecular delayed hyperenhancement may improve the ability of the clinician to predict the clinical stage of disease. 10.2214/AJR.07.2364
    Assessment of left ventricular non-compaction in adults: side-by-side comparison of cardiac magnetic resonance imaging with echocardiography. Thuny Franck,Jacquier Alexis,Jop Bertrand,Giorgi Roch,Gaubert Jean-Yves,Bartoli Jean-Michel,Moulin Guy,Habib Gilbert Archives of cardiovascular diseases BACKGROUND:Two-dimensional echocardiography images obtained at end-diastole and end-systole and cardiac magnetic resonance (CMR) images obtained at end-diastole represent the three imaging methodologies validated for diagnosis of left ventricular non-compaction (LVNC). No study has compared these methodologies in assessing the magnitude of non-compaction. AIMS:To compare two-dimensional echocardiography with CMR in the evaluation of patients with suspected LVNC. METHODS:Sixteen patients (48+/-17 years) with LVNC underwent echocardiography and CMR within the same week. Echocardiography images obtained at end-diastole and end-systole were compared in a blinded fashion with those obtained by CMR at end-diastole to assess non-compaction in 17 anatomical segments. RESULTS:All segments could be analysed by CMR, whereas only 238 (87.5%) and 237 (87.1%) could be analysed by echocardiography at end-diastole and end-systole, respectively (p=0.002). Among the analysable segments, a two-layered structure was observed in 54.0% by CMR, 42.9% by echocardiography at end-diastole and 41.4% by echocardiography at end-systole (p=0.006). Similar distribution patterns were observed with the two echocardiographic methodologies. However, compared with echocardiography, CMR identified a higher rate of two-layered structures in the anterior, anterolateral, inferolateral and inferior segments. Echocardiography at end-systole underestimated the NC/C maximum ratio compared with CMR (p=0.04) and echocardiography at end-diastole (p=0.003). No significant difference was observed between CMR and echocardiography at end-diastole (p=0.83). Interobserver reproducibility of the NC/C maximum ratio was similar for the three methodologies. CONCLUSION:CMR appears superior to standard echocardiography in assessing the extent of non-compaction and provides supplemental morphological information beyond that obtained with conventional echocardiography. 10.1016/j.acvd.2010.01.002
    Trabeculated (noncompacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis. Kawel Nadine,Nacif Marcelo,Arai Andrew E,Gomes Antoinette S,Hundley W Gregory,Johnson W Craig,Prince Martin R,Stacey R Brandon,Lima João A C,Bluemke David A Circulation. Cardiovascular imaging BACKGROUND:A high degree of noncompacted (trabeculated) myocardium in relationship to compact myocardium (trabeculated to compact myocardium [T/M] ratio >2.3) has been associated with a diagnosis of left ventricular noncompaction (LVNC). The purpose of this study was to determine the normal range of the T/M ratio in a large population-based study and to examine the relationship to demographic and clinical parameters. METHODS AND RESULTS:The thickness of trabeculation and the compact myocardium were measured in 8 left ventricular regions on long axis cardiac MR steady-state free precession cine images in 1000 participants (551 women; 68.1±8.9 years) of the Multi-Ethnic Study of Atherosclerosis cohort. Of 323 participants without cardiac disease or hypertension and with all regions evaluable, 140 (43%) had a T/M ratio >2.3 in at least 1 region; in 20 of 323 (6%), T/M >2.3 was present in >2 regions. A multivariable linear regression model revealed no association of age, sex, ethnicity, height, and weight with maximum T/M ratio in participants without cardiac disease or hypertension (P>0.05). In the entire cohort (n=1000), left ventricular ejection fraction (β=-0.02/%; P=0.015), left ventricular end-diastolic volume (β=0.01/mL; P<0.0001), and left ventricular end-systolic volume (β=0.01/mL; P<0.001) were associated with maximum T/M ratio in adjusted models, whereas there was no association with hypertension or myocardial infarction (P>0.05). At the apical level, T/M ratios were significantly lower when obtained on short- compared with long-axis images (P=0.017). CONCLUSIONS:A ratio of T/M of >2.3 is common in a large population-based cohort. These results suggest re-evaluation of the current cardiac MR criteria for left ventricular noncompaction may be necessary. 10.1161/CIRCIMAGING.111.971713
    Left ventricular noncompaction in patients with β-thalassemia: uncovering a previously unrecognized abnormality. Piga Antonio,Longo Filomena,Musallam Khaled M,Veltri Andrea,Ferroni Francesca,Chiribiri Amedeo,Bonamini Rodolfo American journal of hematology Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with potentially serious outcomes. It results in multiple and excessive trabeculations, deep intertrabecular recesses, and a thickened ventricular myocardium with two distinct layers, compacted and noncompacted. The condition is most commonly congenital; however, acquired forms have also been described. A recent report of LVNC detected in a β-thalassemia twin suggested an association with cardiac siderosis. In a cross-sectional study of 135 transfusion-dependent patients with β-thalassemia (130 major and 5 intermedia, mean age 29.6 ± 7.7 years, 49.6% males) presenting for cardiac iron assessment by magnetic resonance imaging (MRI), we evaluated the prevalence and risk factors for LVNC. None of the patients had neuromuscular or congenital heart disease. Eighteen patients (13.3%; 95% confidence interval [CI] = 8.6-20.1) fulfilled the preassigned strict criteria for LVNC on cardiac MRI. There were no statistically significant differences between patients with and without LVNC with respect to demographics; hemoglobin levels; splenectomy status; systemic, hepatic, and cardiac iron overload indices; hepatic disease and infection studies; or iron chelator type. Patients with LVNC were more likely to have heart failure (adjusted odds ratio = 1.77; 95% CI = 0.29-10.89); although with high uncertainty. Patients with β-thalassemia have a higher prevalence of LVNC than normal individuals. As this finding could not be explained by conventional risk factors in this patient population, further investigation of the underlying mechanisms of LVNC is warranted. This remains crucial for an entity with adverse cardiac outcomes, especially in patients with β-thalassemia where cardiac disease remains a primary cause of mortality. 10.1002/ajh.23323
    Left ventricle non-compaction cardiomyopathy: different clinical scenarios and magnetic resonance imaging findings. Martín María,Santamarta Elena,Corros Cecilia,Benito Eva María,León Diego,Velasco Elena,García-Campos Ana,Rodríguez María Luisa,de la Hera Jesús M,Barriales Vicente,Saiz Antonio,Lambert José L Archivos de cardiologia de Mexico Left ventricle non-compaction cardiomyopathy is currently considered as a well-defined individual entity. However, it includes a broad spectrum of clinical, radiological and pathophysiological findings. In this review we describe 3 different scenarios of this entity: an isolated case with severe left ventricle dysfunction, an "associated" case in a patient with previous atrial septum defect and pulmonary stenosis and finally, as a finding in a patient with a transient cerebrovascular ischemic attack. In the 2 last cases, both asymptomatic, morphological criteria of left ventricle non-compaction were found but, ventricular function was normal and cardiac-MRI showed no late gadolinium hyperenhancement. Periodical follow-up and familial screening were recommended. Natural history and prognosis factors of this disease are still not well known. Further and longer series of patients with this diagnosis are needed to completely define radiological criteria, clinical presentation and evolution. 10.1016/j.acmx.2012.12.003
    Reference values for left and right ventricular trabeculation and non-compacted myocardium. André Florian,Burger Astrid,Loßnitzer Dirk,Buss Sebastian J,Abdel-Aty Hassan,Gianntisis Evangelos,Steen Henning,Katus Hugo A International journal of cardiology BACKGROUND:Since the differentiation between physiological and pathological trabeculation is challenging, we assessed its distribution in a reference population of selected healthy volunteers. METHODS:We studied 117 subjects (58 males) stratified into age tertiles and by gender. Cardiovascular magnetic resonance images were acquired using a standard SSFP-sequence. Left and right ventricular (LV/RV) end-diastolic (EDV), end-systolic (ESV) and trabeculated volumes indexed to the body surface area as well as ejection fraction (EF) were quantified in short-axis views. The maximum non-compacted-to-compacted (NC/C) ratio was measured in long-axis views. RESULTS:The trabeculated volumes were significantly larger in men than in women and decreased with age. The correlation between both was moderate (r=0.46; p<0.001). LV trabeculated volume was positively associated with EDV and ESV (r=0.74; r=0.59; both p<0.001) and negatively with EF (r=-0.27; p<0.005). It was no independent predictor for EF. The maximum NC/C ratio was >2.3 in 46.2% and >2.5 in 37.6% of the subjects, which is regarded as abnormal in current literature. The fraction of subjects with a maximum NC/C ratio >2.3 and the mean maximum NC/C ratio differed significantly between gender but not between age groups. An increasing NC/C ratio was associated with a significant decrease in EF (r=-0.21; p<0.05). CONCLUSION:A considerable amount of healthy volunteers fulfils the current diagnostic criteria of LV noncompaction with female subjects showing a higher fraction of false-positive results than males. LV trabeculated volume is more pronounced in young subjects and declines with age. The use of age- and gender-specific reference values as provided in this study may facilitate the delineation of physiological and pathological findings. 10.1016/j.ijcard.2015.03.065
    Quantification of myocardial strain in patients with isolated left ventricular non-compaction and healthy subjects using deformable registration algorithm: comparison with feature tracking. Liu Jia,Li Yumin,Cui Yue,Cao Yukun,Yao Sheng,Zhou Xiaoyue,Wetzl Jens,Zeng Wenjuan,Shi Heshui BMC cardiovascular disorders BACKGROUND:Systolic dysfunction of the left ventricle is frequently associated with isolated left ventricular non-compaction (iLVNC). Clinically, the ejection fraction (EF) is the primary index of cardiac function. However, changes of EF usually occur later in the disease course. Feature tracking (FT) and deformable registration algorithm (DRA) have become appealing techniques for myocardial strain assessment. METHODS:Thirty patients with iLVNC (36.7 ± 13.3 years old) and fifty healthy volunteers (42.3 ± 13.6 years old) underwent cardiovascular magnetic resonance (CMR) examination on a 1.5 T MR scanner. Strain values in the radial, circumferential, longitudinal directions were analyzed based on the short-axis and long-axis cine images using FT and DRA methods. The iLVNC patients were further divided based on the ejection fraction, into EF ≥ 50% group (n = 11) and EF < 50% group (n = 19). Receiver-operating-characteristic (ROC) analysis was performed to assess the diagnostic performance of the global strain values. Intraclass correlation coefficient (ICC) analysis was used to evaluate the intra- and inter-observer agreement. RESULTS:Global radial strain (GRS) was statistically lower in EF ≥ 50% group compared with control group [GRS (DRA)/% vs. controls: 34.6 ± 7.0 vs. 37.6 ± 7.2, P < 0.001; GRS (FT)/% vs. controls: 37.4 ± 13.2 vs. 56.9 ± 16.4, P < 0.01]. ROC analysis of global strain values derived from DRA and FT demonstrated high area under curve (range, 0.743-0.854). DRA showed excellent intra- and inter-observer agreement of global strain in both iLVNC patients (ICC: 0.995-0.999) and normal controls (ICC: 0.934-0.996). While for FT analysis, global radial strain of normal controls showed moderate intra-observer (ICC: 0.509) and poor inter-observer agreement (ICC: 0.394). CONCLUSIONS:In patients with iLVNC, DRA can be used to quantitatively analyze the strain of left ventricle, with global radial strain being an earlier marker of LV systolic dysfunction. DRA has better reproducibility in evaluating both the global and segmental strain. 10.1186/s12872-020-01668-x
    Prognostic value of plasma big endothelin-1 in left ventricular non-compaction cardiomyopathy. Fan Peng,Zhang Ying,Lu Yi-Ting,Yang Kun-Qi,Lu Pei-Pei,Zhang Qiong-Yu,Luo Fang,Lin Ya-Hui,Zhou Xian-Liang,Tian Tao Heart (British Cardiac Society) OBJECTIVE:To determine the prognostic role of big endothelin-1 (ET-1) in left ventricular non-compaction cardiomyopathy (LVNC). METHODS:We prospectively enrolled patients whose LVNC was diagnosed by cardiac MRI and who had big ET-1 data available. Primary end point was a composite of all-cause mortality, heart transplantation, sustained ventricular tachycardia/fibrillation and implanted cardioverter defibrillator discharge. Secondary end point was cardiac death or heart transplantation. RESULTS:Altogether, 203 patients (median age 44 years; 70.9% male) were divided into high-level (≥0.42 pmol/L) and low-level (<0.42 pmol/L) big ET-1 groups according to the median value of plasma big ET-1 levels. Ln big ET-1 was positively associated with Ln N-terminal pro-brain natriuretic peptide, left ventricular diameter, but negatively related to age and Ln left ventricular ejection fraction. Median follow-up was 1.9 years (IQR 0.9-3.1 years). Kaplan-Meier analysis showed that, compared with patients with low levels of big ET-1, those with high levels were at greater risk for meeting both primary (p<0.001) and secondary (p<0.001) end points. The C-statistic estimation of Ln big ET-1 for predicting the primary outcome was 0.755 (95% CI 0.685 to 0.824, p<0.001). After adjusting for confounding factors, Ln big ET-1 was identified as an independent predictor of the composite primary outcome (HR 1.83, 95% CI 1.27 to 2.62, p=0.001) and secondary outcome (HR 1.93, 95% CI 1.32 to 2.83, p=0.001). CONCLUSIONS:Plasma big ET-1 may be a valuable index to predict the clinical adverse outcomes in patients with LVNC. 10.1136/heartjnl-2020-317059
    [MRI quantification of myocardial function, perfusion, and enhancement in patients with left-ventricular noncompaction]. Calvillo P,Martí-Bonmatí L,Chaustre F,Roldán I,Mora V,Peláez A,Cogollos J,Ballestín J Radiologia OBJECTIVE:Left ventricular noncompaction is a congenital malformation characterized by a myocardium organized into two layers, one compacted and one noncompacted. We aimed to quantify myocardial function, perfusion, and delayed enhancement using MRI in patients with left-ventricular noncompaction and to compare these results with those of normal patients. MATERIAL AND METHODS:We included 12 patients with a myocardial noncompaction / compaction ratio>2.3 at end-diastole in at least one segment apart from the apex and 12 healthy subjects matched for age and sex. We calculated the end-diastolic and end-systolic volumes, stroke volume, ejection fraction, cardiac output, myocardial volume and mass, end-diastolic thickness, and left-ventricular wall thickening and motion. From the delayed enhancement images, we obtained the volume and percentage of hyperenhanced myocardium. Student's t test was used to compare groups. RESULTS:We observed a statistically significant increase in end-diastolic and end-systolic volumes in patients with left-ventricular noncompaction, as well as decreased ejection fraction, wall motion, and relative maximum upslope in segments 4, 9, and 10. No significant differences were found in delayed hyperenhancement. CONCLUSION:MRI quantification revealed decreased systolic cardiac function and decreased perfusion (lower relative maximum upslope) in the lower segments in patients with noncompaction. 10.1016/S0033-8338(09)70405-9
    Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Jacquier Alexis,Thuny Franck,Jop Bertrand,Giorgi Roch,Cohen Frederic,Gaubert Jean-Yves,Vidal Vincent,Bartoli Jean Michel,Habib Gilbert,Moulin Guy European heart journal AIMS:To describe a method for measuring trabeculated left ventricular (LV) mass using cardiac magnetic resonance imaging and to assess its value in the diagnosis of left ventricular non-compaction (LVNC). METHODS AND RESULTS:Between January 2003 and 2008, we prospectively included 16 patients with LVNC. During the mean period, we included 16 patients with dilated cardiomyopathy (DCM), 16 patients with hypertrophic cardiomyopathy (HCM), and 16 control subjects. Left ventricular volumes, LV ejection fraction, and trabeculated LV mass were measured in the four different populations. The percentage of trabeculated LV mass was almost three times higher in the patients with LVNC (32 +/- 10%), compared with those with DCM (11 +/- 4%, P < 0.0001), HCM (12 +/- 4%, P < 0.0001), and controls (12 +/- 5%, P < 0.0001). A value of trabeculated LV mass above 20% of the global mass of the LV predicted the diagnosis of LVNC with a sensitivity of 93.7% [95% confidence interval (CI), 71.6-98.8%] and a specificity of 93.7% (95% CI, 83.1-97.8%; kappa = 0.84). CONCLUSION:The method described is reproducible and provides an assessment of the global amount of LV trabeculation. A trabeculated LV mass above 20% of the global LV mass is highly sensitive and specific for the diagnosis of LVNC. 10.1093/eurheartj/ehp595
    Usefulness of cardiac magnetic resonance imaging in left ventricular non-compaction cardiomyopathy. Martín María,Barriales Vicente,Corros Cecilia,Santamarta Elena European journal of heart failure 10.1093/eurjhf/hfr028
    Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. Melendez-Ramirez Gabriela,Castillo-Castellon Francisco,Espinola-Zavaleta Nilda,Meave Aloha,Kimura-Hayama Eric T Journal of cardiovascular computed tomography BACKGROUND:Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by a noncompacted myocardial layer in the left ventricle, primarily diagnosed by echocardiographic and magnetic resonance criteria. Multidetector computed tomography (MDCT) is an imaging method that has been increasingly used in cardiac evaluation. However, tomographic criteria to diagnose LVNC have not been determined. OBJECTIVES:We assessed the structural characteristics of LVNC with MDCT and proposed tomographic criteria that may differentiate LVNC from healthy subjects and patients with other cardiomyopathies that might be associated with increased myocardial trabeculation. METHODS:Between March 2007 and June 2009 we studied 10 consecutive patients with LVNC diagnosed by echocardiogram and/or magnetic resonance imaging who underwent electrocardiogram-gated coronary CT angiography. We evaluated the ratio of noncompacted to compacted myocardium (NC/C ratio) in end diastole in each of the 17 segments established by the American Heart Association (excluding the apex). The results were compared with 9 healthy subjects, 14 patients with hypertrophic cardiomyopathy, and 17 patients with dilated cardiomyopathy to determine the cutoff that would distinguish patients with LVNC. RESULTS:When considering involvement of more than 1 segment, the NC/C ratio of 2.2 distinguished pathologic noncompaction, with sensitivity and specificity of 100% and 95%, respectively. In addition, the involvement of ≥2 segments allows the distinction of all patients with LVNC from other cardiomyopathies and from healthy subjects. CONCLUSIONS:LVNC can be accurately diagnosed with MDCT when using a cutoff NC/C ratio of 2.2 at end diastole involving ≥2 segments. 10.1016/j.jcct.2012.07.001
    Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction. Wan Junyi,Zhao Shihua,Cheng Huaibing,Lu Minjie,Jiang Shiliang,Yin Gang,Gao Xiaojin,Yang Yuejin Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Late gadolinium enhancement (LGE) is identified frequently in LVNC. However, the features of this findings are limited. The purpose of the present study was to describe the frequency and distribution of LGE in patients meeting criteria for left ventricular non-compaction (LVNC), as assessed by cardiovascular magnetic resonance (CMR). METHODS:Forty-seven patients (37 males and 10 females; mean age, 39 ± 18 years) considered to meet standard CMR criteria for LVNC were studied. The LGE images were obtained 15 ± 5 min after the injection of 0.2 mmol/kg of gadolinium-DTPA using an inversion-recovery sequence, and analyzed using a 17-segment model. RESULTS:Mean number of non-compacted segments per patient was 7.4 ± 2.5 and the NC:C was 3.2 ± 0.7. Non-compaction was most commonly noted in the apical segments in all patients. LGE was present in 19 of the 47 patients (40%), and most often located in the ventricular septum. The distribution of LGE was subendocardial (n = 5; 6%), mid-myocardial (n = 61; 68%), subepicardial (n = 10; 11%), and transmural (n = 14; 15%) in total of 90 LGE (+) segments. CONCLUSIONS:In patients considered to meet criteria for LVNC, LGE distributions visible were strikingly heterogeneous with appearances potentially attributable to three or more distinct cardiomyopathic processes. This may be in keeping with previous suggestions that the criteria may be of low specificity. Further work is needed to determine whether conditions such as dilated cardiomyopathy, previous myocardidtis or ischaemic heart disease increase the apparent depth of non-compact relative to compact myocardium. 10.1186/1532-429X-15-20
    Electrocardiographic findings in correlation to cardiac magnetic resonance imaging patterns for isolated ventricular non-compaction patients. Jayaraman Sivaraman Anatolian journal of cardiology 10.5152/akd.2015.15973
    Noncompaction cardiomyopathy and heterotaxy syndrome. Martinez Hugo R,Ware Stephanie M,Schamberger Marcus S,Parent John J Progress in pediatric cardiology Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female). Echocardiography and cardiac magnetic resonance imaging results were independently reviewed by two cardiologists to ensure reproducibility of LVNC. A total of 13/172 (7.5%) patients met imaging criteria for LVNC. The CHD identified in this subgroup included atrioventricular septal defects [11], dextrocardia [10], systemic and pulmonary venous return abnormalities [7], and transposition of the great arteries [5]. From this subgroup, 61% ( = 8) of the patients developed arrhythmias; and 61% ( = 8) required medical management for chronic heart failure. This study indicates that LVNC has increased prevalence among patients with heterotaxy when compared to the general population (0.014-1.3%) suggesting possible common genetic mechanisms. Interestingly, mice with a loss of function of or genes showed abnormal compaction of the ventricles, anomalies in cardiac looping, and septation defects in previous studies. Recognition of the association between LVNC and heterotaxy is important for various reasons. First, the increased risk of arrhythmias demonstrated in our population. Secondly, theoretical risk of thromboembolic events remains in any LVNC population. Finally, many patients with heterotaxy undergo cardiac surgery (corrective and palliative) and when this is associated with LVNC, patients should be presumed to incur a higher peri-operative morbidity based on previous studies. Further research will continue to determine long-term and to corroborate genetic pathways. 10.1016/j.ppedcard.2017.06.007
    Left Ventricular Noncompaction and Vigorous Physical Activity: What Is the Connection? Towbin Jeffrey A,Beasley Gary Journal of the American College of Cardiology 10.1016/j.jacc.2020.08.051
    Left ventricular trabeculae: quantification in different cardiac diseases and impact on left ventricular morphological and functional parameters assessed with cardiac magnetic resonance. Fernández-Golfín Covadonga,Pachón Marta,Corros Cecilia,Bustos Ana,Cabeza Beatriz,Ferreirós Joaquín,de Isla Leopoldo Pérez,Macaya Carlos,Zamorano José Journal of cardiovascular medicine (Hagerstown, Md.) OBJECTIVES:Left ventricle trabeculae (LVT) are frequently seen in different cardiac diseases. Normal reference values of LVT in different cardiac conditions are not known. The aim of the study was to quantify with cardiac magnetic resonance (CMR), LVT mass (LVTM) and LVTM percentage (LVTM%) in different heart diseases and to evaluate their influence on left ventricular morphological and functional parameters. METHODS:Fifty-nine patients (14 controls, 17 ischemic cardiomyopathy, 15 nonischemic dilated cardiomyopathy, 7 valvular heart disease and 6 with left ventricle hypertrophy) were enrolled. Cine-MR images were acquired with steady-state free-precession sequence in a short-axis view. LVTM was calculated as the difference between LVM excluding/including trabecuale from the blood cavity. LVTM% was calculated as the percentage of the whole left ventricle mass excluding trabeculae from the blood cavity. RESULTS:Mean age was 47.60 +/- 22.03 years; male 62.7%. Mean LVTM was of 33.38 +/- 16.1 g with mean LVTM% of 19.22 +/- 6.5%. Significant differences between groups for both parameters with P values of 0.02 were obtained. Nonischemic dilated cardiomyopathy showed the highest degree of LVTM (44.73 +/- 16.0 g) and LVTM% (23.26 +/- 6%). Significant differences were noted in left ventricular morphological and functional parameters with inclusion/exclusion of LVT in the myocardial mass. CONCLUSIONS:Reference values and differences of LVTM and LVTM% in various cardiac conditions are given for the first time. Quantification of these parameters with CMR may be clinically useful in the differential diagnosis between left ventricular noncompaction and other cardiac diseases. Exclusion of LVT from myocardium alters left ventricular morphological and functional parameters, which have significant clinical importance. 10.2459/JCM.0b013e32832e1c60
    Regional thicknesses and thickening of compacted and trabeculated myocardial layers of the normal left ventricle studied by cardiovascular magnetic resonance. Dawson Dana K,Maceira Alicia M,Raj Vimal J,Graham Catriona,Pennell Dudley J,Kilner Philip J Circulation. Cardiovascular imaging BACKGROUND:We used cardiovascular magnetic resonance (CMR) to study normal left ventricular (LV) trabeculation as a basis for differentiation from pathological noncompaction. METHODS AND RESULTS:The apparent end-diastolic (ED) and end-systolic (ES) thicknesses and thickening of trabeculated and compacted myocardial layers were measured in 120 volunteers using a consistent selection of basal, mid, and apical CMR short-axis slices. All had a visible trabeculated layer in 1 or more segments. The compacted but not the trabeculated layer was thicker in men than in women (P<0.01 at ED and ES). When plotted against age, the trabeculated and compacted layer thicknesses demonstrated opposite changes: an increase of the compact layer after the fourth decade at both ED and ES (P<0.05) but a decrease of the trabeculated layer. There was age-related preservation of total wall thickness at ED but an increase at ES (P<0.05). The compacted layer thickened, whereas the trabeculated layer thinned with systole, but neither change differed between sexes. With age, the most trabeculated LV segments showed significantly greater systolic thinning of trabeculated layers and, conversely, greater thickening of the compact segments (P<0.05). Total wall thickening is neither sex nor age dependent. There were no sex differences in the trabeculated/compacted ratio at ES or ED, but the ES trabeculated/compacted ratio was smaller in older (50 to 79 years) versus younger (20 to 49 years) groups (P<0.05). CONCLUSIONS:We demonstrated age- and sex-related morphometric differences in the apparent trabeculated and compacted layer thicknesses and systolic thinning of the visible trabeculated layer that contrasts with compacted myocardial wall thickening. 10.1161/CIRCIMAGING.110.960229
    Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR. Stacey R Brandon,Andersen Mousumi M,St Clair Mitchell,Hundley W Gregory,Thohan Vinay JACC. Cardiovascular imaging OBJECTIVES:This study used cardiac magnetic resonance (CMR) to compare standard criteria for left ventricular noncompaction (LVNC). BACKGROUND:LVNC as a distinct cardiomyopathy is supported by a growing number of publications. Echocardiographic and CMR criteria have been established to diagnosis LVNC but have led to concerns of diagnostic accuracy. METHODS:Trabeculation/possible LVNC by CMR was retrospectively observed in 122 consecutive cases. We compared the standard end-systolic noncompacted-to-compacted ratio (ESNCCR), end-diastolic noncompacted:compacted ratio (EDNCCR), and trabecular mass-to-total mass ratio (TMTMR) along with deaths, embolic events, congestive heart failure (CHF) readmissions, ventricular arrhythmias, myocardial thickening (MT), left ventricular ejection fraction (LVEF), 3-dimensional sphericity index (3DSi), and left ventricular end-diastolic volume index. Adjusting for age, race, sex, body surface area, diabetes mellitus, hypertension, hyperlipidemia, coronary artery disease, and CHF, logistic regression was used to compare combined events (death, CHF readmission, embolism, ventricular arrhythmia) between ESNCCR, EDNCCR, and TMTMR. Adjusting for same covariates except CHF, logistic regression was used to compare the odds of CHF for those who met criteria and those who did not. Using analysis of covariance, adjusted means for LVEF, MT, 3DSi, and left ventricular end-diastolic volume index were generated. RESULTS:ES criteria had a higher odds ratio (8.6; 95% confidence interval [CI]: 2.5 to 33) for combined events than ED criteria (1.8; 95% CI: 0.6 to 5.8) or TMTMR criteria (3.14; 95% CI: 1.09 to 10.2). The odds ratio of CHF for those who met ESNCCR criteria was 29.4 (95% CI: 6.6 to 125), but the odds ratio of CHF for those who met EDNCCR criteria was 3.3 (95% CI: 1.1 to 9.2). After adjustment, those who met criteria for noncompaction by ESNCCR had a lower LVEF and less MT than those who did not (p = 0.01 and p = 0.003, respectively), but there was no difference between those who met criteria for EDNCCR or the TMTMR criteria and those who did not. CONCLUSIONS:ES measures of LVNC have stronger associations with events, CHF, and systolic dysfunction than other measures. 10.1016/j.jcmg.2013.01.014
    Left ventricular noncompaction in Duchenne muscular dystrophy. Statile Christopher J,Taylor Michael D,Mazur Wojciech,Cripe Linda H,King Eileen,Pratt Jesse,Benson D Woodrow,Hor Kan N Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function. METHODS:Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment. RESULTS:LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36. CONCLUSION:The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling. 10.1186/1532-429X-15-67
    Characteristics of trabeculated myocardium burden in young and apparently healthy adults. Tizón-Marcos Helena,de la Paz Ricapito Maria,Pibarot Philippe,Bertrand Olivier,Bibeau Karine,Le Ven Florent,Sinha Swapnil,Engert James,Bédard Elisabeth,Pasian Sergio,Deschepper Christian,Larose Eric The American journal of cardiology Increased myocardial trabeculations define noncompaction cardiomyopathy (NCC). Imaging advancements have led to increasingly common identification of prominent trabeculations with unknown implications. We quantified and determined the impact of trabeculations' burden on cardiac function and stretch in a population of healthy young adults. One hundred adults aged 18 to 35 years (28±4 years, 55% women) without known cardiovascular disease were prospectively studied by cardiovascular magnetic resonance. Left ventricular (LV) volumes, segmental function, and ejection fraction (EF) and left atrial volumes were determined. Thickness and area of trabeculated (T) and dense (D) myocardium were measured for each standardized LV segment. N-terminal pro-brain natriuretic peptide (Nt-pro-BNP) was measured. Eighteen percent of the subjects had ≥1 positive traditional criteria for NCC, and 11% meet new proposed NCC cardiovascular magnetic resonance criteria. Trabeculated over dense myocardium ratio (T/D) ratios were uniformly greater at end-diastole versus end-systole (0.90±0.25 vs 0.42±0.13, p<0.0001), in women versus men (0.85±0.24 vs 0.72±0.19, p=0.006), at anterior versus nonanterior segments (1.41±0.59 vs 0.88±0.35, p<0.0001), and at apical versus nonapical segments (1.31±0.56 vs 0.87±0.38, p<0.0001). The largest T/D ratios were associated with lower LVEF (57.0±5.3 vs 62±5.5, p=0.0001) and greater Nt-pro-BNP (203±98 vs 155±103, p=0.04). Multivariable regression identified greater end-systolic T/D ratios as the strongest independent predictor of lower LVEF, beyond age and gender, left atrial or LV volumes, and Nt-pro-BNP (β=-9.9, 95% CI -15 to 4.9, p<0.001). In conclusion, healthy adults possess variable amounts of trabeculations that regularly meet criteria for NCC. Greater trabeculations are associated with decreased LV function. Apparently healthy young adults with increased trabecular burden possess evidence of mildly impaired cardiac function. 10.1016/j.amjcard.2014.07.025
    Mitral regurgitation in left ventricular noncompaction cardiomyopathy assessed by cardiac MRI. Stacey R Brandon,Haag Jason,Hall Michael E,McLeod George,Upadhya Bharathi,Hundley W Gregory,Thohan Vinay The Journal of heart valve disease BACKGROUND AND AIM OF THE STUDY:Previous case reports have described patients with left ventricular noncompaction cardiomyopathy (LVNC) with significant mitral regurgitation (MR). The study aim was to determine if LVNC is associated with MR, as assessed by cardiac magnetic resonance imaging (cMRI). METHODS:LVNC, assessed with cMRI, was observed retrospectively among 122 consecutive cases, 31 of whom had an end-systolic noncompacted-to-compacted ratio (ESNCCR) ≥ 2. In addition, 40 normal subjects undergoing cMRI and 40 with moderate to severe MR were included as controls. Using cine images, the ESNCCR and left ventricular (LV) and right ventricular (RV) stroke volumes were measured. The mitral regurgitant fraction (MRF) was calculated by dividing the difference between the RV and LV stroke volumes by the LV stroke volume. The total papillary muscle (TPM) area was measured from the mid short-axis view. Adjusting for age, race, gender and body surface area, an analysis of covariance was conducted to determine whether MRF and TPM were associated with ESNCCR ≥ 2. Adjusted means were presented with 95% confidence intervals. RESULTS:After adjustment, the MRF in patients with ESNCCR ≥ 2 was higher than in controls, but did not differ from that in patients with significant MR (21 ± 6.5% versus 2.7 ± 12% versus 29.2 ± 11%, p = 0.039 and p = 0.3, respectively). Further, patients with ESNCCR ≥ 2 had a lower TPM than normal controls or those with moderate to severe MR (1.89 ± 0.28 cm2 versus 3.6 ± 0.26 cm2 versus 3.7 ± 0.24 cm2; p < 0.001 and p < 0.001, respectively). CONCLUSION:LVNC is associated with increased MR, as assessed using cMRI. LVNC is also associated with abnormalities in the papillary muscle anatomy, which may predispose to MR.
    Left ventricular non-compaction in patients with single ventricle heart disease. Cardiology in the young OBJECTIVE:Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function. METHODS:Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data. RESULTS:The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71). CONCLUSIONS:Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function. 10.1017/S1047951119001872
    Speckle tracking echocardiography and left ventricular twist mechanics: predictive capabilities for noncompaction cardiomyopathy in the first degree relatives. Akhan Onur,Demir Emre,Dogdus Mustafa,Cakan Filiz Ozerkan,Nalbantgil Sanem The international journal of cardiovascular imaging In non-compaction cardiomyopathy (NCCM), there are several echocardiographic and cardiac magnetic resonance (CMR)-based quantitative diagnostic indices, current criteria mainly placed on morphological features, and none of the diagnostic indices includes left ventricular (LV) function. LV function and hemodynamics could be normal in NCCM patients. Evaluation of left ventricular function at the subclinical stage, strain echocardiographic parameters could be used alternative to standard echocardiographic examinations. The aim of this study to evaluate; NCCM patients, their first-degree relatives, ventricular motion patterns, strain characteristics, and the predictive capabilities of these features for early diagnosis of cardiomyopathy. This cross-sectional, case-control study included 32 NCCM patients, 30 first-degree relatives (father, mother, siblings and children) and 31 healthy volunteers. All patients evaluated with baseline echocardiography, strain measurements, and ventricular wall motion pattern. There were no differences between the groups in terms of age, weight, and body surface area. We observed a statistically significant decrease in ejection fraction (EF), fractional shortening (FS), E/E' and global strain values in patients' relatives compared to healthy volunteers (Patients' relatives: LVEF:60.9 ± 7.2%, FS:0.34 ± 0.07, E/E':7.51 ± 1.83, GLS: - 18.6 ± 3.6, GLSr: - 1.1 ± 0.1, GCS: - 17.1 ± 3.1, GCSr: - 1.2 ± 0.1, GRS:37.1 ± 6.2, GRSr:1.7 ± 0.1; all p values< 0.05). 'Rigid Body Rotation (RBR)' movement pattern was also observed in some of the patient's relative's like in the patients. RBR movement pattern determined patients; EF, longitudinal strain-strain rate, and basal layer rotation values were significantly lower, but radial strain values were higher with the RBR movement pattern (for all values p < 0.05). RBR movement pattern, deterioration of strain parameters, and accompanying echocardiographic features like LVEF, fractional shortening (FS), E/E' in patients' relative groups may contribute to reveal the subclinical status of disease and could be predictive for early diagnosis of cardiomyopathy. 10.1007/s10554-020-02008-y
    Higher spatial resolution improves the interpretation of the extent of ventricular trabeculation. Riekerk Hanne C E,Coolen Bram F,J Strijkers Gustav,van der Wal Allard C,Petersen Steffen E,Sheppard Mary N,Oostra Roelof-Jan,Christoffels Vincent M,Jensen Bjarke Journal of anatomy The ventricular walls of the human heart comprise an outer compact layer and an inner trabecular layer. In the context of an increased pre-test probability, diagnosis left ventricular noncompaction cardiomyopathy is given when the left ventricle is excessively trabeculated in volume (trabecular vol >25% of total LV wall volume) or thickness (trabecular/compact (T/C) >2.3). Here, we investigated whether higher spatial resolution affects the detection of trabeculation and thus the assessment of normal and excessively trabeculated wall morphology. First, we screened left ventricles in 1112 post-natal autopsy hearts. We identified five excessively trabeculated hearts and this low prevalence of excessive trabeculation is in agreement with pathology reports but contrasts the prevalence of approximately 10% of the population found by in vivo non-invasive imaging. Using macroscopy, histology and low- and high-resolution MRI, the five excessively trabeculated hearts were compared with six normal hearts and seven abnormally trabeculated and excessive trabeculation-negative hearts. Some abnormally trabeculated hearts could be considered excessively trabeculated macroscopically because of a trabecular outflow or an excessive number of trabeculations, but they were excessive trabeculation-negative when assessed with MRI-based measurements (T/C <2.3 and vol <25%). The number of detected trabeculations and T/C ratio were positively correlated with higher spatial resolution. Using measurements on high resolution MRI and with histological validation, we could not replicate the correlation between trabeculations of the left and right ventricle that has been previously reported. In conclusion, higher spatial resolution may affect the sensitivity of diagnostic measurements and in addition could allow for novel measurements such as counting of trabeculations. 10.1111/joa.13559
    MR imaging features of ventricular noncompaction: emphasis on distribution and pattern of fibrosis. Dursun Memduh,Agayev Ayaz,Nisli Kemal,Ertugrul Turkan,Onur Imran,Oflaz Huseyin,Yekeler Ensar European journal of radiology OBJECTIVE:The purpose of this study is to describe morphologic features and delayed contrast-enhancement pattern of the noncompaction of the left ventricle in cardiac magnetic resonance (MR) imaging. METHODS:We retrospectively reviewed morphological cardiac MR imaging findings of ventricular noncompaction in 15 patients (eight men, seven women, and ages 6 months to 73 years old, mean 22 year). In 10 patients delayed contrast enhanced images were obtained after the morphological examination. RESULTS:In all patients, noncompaction was seen in the apical and midventricular-lateral segment. Basal-septal segment involvement was not determined in any patients. Noncompacted/compacted ratio was 2-4.5 (mean 3). In nine patients, right ventricular involvement was observed in addition to left ventricular noncompaction. Delayed contrast-enhancement was seen in eight out of 10 patients not only involved segments but also normal segments of the heart. CONCLUSION:Cardiac MR imaging is a valuable imaging method in patients with suspected ventricular noncompaction by showing increased trabeculations, deep intertrabecular recesses and fibrosis. Fibrosis is a common finding in ventricular noncompaction. 10.1016/j.ejrad.2009.01.015
    Left ventricular noncompaction: a 25-year odyssey. Paterick Timothy E,Umland Matt M,Jan M Fuad,Ammar Khawaja Afzal,Kramer Christopher,Khandheria Bijoy K,Seward James B,Tajik A Jamil Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Left ventricular noncompaction (LVNC) is a cardiomyopathy associated with sporadic or familial disease, the latter having an autosomal dominant mode of transmission. The clinical features associated with LVNC vary from asymptomatic to symptomatic patients, with the potential for heart failure, supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. Echocardiography is the diagnostic modality of choice, revealing the pathognomonic features of a thick, bilayered myocardium; prominent ventricular trabeculations; and deep intertrabecular recesses. Widespread use and advances in the technology of echocardiography and cardiac magnetic resonance imaging are increasing awareness of LVNC, and cardiac magnetic resonance imaging is improving the ability to stage the severity of the disease and potential for adverse clinical consequences. Study of LVNC through research in embryology, imaging, and genetics has allowed enormous strides in the understanding of this heterogeneous disease over the past 25 years. 10.1016/j.echo.2011.12.023
    Left ventricular noncompaction associated with hypertrophic cardiomyopathy: echocardiographic diagnosis and genetic analysis of a new pedigree in China. Yuan Li,Xie Mingxing,Cheng Tsung O,Wang Xinfang,Zhu Feng,Kong Xiangquan,Ghoorah Devina International journal of cardiology BACKGROUND:Hypertrophic cardiomyopathy (HCM) and left ventricular noncompaction (LVNC) are both genetically determined and familial diseases that possess variable but overlapping genetic defects. Previous literature has mostly reported their occurrences as either separate disorders in different members of a family or coexisting entities in sporadic cases rather than familial cases. This study explored the echocardiographic diagnostic values and familial features in a family with coexistence of HCM and LVNC. METHODS:A four-generation family comprised of 30 members was studied; 28 members underwent familial screening by routine transthoracic echocardiography (TTE), contrast echocardiography (CE), and/or cardiac magnetic resonance imaging (cMRI). Echocardiographic and cMRI findings were then compared. RESULTS:Four members (13.3%) died of sudden death or heart failure. Eleven members (39%) suffered from HCM, LVNC or both. There were 13 left ventricular hypertrophic segments among the echocardiographic images of 9 locally archived patients, including septal, inferior and anterior wall segments (8, 3, 2 respectively) as well as 20 noncompaction segments, including lateral, apical, anterior, antero-septal and inferior wall segments (8, 5, 4, 2, 1 respectively). Left atrial dilatation and diastolic dysfunction were significant in these subjects. Findings from TTE and CE were in accordance with those from cMRI in lesion locations. CE provided more information about noncompaction segments located in the antero-septum and near field than TTE. CONCLUSIONS:HCM and LVNC coexist in one Chinese family, with overlapping phenotypes and different ages, clinical manifestations and multimodality imaging findings. TTE is an excellent tool to diagnose HCM and LVNC with supplementation by CE. 10.1016/j.ijcard.2014.03.006
    Quantification of left ventricular trabeculae using cardiovascular magnetic resonance for the diagnosis of left ventricular non-compaction: evaluation of trabecular volume and refined semi-quantitative criteria. Choi Yeonu,Kim Sung Mok,Lee Sang-Chol,Chang Sung-A,Jang Shin Yi,Choe Yeon Hyeon Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Left ventricular non-compaction (LVNC) is an unclassified cardiomyopathy and there is no consensus on the diagnosis of LVNC. The aims of this study were to establish quantitative methods to diagnose LVNC using cardiovascular magnetic resonance (CMR) and to suggest refined semi-quantitative methods to diagnose LVNC. METHODS:This retrospective study included 145 subjects with mild to severe trabeculation of the left ventricle myocardium [24 patients with isolated LVNC, 33 patients with non-isolated LVNC, 30 patients with dilated cardiomyopathy (DCM) with non-compaction (DCMNC), 27 patients with DCM, and 31 healthy control subjects with mild trabeculation]. The left ventricular (LV) ejection fraction, global LV myocardial volume, trabeculated LV myocardial volume, and number of segments with late gadolinium enhancement were measured. In addition, the most prominent non-compacted (NC), compacted (C), normal mid-septum, normal mid-lateral wall, and apical trabeculation thicknesses on the end-diastolic frames of the long-axis slices were measured. RESULTS:In the patients with isolated LVNC, the percentage of trabeculated LV volume (TV%, ​42.6 ± 13.8 %) ​relative to total LV myocardial volume was 1.4 times higher than in those with DCM (30.3 ± 14.3 %, p < 0.001), and 1.7 times higher than in the controls (24.8 ± 7.1 %, p < 0.001). However, there was no significant difference in TV% between the isolated LVNC and DCMNC groups (47.1 ± 17.3 % in the DCMNC group; p = 0.210). The receiver operating characteristic curve analysis using Jenni's method for CMR classification as the standard diagnostic criteria revealed that a value of TV% above 34.6 % was predictive of NC with a specificity of 89.7 % (CI: 74.2 - 98.0 %) and a sensitivity of 66.1 % (CI: 52.6 - 77.9 %). A value of NC/septum over 1.27 was considered predictive for NC with a specificity of 82.8 % (CI: 64.2 - 94.2 %) and a sensitivity of 57.6 % (CI: 44.1 - 70.4 %). In addition, a value of apex/C above 3.15 was considered predictive of NC with a specificity of 93.1 % (CI: 77.2 - 99.2 %) and a sensitivity of 69.5 % (CI: 56.1 - 80.8 %). CONCLUSIONS:A trabeculated LV myocardial volume above 35 % of the total LV myocardial volume is diagnostic for LVNC with high specificity. Also, the apex/C and NC/septum ratios could be useful as supplementary diagnostic criteria. 10.1186/s12968-016-0245-2
    Prolonged QTc indicates the clinical severity and poor prognosis in patients with isolated left ventricular non-compaction. Zhou Hongmei,Lin Xue,Fang Ligang,Zhu Wenlin,Zhao Xihai,Ding Haiyan,Jiang Meng,Ge Heng,Fang Quan,He Ben The international journal of cardiovascular imaging Isolated left ventricular non-compaction (LVNC) is a rare cardiomyopathy that leads to severe clinical complications. This study is to investigate whether or not prolonged QTc is a good indicator for evaluating the severity of fibrosis and predicting the prognosis of LVNC, and if native T1 can be used to quantify the fibrosis. 32 LVNC patients and 14 healthy controls with matched age and sex were examined by CMR and ECG to acquire native T1, QTc interval, and ECG abnormalities. 18 LVNC patients had normal QTc and 14 LVNC patients had prolonged QTc. The mean native T1 value of the normal controls, normal QTc and prolonged QTc patients was 1096.0 ± 41.5, 1141.98 ± 45.46, and 1182.67 ± 42.02 ms, respectively. One-way ANVOA showed significant differences in native T1 among three groups (F = 14.9, p < 0.001). In LVNC patients, the QTc interval significantly correlated with LVEF (p = 0.003, r = 0.51) and native T1 values (p = 0.015, R = -0.47). This suggests that prolonged QTc is associated with more severe compacted myocardial fibrosis, more cardiac dysfunction, and a poorer prognosis in LVNC patients. Follow-up data showed significant differences in adverse events between patients with normal QTc and patients with prolonged QTc (p = 0.036). Prolonged QTc interval leads to more severe compacted myocardial fibrosis, poorer cardiac dysfunction, and poorer prognosis in LVNC patients. 10.1007/s10554-017-1209-9
    Fractal analysis of left ventricular trabeculations is associated with impaired myocardial deformation in healthy Chinese. Cai Jiashen,Bryant Jennifer Ann,Le Thu-Thao,Su Boyang,de Marvao Antonio,O'Regan Declan P,Cook Stuart A,Chin Calvin Woon-Loong Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Left ventricular (LV) non-compaction (LVNC) is defined by extreme LV trabeculation, but is measured variably. Here we examined the relationship between quantitative measurement in LV trabeculation and myocardial deformation in health and disease and determined the clinical utility of semi-automated assessment of LV trabeculations. METHODS:Cardiovascular magnetic resonance (CMR) was performed in 180 healthy Singaporean Chinese (age 20-69 years; males, n = 91), using balanced steady state free precession cine imaging at 3T. The degree of LV trabeculation was assessed by fractal dimension (FD) as a robust measure of trabeculation complexity using a semi-automated technique. FD measures were determined in healthy men and women to derive normal reference ranges. Myocardial deformation was evaluated using feature tracking. We tested the utility of this algorithm and the normal ranges in 10 individuals with confirmed LVNC (non-compacted/compacted; NC/C ratio > 2.3 and ≥1 risk factor for LVNC) and 13 individuals with suspected disease (NC/C ratio > 2.3). RESULTS:Fractal analysis is a reproducible means of assessing LV trabeculation extent (intra-class correlation coefficient: intra-observer, 0.924, 95% CI [0.761-0.973]; inter-observer, 0.925, 95% CI [0.821-0.970]). The overall extent of LV trabeculation (global FD: 1.205 ± 0.031) was independently associated with increased indexed LV end-diastolic volume and mass (sβ = 0.35; p < 0.001 and sβ = 0.13; p < 0.01, respectively) after adjusting for age, sex and body mass index. Increased LV trabeculation was independently associated with reduced global circumferential strain (sβ = 0.17, p = 0.013) and global diastolic circumferential and radial strain rates (sβ = 0.25, p < 0.001 and sβ = -0.15, p = 0.049, respectively). Abnormally high FD was observed in all patients with a confirmed diagnosis of LVNC. Five out of 13 individuals with suspected LVNC had normal FD, despite NC/C > 2.3. CONCLUSION:This study defines the normal range of LV trabeculation in healthy Chinese that can be used to make or refute a diagnosis of LVNC using the fractal analysis tool, which we make freely available. We also show that increased myocardial trabeculation is associated with higher LV volumes, mass and reduced myocardial strain. 10.1186/s12968-017-0413-z
    Cardiovascular magnetic resonance based diagnosis of left ventricular non-compaction cardiomyopathy: impact of cine bSSFP strain analysis. Dreisbach John G,Mathur Shobhit,Houbois Christian P,Oechslin Erwin,Ross Heather,Hanneman Kate,Wintersperger Bernd J Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Investigation of the myocardial strain characteristics of the left ventricular non-compaction (LVNC) phenotype with cardiovascular magnetic resonance (CMR) feature tracking. METHODS:CMR cine balanced steady-state free precession data sets of 59 retrospectively identified LVNC phenotype patients (40 years, IQR: 28-50 years; 51% male) and 36 healthy subjects (39 years, IQR: 30-47 years; 44% male) were evaluated for LV volumes, systolic function and mass. Hypertrabeculation in patients and healthy subjects was evaluated against established CMR diagnostic criteria. Global circumferential strain (GCS), global radial strain (GRS) and global longitudinal strain (GLS) were evaluated with feature-tracking software. Subgroup analyses were performed in patients (n = 25) and healthy subjects (n = 34) with normal LV volumetrics, and with healthy subjects (n = 18) meeting at least one LVNC diagnostic criteria. RESULTS:All LVNC phenotype patients, as well as a significant proportion of healthy subjects, met morphology-based CMR diagnostic criteria: non-compacted (NC): compacted myocardial diameter ratio > 2.3 (100% vs. 19.4%), NC mass > 20% (100% vs. 44.4%) and > 25% (100% vs. 13.9%), and NC mass indexed to body surface area > 15 g/m (100% vs. 41.7%). LVNC phenotype patients demonstrated reduced GRS (26.4% vs. 37.1%; p < 0.001), GCS (- 16.5% vs. -20.5%; p < 0.001) and GLS (- 14.6% vs. -17.1%; p < 0.001) compared to healthy subjects, with statistically significant differences persisting on subgroup comparisons of LVNC phenotype patients with healthy subjects meeting diagnostic criteria. GCS also demonstrated independent and incremental diagnostic value beyond each of the morphology-based CMR diagnostic criteria. CONCLUSIONS:LVNC phenotype patients demonstrate impaired strain by CMR feature tracking, also present on comparison of subjects with normal LV volumetrics meeting diagnostic criteria. The high proportion of healthy subjects meeting morphology-based CMR diagnostic criteria emphasizes the important potential complementary diagnostic value of strain in differentiating LVNC from physiologic hypertrabeculation. 10.1186/s12968-020-0599-3
    Usefulness of Neuromuscular Co-morbidity, Left Bundle Branch Block, and Atrial Fibrillation to Predict the Long-Term Prognosis of Left Ventricular Hypertrabeculation/Noncompaction. Stöllberger Claudia,Hasun Matthias,Winkler-Dworak Maria,Finsterer Josef The American journal of cardiology The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is assessed controversially. LVHT is associated with other cardiac abnormalities and with neuromuscular disorders (NMD). Aim of the study was to assess cardiac and neurological findings as predictors of mortality rate in adult LVHT-patients. Included were patients with LVHT diagnosed between 1995 and 2019 in 1 echocardiographic laboratory. Patients underwent a baseline cardiologic examination and were invited for a neurological investigation. In January 2020, their survival status was assessed. End points were death or heart transplantation. LVHT was diagnosed by echocardiography in 310 patients (93 female, aged 53 ± 18 years) with a prevalence of 0.4%/year. A neurologic investigation was performed in 205 patients (67%). A specific NMD was found in 33 (16%), NMD of unknown etiology in 123 (60%) and the neurological investigation was normal in 49 (24%) patients. During follow-up of 84 ± 71 months, 59 patients received electronic devices, 105 patients died, and 6 underwent heart transplantation. The mortality was 4.7%/year, the rate of heart transplantation/death 5%/year. By multivariate analysis, the following parameters were identified to elevate the risk of mortality/heart transplantation: increased age (p = 0.005), inpatient (p = 0.001), presence of a specific NMD (p = 0.0312) or NMD of unknown etiology (p = 0.0365), atrial fibrillation (p = 0.0000), ventricular premature complexes (p = 0.0053), exertional dyspnea (p = 0.0023), left bundle branch block (p = 0.0201), and LVHT of the posterior wall (p = 0.0158). In conclusion, LVHT patients should be systematically investigated neurologically since neurological co-morbidity has a prognostic impact. 10.1016/j.amjcard.2020.04.040
    Prevalence of left ventricular hypertrabeculation/noncompaction among patients with congenital dyserythropoietic anemia Type 1 (CDA1). Abramovich-Yoffe Hadar,Shalev Aryeh,Barrett Orit,Shalev Hanna,Levitas Aviva International journal of cardiology BACKGROUND:Congenital dyserythropoietic anemia type 1 (CDA1) is a rare autosomal recessive disease characterized by macrocytic anemia, ineffective erythropoiesis, and secondary hemochromatosis. Left-ventricular noncompaction (LVNC) is a cardiomyopathy that is commonly attributed to intrauterine arrest of normal compaction during the endomyocardial morphogenesis. LV hypertrabeculation/noncompaction (LVHT/NC) morphology, however, might exist in various hemoglobinopathies. Our primary objective was to determine whether the pattern of LVHT/NC is more prevalent among patients with CDA1, in comparison to subjects without CDA1, and to find potential risk factors for LVHT/NC among these patients. Our secondary objective was to evaluate the clinical implication of LVHT/NC. METHODS:We retrospectively assessed 32 CDA1 patients (median age 17.5, range 6-61) that underwent routine assessment of iron overload by cardiac magnetic resonance. Number and distribution of noncompacted LV segments were assessed in CDA1 patients and compared to 64 age- and gender-matched patients without CDA1. The ratio of noncompacted to compacted myocardium (NC/C ratio) in end-diastole was calculated for each of the three long-axis views. NC/C ratio > 2.3 was considered diagnostic for LVHT/NC. RESULTS:In multivariate analysis, the presence of CDA1 was independently associated with NC/C ratio > 2.3, a feature of LVHT/NC (adjusted OR = 11.46, 95%CI = 2.6-50.68, p = .001). CDA1 was strongly associated with increased number of myocardial segments exhibiting LVHT/NC pattern. Cardiac volumes and ejection fraction were preserved without clinical adverse events in long term follow-up. CONCLUSIONS:CDA1 patients have a higher prevalence of LVHT/NC than normal individuals, independent of myocardial iron overload and without effect on ejection fraction or clinical outcome. 10.1016/j.ijcard.2020.05.092
    Long term clinical outcomes associated with CMR quantified isolated left ventricular non-compaction in adults. Femia Giuseppe,Zhu Danyi,Choudhary Preeti,Ross Samantha B,Muthurangu Vivek,Richmond David,Celermajer David S,Semsarian Christopher,Puranik Rajesh International journal of cardiology BACKGROUND:Left ventricular non-compaction (LVNC) is a complex clinical condition with several diagnostic criteria but no diagnostic gold standard. We aimed to evaluate our thresholding technique in a group of patients with LVNC and assess the risk of major adverse cardiovascular and cerebrovascular events (MACCE). METHODS:We retrospectively analyzed cardiac magnetic resonance (CMR) scans of patients with Petersen criteria LVNC and quantified noncompacted myocardial mass. We assessed the association of noncompacted myocardial mass, CMR derived LV volumetric parameters and late gadolinium enhancement (LGE) to MACCE including cardiac death, cardiac transplantation, sustained ventricular tachycardia/ventricular fibrillation (VT/VF) and ischemic stroke. Patients with known genetic mutations and cardiovascular disease were excluded. RESULTS:98 patients with LVNC were included (55 males,56.7%); 17(17.3%) patients had impaired LV function and five (5.1%) had LGE. Patients with impaired LV function had more end-systolic noncompacted mass (61.9 g±22.4 vs. 38.1 g±15.8, p < 0.001) and larger end-systolic noncompacted to total myocardial mass (44%±9 vs. 36%±12, p = 0.003). At 78 months follow-up [interquartile range(IQR) 66-90], MACCE occurred in 11(11.3%) patients; nine(81.8%) had impaired LV function and two(18.2%) had LGE. Impaired LV function and LV LGE were predictors of MACCE (HR = 35.6, 95% CI = 7.65-165.21, p < 0.001 and HR = 16.2, 95% CI = 4.54-57.84, p < 0.001) whereas noncompacted mass were not. CONCLUSION:Noncompacted mass was not an independent predictor of major adverse events but in patients with impaired LV function and/or LV LGE, the risk of MACCE was high. These results highlight the importance of including LV volumetrics and scar in the assessment of patients with LV noncompaction. 10.1016/j.ijcard.2020.12.017
    The prognostic role of CMR using global planimetric criteria in patients with excessive left ventricular trabeculation. Macaione Francesca,Meloni Antonella,Positano Vincenzo,Barison Andrea,Todiere Giancarlo,Pistoia Laura,Di Lisi Daniela,Novo Giuseppina,Novo Salvatore,Pepe Alessia European radiology OBJECTIVES:Although cardiovascular magnetic resonance (CMR) is widely used in the assessment of left ventricular non-compaction (LVNC), there are no universally accepted diagnostic criteria and limited data regarding their prognostic value. We assessed the long-term prognostic role of the planimetric global Grothoff's criteria and of the CMR findings in predicting adverse cardiovascular events (CE). METHODS:We prospectively enrolled 78 patients (46.7 ± 18.7 years, 33.3% females) with documented positive Jenni's echocardiographic criteria for LVNC. Cine images were used to quantify function parameters and to assess for the presence of all four quantitative Grothoff's criteria (global Grothoff's criteria). Late gadolinium enhancement (LGE) images were acquired to detect the presence of replacement myocardial fibrosis. RESULTS:Petersen's CMR criterion for LVNC (NC/C ratio > 2.3 in at least one myocardial segment) was fulfilled in the whole population. Twenty-six patients fulfilled the global Grothoff's criteria (four out of four). The mean duration of the follow-up was 44.2 ± 27.4 months and 28 CE were registered: 10 ventricular tachycardias, 12 episodes of heart failure (HF), four strokes, and two cardiac deaths. In the multivariate analysis, the independent predictive factors for CE were positive global Grothoff's criteria (hazard ratio, HR = 3.33, 95% CI = 1.52-7.29; p = 0.003) and myocardial fibrosis (HR = 2.41, 95% CI = 1.08-5.36; p = 0.032). CONCLUSIONS:Positive global Grothoff's criteria and myocardial fibrosis were powerful predictors of CE in patients with a diagnosis of LVNC by CMR Petersen's criterion. Thus, we strongly suggest a step approach confirming the diagnosis of LVNC by using the global planimetric Grothoff's criteria, which showed a prognostic impact. KEY POINTS:• Positive global Grothoff's criteria and replacement myocardial fibrosis were powerful predictors of cardiovascular events in patients with a diagnosis of LVNC by CMR Petersen's criterion. • Positive global Grothoff's criteria were associated with a higher frequency of ventricular arrhythmias in patients with a diagnosis of LVNC by CMR Petersen's criterion. 10.1007/s00330-021-07875-0
    Left ventricular basal region involvement in noncompaction cardiomyopathy. de Melo Marcelo Dantas Tavares,Benvenuti Luiz A,Mady Charles,Kalil-Filho Roberto,Salemi Vera M C Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology A previously healthy 16-year-old woman experienced progressive dyspnea on exertion. The echocardiogram and cardiac magnetic resonance imaging showed a significant increase in cardiac chambers, severe biventricular systolic dysfunction, and prominent ventricular trabeculations suggesting noncompaction cardiomyopathy (NCC). The patient underwent heart transplantation 5 years after the NCC diagnosis, and the anatomopathological examination evidenced diffuse biventricular hypertrabeculation compromise, including the basal region of the biventricular wall. There is no consensus about the gold-standard diagnostic criteria, which demands a conceptual review and attention to another point: the relation of trabeculation volume and prognosis. 10.1016/j.carpath.2013.05.001
    Right ventricular morphology and systolic function in left ventricular noncompaction cardiomyopathy. Stacey Richard Brandon,Andersen Mousumi,Haag Jason,Hall Michael E,McLeod George,Upadhya Bharathi,Hundley William Gregory,Thohan Vinay The American journal of cardiology The distinction between normal right ventricular (RV) trabeculations from abnormal has been difficult. We evaluated whether RV volume and function are related to left ventricular (LV) noncompaction (NC) cardiomyopathy and clinical events. Trabeculations or possible LVNC by cardiac magnetic resonance imaging was retrospectively observed among 105 consecutive cases. We measured LV end-systolic (ES) noncompacted-to-compacted ratio, RV ejection fraction (EF), RV apical trabecular thickness, and RV end-diastolic (ED) noncompacted-to-compacted ratio. A control group of 40 subjects was also reviewed to assess the exploratory measures. Comparing those with LVES noncompacted-to-compacted ratio ≥2, those with LVES noncompacted-to-compacted ratio <2, and the normal control group, adjusted means for RV apical trabecular thickness and RVED noncompacted-to-compacted ratio were generated. Logistic regression was used to evaluate the association of composite events traditionally associated with LVNC with RVEF after adjustment for aforementioned covariates, cardiovascular risk factors, delayed enhancement, LVEF, and LVES noncompacted-to-compacted ratio. Analysis of RV morphology found greater apical trabecular thickness among those with LVES noncompacted-to-compacted ratio ≥2 compared with those with LVES noncompacted-to-compacted ratio <2 or normal control group (31 ± 5 vs 27 ± 2.6 vs 22 ± 4 mm; p = 0.03 and p = 0.003, respectively). There was no difference between the groups in relation to the RVED noncompacted-to-compacted ratio. Low RVEF and LVES noncompacted-to-compacted ratio ≥2 had significant association with clinical events in this population even after adjusting for clinical and imaging parameters (p = 0.04 and p <0.001, respectively). In conclusion, RV dysfunction in a morphologic LVNC population is strongly associated with adverse clinical events. LVNC is associated with increased trabeculations of the RV apex. 10.1016/j.amjcard.2013.12.008
    Cardiovascular magnetic resonance determinants of left ventricular noncompaction. Dawson Dana K,McLernon David J,Raj Vimal J,Maceira Alicia M,Prasad Sanjay,Frenneaux Michael P,Pennell Dudley J,Kilner Philip J The American journal of cardiology Insufficient precision remains in accurately identifying left ventricular noncompaction (LVNC) from the healthy normal morphologic spectrum. We aim to provide a better distinction between normal left ventricular trabeculations and LVNC. We used a previously well-defined cohort of 120 healthy volunteers for normal reference values of the trabecular/compacted ratio derived from a consistent selection of short-axis cardiovascular magnetic resonance images. We performed forward selection of logistic regression models, selecting the best model that was subsequently assessed for discrimination and calibration, validated, and converted into a clinical diagnostic chart to benchmark the boundaries of detection from a cohort of 30 patients considered to have LVNC. We showed that 3 combinations of a maximal end-diastolic trabecular/compacted ratio (≥1 [apex], >1.8 [midcavity]), (>2 [apex], ≥0.6 [midcavity]), or (>0.5 [base], >1.8 [midcavity]) separate the cohorts with the highest accuracy (C statistic [95% confidence interval] of 0.9749 (0.9748 to 0.9751) for the diagnostic chart). Quantitative cardiovascular magnetic resonance also shows that patients considered to have LVNC have a significantly reduced ejection fraction compared with normal volunteers. At midcavity and apical level, it is difficult to identify papillary muscles that are replaced by a dense trabecular meshwork. In conclusion, we developed a new, refined, diagnostic tool for identifying LVNC, based on an a priori assessment of the trabecular architecture in healthy volunteers. 10.1016/j.amjcard.2014.05.017
    Left Ventricular Twist Mechanics to Identify Left Ventricular Noncompaction in Childhood. Sabatino Jolanda,Di Salvo Giovanni,Krupickova Sylvia,Fraisse Alain,Prota Costantina,Bucciarelli Valentina,Josen Manjit,Paredes Josefa,Sirico Domenico,Voges Inga,Indolfi Ciro,Prasad Sanjay,Daubeney Piers Circulation. Cardiovascular imaging BACKGROUND:Left ventricular noncompaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood. Standard diagnostic criteria are still controversial, especially in young patients. Recent studies in adults demonstrated that left ventricular (LV) twist is abnormal in LVNC, but it has not been investigated in pediatric patients to date. Our aim was to assess LV cardiac mechanics, LV twist, and the prevalence of rigid body rotation, using 2-dimensional speckle tracking echocardiography, in young patients with LVNC and LV hypertrabeculation. METHODS:Forty-seven children (age range: 0-18 years) were assessed for suspected LVNC. All patients underwent 2-dimensional speckle tracking echocardiography and cardiovascular magnetic resonance imaging at 1.5 Tesla (T). Twenty-three patients fulfilled the cardiovascular magnetic resonance imaging diagnostic criteria for LVNC (LVNC group), while the remaining 24 did not and were included in the LV hypertrabeculation group. Forty-seven age- and sex-matched healthy volunteers were used as controls. RESULTS:The average LV twist was significantly reduced in LVNC compared with control and LV hypertrabeculation. Rigid body rotation was recognized in 13 (56%) children with LVNC and in 1 (4%) child with LV hypertrabeculation and a strong family history for LVNC. Multivariable analysis demonstrated that LV twist is an independent predictor of LVNC ( P=0.006; coefficient=0.462). The receiver operating characteristics curve showed that LV twist had optimal predictive value to discriminate patients with LVNC (cutoff value <5.8°; sensitivity, 82%; specificity, 92%; area under the curve=0.914). CONCLUSIONS:LV twist has good predictive value in diagnosing LVNC in young patients. Our findings strongly support the routine use of 2-dimensional speckle tracking echocardiography in the evaluation of young patients with suspected LVNC. 10.1161/CIRCIMAGING.118.007805
    Improving the diagnosis of LV non-compaction with cardiac magnetic resonance imaging. Choudhary P,Hsu C J,Grieve S,Smillie C,Singarayar S,Semsarian C,Richmond D,Muthurangu V,Celermajer D S,Puranik R International journal of cardiology BACKGROUND:Current diagnostic criteria for left ventricular non-compaction (LVNC) poorly correlate with clinical outcomes. We aimed to develop a cardiac magnetic resonance (CMR) based semi-automated technique for quantification of non-compacted (NC) and compacted (C) masses and to ascertain their relationships to global and regional LV function. METHODS:We analysed CMR data from 30 adults with isolated LVNC and 20 controls. NC and C masses were measured using relative signal intensities of myocardium and blood pool. Global and regional LVNC masses was calculated and correlated with both global and regional LV systolic function as well as occurrence of arrhythmia. RESULTS:LVNC patients had significantly higher end-systolic (ES) and end-diastolic (ED) NC:C ratios compared to controls (ES 0.21 [SD 0.09] vs. 0.12 [SD 0.02], p<0.001; ED 0.39 [SD 0.08] vs. 0.26 [SD 0.05], p<0.001). NC:C ratios correlated inversely with global ejection fraction, with a stronger correlation in ES vs. ED (r=-0.58, p<0.001 vs. r=-0.30, p=0.03). ES basal, mid and apical NC:C ratios also showed a significant inverse correlation with global LV ejection fraction (ES basal r=-0.29, p=0.04; mid-ventricular r=-0.50, p<0.001 and apical r=-0.71, p<0.001). Upon ROC testing, an ES NC:C ratio of 0.16 had a sensitivity of 70% and a specificity of 95% for detection of significant LVNC. Patients with sustained ventricular tachycardia had a significantly higher ES NC:C ratio (0.31 [SD 0.18] vs. 0.20 [SD 0.06], p=0.02). CONCLUSIONS:The NC:C ratio derived from relative signal intensities of myocardium and blood pool improves the ability to detect clinically relevant NC compared to previous CMR techniques. 10.1016/j.ijcard.2014.12.053
    Comparison of cardiovascular magnetic resonance characteristics and clinical consequences in children and adolescents with isolated left ventricular non-compaction with and without late gadolinium enhancement. Cheng Huaibing,Lu Minjie,Hou Cuihong,Chen Xuhua,Li Li,Wang Jing,Yin Gang,Chen Xiuyu,Xiangli Wei,Cui Chen,Chu Jianmin,Zhang Shu,Prasad Sanjay K,Pu Jielin,Zhao Shihua Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Although cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to CMR is conducted in children with LVNC. This study was performed to characterize and compare CMR features and clinical outcomes in children with LVNC with and without late gadolinium enhancement (LGE). METHODS:A cohort of 40 consecutive children (age, 13.7 ± 3.3 years; 29 boys and 11 girls) with isolated LVNC underwent a baseline CMR scan with subsequent clinical follow-up. Short-axis cine images were used to calculate left ventricular (LV) ejection fraction (EF), end-diastolic volume (EDV), end-systolic volume (ESV), myocardial mass, ratio of non-compacted-to-compacted myocardial thickness (NC/C ratio), and number of non-compacted segments. The LGE images were analyzed to assess visually presence and patterns of LGE. The primary end point was a composite of cardiac death and heart transplantation. RESULTS:The LGE was present in 10 (25%) children, and 46 (27%) segments were involved, including 23 non-compacted segments and 23 normal segments. Compared with LGE- cohort, LGE+ cohort had significantly lower LVEF (23.8 ± 10.7% vs. 42.9 ± 16.7%, p < 0.001) and greater LVEDV (169.2 ± 65.1 vs. 118.2 ± 48.9 mL/m2, p = 0.010), LVESV (131.3 ± 55.5 vs. 73.3 ± 46.7 mL/m2, p = 0.002), and sphericity indices (0.75 ± 0.19 vs. 0.60 ± 0.20, p = 0.045). There were no differences in terms of number and distribution of non-compacted segments, NC/C ratio, and myocardial mass index between LGE+ and LGE- cohort. In the LGE+ cohort, adverse events occurred in 6 patients compared to 2 events in the LGE- cohort. Kaplan-Meier analysis showed a significant difference in outcome between LGE+ and LGE- cohort for cardiac death and heart transplantation (p = 0.011). CONCLUSIONS:The LGE was present in up to one-fourth of children with LVNC, and the LGE+ children exhibited a more maladaptive LV remodeling and a higher incidence of cardiovascular death and heart transplantation. 10.1186/s12968-015-0148-7
    Masking and Unmasking of Isolated Noncompaction of the Left Ventricle With Real-Time Contrast Echocardiography. Aggarwal Sourabh,Xie Feng,Porter Thomas R Circulation. Cardiovascular imaging 10.1161/CIRCIMAGING.117.006999
    Semi-automatic detection of myocardial trabeculation using cardiovascular magnetic resonance: correlation with histology and reproducibility in a mouse model of non-compaction. Frandon Julien,Bricq Stéphanie,Bentatou Zakarya,Marcadet Laetitia,Barral Pierre Antoine,Finas Mathieu,Fagret Daniel,Kober Frank,Habib Gilbert,Bernard Monique,Lalande Alain,Miquerol Lucile,Jacquier Alexis Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:The definition of left ventricular (LV) non-compaction is controversial, and discriminating between normal and excessive LV trabeculation remains challenging. Our goal was to quantify LV trabeculation on cardiovascular magnetic resonance (CMR) images in a genetic mouse model of non-compaction using a dedicated semi-automatic software package and to compare our results to the histology used as a gold standard. METHODS:Adult mice with ventricular non-compaction were generated by conditional trabecular deletion of Nkx2-5. Thirteen mice (5 controls, 8 Nkx2-5 mutants) were included in the study. Cine CMR series were acquired in the mid LV short axis plane (resolution 0.086 × 0.086x1mm) (11.75 T). In a sub set of 6 mice, 5 to 7 cine CMR were acquired in LV short axis to cover the whole LV with a lower resolution (0.172 × 0.172x1mm3). We used semi-automatic software to quantify the compacted mass (M), the trabeculated mass (M) and the percentage of trabeculation (M/M) on all cine acquisitions After CMR all hearts were sliced along the short axis and stained with eosin, and histological LV contouring was performed manually, blinded from the CMR results, and M, M and M/M were quantified. Intra and interobserver reproducibility was evaluated by computing the intra class correlation coefficient (ICC). RESULTS:Whole heart acquisition showed no statistical significant difference between trabeculation measured at the basal, midventricular and apical parts of the LV. On the mid-LV cine CMR slice, the median M was 0.92 mg (range 0.07-2.56 mg), M was 12.24 mg (9.58-17.51 mg), M/M was 6.74% (0.66-17.33%). There was a strong correlation between CMR and the histology for M, M and M/ M with respectively: r = 0.94 (p < 0.001), r = 0.91 (p < 0.001), r = 0.83 (p < 0.001). Intra- and interobserver reproducibility was 0.97 and 0.8 for M; 0.98 and 0.97 for M; 0.96 and 0.72 for M/M, respectively and significantly more trabeculation was observed in the M Mutant mice than the controls. CONCLUSION:The proposed semi-automatic quantification software is accurate in comparison to the histology and reproducible in evaluating M, M and M/ M on cine CMR. 10.1186/s12968-018-0489-0
    Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. Nucifora Gaetano,Aquaro Giovanni D,Masci Pier Giorgio,Pingitore Alessandro,Lombardi Massimo The American journal of cardiology The aim of the present study was to evaluate the prevalence and correlates of right ventricular (RV) noncompaction (RVNC), RV systolic dysfunction, and RV myocardial fibrosis in patients with isolated left ventricular (LV) noncompaction (LVNC). For this purpose, cine and contrast-enhanced cardiac magnetic resonance imaging (MRI) was used. A total of 56 consecutive patients with isolated LVNC were included in the study. The diagnosis of isolated LVNC was based on the presence of standard cardiac MRI and clinical criteria. For each patient, cine and contrast-enhanced cardiac MR images were analyzed to evaluate the prevalence and correlates of RVNC, RV dysfunction, and late gadolinium enhancement (a surrogate of myocardial fibrosis) involving the RV. Mean age of the patient population was 45 ± 19 years; 35 patients (63%) were men. RVNC was observed in 5 patients (9%). Impaired RV systolic function was observed in 9 patients (16%). Late gadolinium enhancement was not observed in any RV segment. No association was found between wall motion abnormalities and noncompaction at RV segmental level (φ coefficient 0.041, p = 0.26). At multivariate analysis, LV ejection fraction was the only variable independently related to RV ejection fraction (β = 0.62, p <0.001). In conclusion, RV systolic dysfunction is present in a non-negligible proportion of patients with isolated LVNC; LV systolic function is the only variable independently related to RV systolic function. 10.1016/j.amjcard.2013.08.049
    Cardiac magnetic resonance evaluation of left ventricular functional, morphological, and structural features in children and adolescents vs. young adults with isolated left ventricular non-compaction. Nucifora Gaetano,Sree Raman Karthigesh,Muser Daniele,Shah Ranjit,Perry Rebecca,Awang Ramli Kama A,Selvanayagam Joseph B International journal of cardiology AIM:To investigate the left ventricular (LV) functional, morphological, and structural features revealed by cardiac magnetic resonance (CMR) in children/adolescents with isolated LV non-compaction (iLVNC), and to compare them with those observed in young adults with iLVNC and healthy controls. METHODS:56 subjects were included: 12 children/adolescents (mean age 15±3years, 75% male) and 20 young adults (mean age 35±7years, 75% male) with first diagnosis of iLVNC, 12 healthy children/adolescents (mean age 15±3years, 75% male) and 12 healthy young adults (mean age 34±8years, 75% male). CMR with late gadolinium enhancement (LGE) imaging was performed to evaluate LV function, extent of LV trabeculation, and presence/extent of LV LGE, a surrogate of myocardial fibrosis. Tissue-tracking analysis was applied to assess LV global longitudinal (GLS), circumferential (GCS) and radial (GRS) strain. RESULTS:The extent of LVNC and the presence/extent of LV LGE in children/adolescents and young adults with iLVNC were similar. Compared to healthy subjects, young adults with iLVNC had significantly lower LVEF; conversely, no significant difference in this parameter was observed between children/adolescents with iLVNC and healthy subjects. However, compared to healthy subjects, LV strain parameters were lower in both children/adolescents and young adults with iLVNC. CONCLUSIONS:Complete phenotypic expression, subclinical impairment of myocardial deformation properties, and cardiac injury occur early in iLVNC patients, being already noticeable in the pediatric age group. The application of CMR myocardial deformation imaging permits earlier detection of LV functional impairment in children/adolescents with iLVNC, which would otherwise be missed with standard CMR imaging. 10.1016/j.ijcard.2017.05.100
    Supplementary Diagnostic Landmarks of Left Ventricular Non-Compaction on Magnetic Resonance Imaging. Boban Marko,Pesa Vladimir,Beck Natko,Manola Sime,Zulj Marinko,Rotim Ante,Vcev Aleksandar Yonsei medical journal PURPOSE:Diagnostic criteria for left ventricular non-compaction (LVNC) are still a matter of dispute. The aim of our present study was to test the diagnostic value of two novel diagnostic cardiac magnetic resonance (CMR) parameters: proof of non-compact (NC) myocardium blood flow using T2 sequences and changes in geometry of the left ventricle. MATERIALS AND METHODS:The study included cases with LVNC and controls, from a data base formed in a period of 3.5 years (n=1890 exams), in which CMR protocol included T2 sequences. Measurement of perpendicular maximal and minimal end diastolic dimensions in the region with NC myocardium from short axis plane was recorded, and calculated as a ratio (MaxMinEDDR), while flow through trabecula was proven by intracavital T2-weighted hyperintensity (ICT2HI). LVNC diagnosis met the following three criteria: thickening of compact (C) layer, NC:C>2.3:1 and NC>20%LV. RESULTS:The study included 200 patients; 71 with LVNC (35.5%; i.e., 3.76% of CMRs) and 129 (64.5%) controls. MaxMinEDDR in patients with LVNC was significantly different from that in controls (1.17±0.08 vs. 1.06±0.04, respectively; p<0.001). MaxMinEDDR >1.10 had sensitivity of 91.6% [95% confidence intervals (CI) 82.5-96.8], specificity of 85.3% (95% CI 78.0-90.0), and area under curve (AUC) 0.919 (95% CI 0.872-0.953; p<0.001) for LVNC. Existence of ICT2HI had sensitivity of 100.0% (95% CI 94.9-100.0), specificity of 91.5% (95% CI 85.3-95.7), and AUC 0.957 (95% CI 0.919-0.981; p<0.001) for LVNC. CONCLUSION:Two additional diagnostic parameters for LVNC were identified in this study. ICT2HI and geometric eccentricity of the ventricle both had relatively high sensitivity and specificity for diagnosing LVNC. 10.3349/ymj.2018.59.1.63
    Left ventricular noncompaction in pediatric population: could cardiovascular magnetic resonance derived fractal analysis aid diagnosis? Krupickova Sylvia,Hatipoglu Suzan,DiSalvo Giovanni,Voges Inga,Redfearn Daniel,Foldvari Sandrine,Eichhorn Christian,Chivers Sian,Puricelli Filippo,Delle-Donne Grazia,Barth Courtney,Pennell Dudley J,Prasad Sanjay K,Daubeney Piers E F Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance BACKGROUND:Cardiovascular magnetic resonance (CMR) derived fractal analysis of the left ventricle (LV) has been shown in adults to be a useful quantitative measure of trabeculation with high reproducibility and accuracy for the diagnosis of LV non-compaction (LVNC). The aim of this study was to investigate the utility and feasibility of fractal analysis in children. METHODS:Eighty-four subjects underwent CMR: (1) 28 patients with LVNC (as defined by the Petersen criteria with NC/C ratio [Formula: see text] 2.3); (2) 28 patients referred by clinicians for assessment of hyper-trabeculation and found not to qualify as LVNC (NC/C [Formula: see text] 1.8 and < 2.3); (3) 28 controls. The fractal scores for each group were presented as global and maximal fractal dimension as well as for 3 segments of the LV: basal, mid, and apical. Statistical comparison of the fractal scores between the 3 groups was performed. RESULTS:Global fractal dimension (FD) was higher in the LVNC group than in the hyper-trabeculated group: 1.345 (SEM 0.053) vs 1.252 (SEM 0.034), p < 0.001 and higher in hyper-trabeculated group than in controls: 1.252 (SEM 0.034) vs 1.158 (SEM 0.038), p < 0.001. The highest maximum FD was in the apical portion of the LV in the LVNC group, (1.467; SEM 0.035) whereas it was in the mid ventricle in the hyper-trabeculated (1.327; SEM 0.025) and healthy groups (1.251; SEM 0.042). Fractal analysis showed lower intra- and interobserver variability than the Petersen and Jacquier methods. CONCLUSIONS:It is technically feasible to perform fractal analysis in children using CMR and that it is quick, accurate and reproducible. Fractal scoring accurately distinguishes between LVNC, hyper-trabeculation and healthy controls as defined by the Petersen criteria. 10.1186/s12968-021-00778-5
    Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction. Casas Guillem,Limeres Javier,Oristrell Gerard,Gutierrez-Garcia Laura,Andreini Daniele,Borregan Mar,Larrañaga-Moreira Jose M,Lopez-Sainz Angela,Codina-Solà Marta,Teixido-Tura Gisela,Sorolla-Romero José Antonio,Fernández-Álvarez Paula,González-Carrillo Josefa,Guala Andrea,La Mura Lucia,Soler-Fernández Rafaela,Sao Avilés Augusto,Santos-Mateo Juan José,Marsal Josep Ramon,Ribera Aida,de la Pompa José Luis,Villacorta Eduardo,Jiménez-Jáimez Juan,Ripoll-Vera Tomás,Bayes-Genis Antoni,Garcia-Pinilla José Manuel,Palomino-Doza Julián,Tiron Coloma,Pontone Gianluca,Bogaert Jan,Aquaro Giovanni D,Gimeno-Blanes Juan Ramon,Zorio Esther,Garcia-Pavia Pablo,Barriales-Villa Roberto,Evangelista Artur,Masci Pier Giorgio,Ferreira-González Ignacio,Rodríguez-Palomares José F Journal of the American College of Cardiology BACKGROUND:Left ventricular noncompaction (LVNC) is a heterogeneous entity with uncertain prognosis. OBJECTIVES:This study sought to develop and validate a prediction model of major adverse cardiovascular events (MACE) and to identify LVNC cases without events during long-term follow-up. METHODS:This is a retrospective longitudinal multicenter cohort study of consecutive patients fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance. MACE were defined as heart failure (HF), ventricular arrhythmias (VAs), systemic embolisms, or all-cause mortality. RESULTS:A total of 585 patients were included (45 ± 20 years of age, 57% male). LV ejection fraction (LVEF) was 48% ± 17%, and 18% presented late gadolinium enhancement (LGE). After a median follow-up of 5.1 years, MACE occurred in 223 (38%) patients: HF in 110 (19%), VAs in 87 (15%), systemic embolisms in 18 (3%), and 34 (6%) died. LVEF was the main variable independently associated with MACE (P < 0.05). LGE was associated with HF and VAs in patients with LVEF >35% (P < 0.05). A prediction model of MACE was developed using Cox regression, composed by age, sex, electrocardiography, cardiovascular risk factors, LVEF, and family aggregation. C-index was 0.72 (95% confidence interval: 0.67-0.75) in the derivation cohort and 0.72 (95% confidence interval: 0.71-0.73) in an external validation cohort. Patients with no electrocardiogram abnormalities, LVEF ≥50%, no LGE, and negative family screening presented no MACE at follow-up. CONCLUSIONS:LVNC is associated with an increased risk of heart failure and ventricular arrhythmias. LVEF is the variable most strongly associated with MACE; however, LGE confers additional risk in patients without severe systolic dysfunction. A risk prediction model is developed and validated to guide management. 10.1016/j.jacc.2021.06.016
    Noncompaction of the myocardium the value of cardiovascular magnetic resonance imaging. Schuster Andreas,Duckett Simon G,Hedström Erik,Chiribiri Amedeo,Techen Georg,Nagel Eike Journal of the American College of Cardiology 10.1016/j.jacc.2011.01.075
    Association of left ventricular noncompaction with polycystic kidney disease as shown by cardiac magnetic resonance imaging. Katukuri Neelima Penugonda,Finger John,Vaitkevicius Peter,Riba Arthur,Spears James Richard Texas Heart Institute journal 10.14503/THIJ-13-3868
    The planimetric Grothoff's criteria by cardiac magnetic resonance can improve the specificity of left ventricular non-compaction diagnosis in thalassemia intermedia. Macaione Francesca,Meloni Antonella,Positano Vincenzo,Pistoia Laura,Barison Andrea,Di Lisi Daniele,Spasiano Anna,Campisi Saveria,Spiga Alessandra,Righi Riccardo,Novo Giuseppina,Novo Salvatore,Pepe Alessia The international journal of cardiovascular imaging We differentiated the left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to a negative remodeling in thalassemia intermedia (TI) patients applying linear and planimetric criteria and comparing the cardiovascular magnetic resonance (CMR) findings. CMR images were analyzed in 181 TI patients enrolled in the Myocardial Iron Overload in Thalassemia Network and 27 patients with proved LVNC diagnosis. The CMR diagnostic criteria applied in TI patients were: a modified linear CMR Petersen's criterion based on a more restrictive ratio of diastolic NC/C > 2.5 at segmental level and the combination of planimetric Grothoff's criteria (percentage of trabeculated LV myocardial mass LV-MM ≥ 25% of global LV mass and total LV-MMI NC ≥ 15 g/m). Seventeen TI patients showed at least one positive NC/C segment. Compared to LVNC patients, these patients showed a lower frequency of segments with non-compaction areas (2.41 ± 1.33 vs 5.48 ± 2.26; P < 0.0001), significantly lower LV-MM NC percentage (10.99 ± 4.09 vs 28.20 ± 4.27%; P < 0.0001), LV-MMI (7.58 ± 4.86 vs 19.88 ± 5.02 g/m; P < 0.0001) and extension of macroscopic fibrosis (0.44 ± 0.18 vs 4.65 ± 2.89; P = 0.004), and significantly higher LV ejection fraction (61.29 ± 5.17 vs 48.50 ± 17.55%; P = 0.016) and cardiac index (4.80 ± 1.49 vs 3.46 ± 1.11 l/min/m; P = 0.002). No TI patient fulfilled the Grothoff's criteria. All TI patients with an NC/C ratio > 2.5 showed morphological and functional CMR parameters significantly different from the patients with a proved diagnosis of LVNC. Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. We suggest using planimetric Grothoff's criteria to improve the specificity of LVNC diagnosis. 10.1007/s10554-020-01797-6
    Novel Approach to Risk Stratification in Left Ventricular Non-Compaction Using A Combined Cardiac Imaging and Plasma Biomarker Approach. Ramchand Jay,Podugu Pooja,Obuchowski Nancy,Harb Serge C,Chetrit Michael,Milinovich Alex,Griffin Brian,Burrell Louise M,Wilson Tang W H,Kwon Deborah H,Flamm Scott D Journal of the American Heart Association Background Left ventricular non-compaction remains a poorly described entity, which has led to challenges of overdiagnosis. We aimed to evaluate if the presence of a thin compacted myocardial layer portends poorer outcomes in individuals meeting cardiac magnetic resonance criteria for left ventricular non-compaction . Methods and Results This was an observational, retrospective cohort study involving individuals selected from the Cleveland Clinic Foundation cardiac magnetic resonance database (N=26 531). Between 2000 and 2018, 328 individuals ≥12 years, with left ventricular non-compaction or excessive trabeculations based on the cardiac magnetic resonance Petersen criteria were included. The cohort comprised 42% women, mean age 43 years. We assessed the predictive ability of myocardial thinning for the primary composite end point of major adverse cardiac events (composite of all-cause mortality, heart failure hospitalization, left ventricular assist device implantation/heart transplant, ventricular tachycardia, or ischemic stroke). At mean follow-up of 3.1 years, major adverse cardiac events occurred in 102 (31%) patients. After adjusting for comorbidities, the risk of major adverse cardiac events was nearly doubled in the presence of significant compacted myocardial thinning (hazard ratio [HR], 1.88 [95% CI, 1.18‒3.00]; =0.016), tripled in the presence of elevated plasma B-type natriuretic peptide (HR, 3.29 [95% CI, 1.52‒7.11]; =0.006), and increased by 5% for every 10-unit increase in left ventricular end-systolic volume (HR, 1.01 [95% CI, 1.00‒1.01]; =0.041). Conclusions The risk of adverse clinical events is increased in the presence of significant compacted myocardial thinning, an elevated B-type natriuretic peptide or increased left ventricular dimensions. The combination of these markers may enhance risk assessment to minimize left ventricular non-compaction overdiagnosis whilst facilitating appropriate diagnoses in those with true disease. 10.1161/JAHA.120.019209
    Correlation between left ventricular fractal dimension and impaired strain assessed by cardiac MRI feature tracking in patients with left ventricular noncompaction and normal left ventricular ejection fraction. Yu Shiqin,Chen Xiuyu,Yang Kai,Wang Jiaxin,Zhao Kankan,Dong Wenhao,Yan Weipeng,Su Guohai,Zhao Shihua European radiology OBJECTIVES:To investigate the correlation between the extent of excessive trabeculation assessed by fractal dimension (FD) and myocardial contractility assessed by cardiac MRI feature tracking in patients with left ventricular noncompaction (LVNC) and normal left ventricular ejection fraction (LVEF). METHODS:Forty-one LVNC patients with normal LVEF (≥ 50%) and 41 healthy controls were retrospectively included. All patients fulfilled three available diagnostic criteria on MRI. Cardiac MRI feature tracking was performed on cine images to determine left ventricular (LV) peak strains in three directions: global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain (GLS). The complexity of excessive trabeculation was quantified by fractal analysis on short-axis cine stacks. RESULTS:Compared with controls, patients with LVNC had impaired GRS, GCS, and GLS (all p < 0.05). The global, maximal, and regional FD values of the LVNC population were all significantly higher than those of the controls (all p < 0.05). Global FD was positively correlated with the end-diastolic volume index, end-systolic volume index, and stroke volume index (r = 0.483, 0.505, and 0.335, respectively, all p < 0.05), but negatively correlated with GRS and GCS (r =  - 0.458 and 0.508, respectively, both p < 0.001). Moreover, apical FD was also weakly associated with LVEF and GLS (r =  - 0.249 and 0.252, respectively, both p < 0.05). CONCLUSION:In patients with LVNC, LV systolic dysfunction was detected early by cardiac MRI feature tracking despite the presence of normal LVEF and was associated with excessive trabecular complexity assessed by FD. KEY POINTS:• Left ventricular global strain was already impaired in patients with extremely prominent excessive trabeculation but normal left ventricular ejection fraction. • An increased fractal dimension was associated with impaired deformation in left ventricular noncompaction. 10.1007/s00330-021-08346-2
    Electrocardiographic findings in correlation to magnetic resonance imaging patterns in African patients with isolated ventricular noncompaction. Akhbour Salwa,Fellat Ibtissam,Fennich Nada,Abdelali Salima,Doghmi Nawal,Ellouali Fedoua,Cherti Mohammed Anatolian journal of cardiology OBJECTIVE:Isolated ventricular noncompaction is a rare primary genetic cardiomyopathy characterized by persistent embryonic myocardial morphology without any other cardiac anomalies. Arrhythmias are frequently present, including both tachyarrhythmia and conduction disturbance. Our study aimed to describe the electrocardiographic findings and to correlate them with the clinical presentation and cardiac magnetic resonance imaging findings. METHODS:We retrospectively reviewed 24 patients diagnosed with isolated ventricular noncompaction (IVNC) by cardiac magnetic resonance imaging. Correlations were investigated between arrhythmias and the site of ventricular noncompaction, number of noncompacted segments, presence of fibrosis, and left ventricular dysfunction. RESULTS:The mean age was 42.7±13.1 years. Patients were first presented with heart failure in 41.7% and arrhythmia in 45.8%. Electrocardiogram was abnormal in 91.6% of patients; the most common anomaly was left bundle branch block (LBBB) (41.7%), followed by supraventricular arrhythmias (29.1%), repolarization abnormalities (29.1%), and ventricular tachycardia (20.8%). A normal left ventricular systolic function was frequently observed in patients who first presented with rhythm disorders than heart failure (p=0.008). There was also a delayed diagnosis of IVNC when presented with arrhythmia versus heart failure (p=0.02). We found no correlation between arrhythmias and the noncompaction site or fibrosis, except for LBBB, which was associated to left ventricle lateral wall involvement (p=0.028). No correlation between systolic dysfunction and the number of noncompacted segments, fibrosis, or arrhythmia was demonstrated. CONCLUSION:While electrocardiographic abnormalities are frequent in isolated ventricular noncompaction, no specific patterns were identified. More large studies are needed for stratification of arrhythmic risk of this highly arrhythmogenic substrate. 10.5152/akd.2014.5577
    Deletion of Nkx2-5 in trabecular myocardium reveals the developmental origins of pathological heterogeneity associated with ventricular non-compaction cardiomyopathy. Choquet Caroline,Nguyen Thi Hong Minh,Sicard Pierre,Buttigieg Emeline,Tran Thi Thom,Kober Frank,Varlet Isabelle,Sturny Rachel,Costa Mauro W,Harvey Richard P,Nguyen Catherine,Rihet Pascal,Richard Sylvain,Bernard Monique,Kelly Robert G,Lalevée Nathalie,Miquerol Lucile PLoS genetics Left ventricular non-compaction (LVNC) is a rare cardiomyopathy associated with a hypertrabeculated phenotype and a large spectrum of symptoms. It is still unclear whether LVNC results from a defect of ventricular trabeculae development and the mechanistic basis that underlies the varying severity of this pathology is unknown. To investigate these issues, we inactivated the cardiac transcription factor Nkx2-5 in trabecular myocardium at different stages of trabecular morphogenesis using an inducible Cx40-creERT2 allele. Conditional deletion of Nkx2-5 at embryonic stages, during trabecular formation, provokes a severe hypertrabeculated phenotype associated with subendocardial fibrosis and Purkinje fiber hypoplasia. A milder phenotype was observed after Nkx2-5 deletion at fetal stages, during trabecular compaction. A longitudinal study of cardiac function in adult Nkx2-5 conditional mutant mice demonstrates that excessive trabeculation is associated with complex ventricular conduction defects, progressively leading to strain defects, and, in 50% of mutant mice, to heart failure. Progressive impaired cardiac function correlates with conduction and strain defects independently of the degree of hypertrabeculation. Transcriptomic analysis of molecular pathways reflects myocardial remodeling with a larger number of differentially expressed genes in the severe versus mild phenotype and identifies Six1 as being upregulated in hypertrabeculated hearts. Our results provide insights into the etiology of LVNC and link its pathogenicity with compromised trabecular development including compaction defects and ventricular conduction system hypoplasia. 10.1371/journal.pgen.1007502
    Left Ventricular Noncompaction and Cardiogenic Shock. Kazmirczak Felipe,Martin Cindy M,Shenoy Chetan Circulation 10.1161/CIRCULATIONAHA.119.043716
    Regional and global ventricular systolic function in isolated ventricular non-compaction: pathophysiological insights from magnetic resonance imaging. Dellegrottaglie Santo,Pedrotti Patrizia,Roghi Alberto,Pedretti Stefano,Chiariello Massimo,Perrone-Filardi Pasquale International journal of cardiology BACKGROUND:Isolated ventricular non-compaction (IVNC) is frequently, but not invariably, associated with left ventricular (LV) systolic dysfunction. Factors impacting on regional and global LV function are unknown. The aim of the study was to apply magnetic resonance imaging (MRI) to evaluate the impact of extent and severity of ventricular non-compaction on LV systolic function in patients with IVNC. METHODS:Sixteen adult patients with IVNC as defined by previously validated MRI criteria [ratio between end-diastolic thickness of non-compacted and compacted myocardium (NC/C ratio)> 2.3 in ≥ 1 LV segment] were enrolled. Short-axis cine images were employed for analysis. Applying a 16-segment LV model, regional systolic performance was assessed qualitatively (wall motion score, WMS; 1 = normal, 2 = mild hypokinesia, 3 = moderate-to-severe hypokinesia, and 4 = a/dyskinesia) as well as quantitatively [fractional wall thickening, FWT (%)=100 × (end-diastolic wall thickness-end-systolic wall thickness)/end-diastolic wall thickness)]. RESULTS:Mean LV ejection fraction was 43.8 ± 15.4% (range, 17-68%). Regional disease severity, as expressed by the NC/C ratio, revealed a significant correlation with WMS (r=0.26; p=0.018) and FWT (r=-0.30; p=0.006). The total number of non-compacted segments/patient (NoNC) as an index of disease extent was a significant independent correlate of LV ejection fraction by multivariate regression analysis (β=-5.24; p=0.038) and an excellent predictor of global LV dysfunction (ROC analysis, AUC=0.98; p<0.0001). CONCLUSIONS:In patients with IVNC, disease severity correlates with the degree of LV dysfunction at a regional level. The extent of myocardial non-compaction is an independent predictor of global LV dysfunction. 10.1016/j.ijcard.2011.01.063
    Noncompaction myocardium in association with type Ib glycogen storage disease. Goeppert Benjamin,Lindner Martin,Vogel Monika Nadja,Warth Arne,Stenzinger Albrecht,Renner Marcus,Schnabel Philipp,Schirmacher Peter,Autschbach Frank,Weichert Wilko Pathology, research and practice Noncompaction myocardium is a rare disorder assumed to occur as an arrest of the compaction process during the normal development of the heart. Left ventricular noncompaction has been reported to be associated with a variety of cardiac and extracardiac, especially neuromuscular abnormalities. Moreover, it has been suggested that metabolic alterations could be responsible for the noncompaction. However, no association of noncompaction myocardium with type Ib glycogen storage disease (GSD) has been reported so far. Type Ib GSD is due to a defect of a transmembrane protein which results, similar to type Ia GSD, in hypoglycemia, a markedly enlarged liver and, additionally, in neutropenia, recurrent infections, and inflammatory bowel disease. Until now, no muscular or cardiac involvement has been described in type Ib GSD patients. The present case represents the first report of a noncompaction myocardium in a child with type Ib GSD who died of sudden clinical deterioration at the age of four. 10.1016/j.prp.2012.06.007
    Biventricular noncompaction cardiomyopathy with severe systolic and diastolic dysfunction in a systemic sclerosis patient. Kalaycı Belma,Kalaycı Süleyman,Karabağ Turgut,Aydın Mustafa Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by deep increased trabeculation in one or more segments of the ventricle. The apical segment of the left ventricle is most commonly affected, but left ventricular basal segment, biventricular involvement or right ventricle predominance have also been described. While some neuromuscular anomalies and myopathies had been described in systemic sclerosis patients, coexistence of chronic inflammatory disorders and NCM is unclear. This paper presents a case of biventricular NCM with severe systolic and diastolic dysfunction in a 40-year-old female diffuse cutaneous systemic sclerosis patient. 10.5543/tkda.2015.62392
    [Left ventricular noncompaction: A clinical and morphological study]. Mitrofanova L B,Moiseeva O M,Khashchevskaya D A,Mitrofanov N A,Pervunina T M,Zaklyazminskaya E V,Kovalsky G B Arkhiv patologii The data of clinical, macro- and micrometric, histological, and immunohistochemical studies of the heart were analyzed in patients with left ventricular noncompaction (LVNC). Materials from 7 patients: 5 hearts of recipients after heart transplantation, one heart of a dead patient, and one endomyocardial biopsy specimen were investigated. The investigations showed that this disease was accompanied by a preponderance of a noncompact layer with its ratio to a compact layer (2.4:6.6) in the left ventricle and by myocardial hypertrophy and fibrosis in all cases, by endocardial fibroelastosis and discomplexation of muscle fibers by more than 15% of the specimen area in 6 of the 7 cases, by right ventricular hypertrabeculation and myocarditis in 5 cases, and by lipomatosis and impaired connexin 43 expression in 4 cases. Only one of the four patients was found to have MYH 7 gene mutation. The results of MRI of the extracted heart coincided with morphological findings in 100% of cases. The comparative study demonstrated that this disease had simultaneously morphological features of both LVNC and restrictive, hypertrophic, dilated cardiomyopathy. The findings may suggest that the LVNC phenotype may be formed under the influence of various modifying factors (hemodynamic and inflammatory ones). 10.17116/patol201678229-35
    2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Towbin Jeffrey A,McKenna William J,Abrams Dominic J,Ackerman Michael J,Calkins Hugh,Darrieux Francisco C C,Daubert James P,de Chillou Christian,DePasquale Eugene C,Desai Milind Y,Estes N A Mark,Hua Wei,Indik Julia H,Ingles Jodie,James Cynthia A,John Roy M,Judge Daniel P,Keegan Roberto,Krahn Andrew D,Link Mark S,Marcus Frank I,McLeod Christopher J,Mestroni Luisa,Priori Silvia G,Saffitz Jeffrey E,Sanatani Shubhayan,Shimizu Wataru,van Tintelen J Peter,Wilde Arthur A M,Zareba Wojciech Heart rhythm Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia. 10.1016/j.hrthm.2019.05.007
    2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary. Towbin Jeffrey A,McKenna William J,Abrams Dominic J,Ackerman Michael J,Calkins Hugh,Darrieux Francisco C C,Daubert James P,de Chillou Christian,DePasquale Eugene C,Desai Milind Y,Estes N A Mark,Hua Wei,Indik Julia H,Ingles Jodie,James Cynthia A,John Roy M,Judge Daniel P,Keegan Roberto,Krahn Andrew D,Link Mark S,Marcus Frank I,McLeod Christopher J,Mestroni Luisa,Priori Silvia G,Saffitz Jeffrey E,Sanatani Shubhayan,Shimizu Wataru,van Tintelen J Peter,Wilde Arthur A M,Zareba Wojciech Heart rhythm Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia. 10.1016/j.hrthm.2019.09.019
    Defects in Trabecular Development Contribute to Left Ventricular Noncompaction. Choquet Caroline,Kelly Robert G,Miquerol Lucile Pediatric cardiology Left ventricular noncompaction (LVNC) is a genetically heterogeneous disorder the etiology of which is still debated. During fetal development, trabecular cardiomyocytes contribute extensively to the working myocardium and the ventricular conduction system. The impact of developmental defects in trabecular myocardium in the etiology of LVNC has been debated. Recently we generated new mouse models of LVNC by the conditional deletion of the key cardiac transcription factor encoding gene Nkx2-5 in trabecular myocardium at critical steps of trabecular development. These conditional mutant mice recapitulate pathological features similar to those observed in LVNC patients, including a hypertrabeculated left ventricle with deep endocardial recesses, subendocardial fibrosis, conduction defects, strain defects, and progressive heart failure. After discussing recent findings describing the respective contribution of trabecular and compact myocardium during ventricular morphogenesis, this review will focus on new data reflecting the link between trabecular development and LVNC. 10.1007/s00246-019-02161-9
    Cardiac magnetic resonance imaging characteristics of isolated left ventricular noncompaction in a Chinese adult Han population. Cheng Huaibing,Zhao Shihua,Jiang Shiliang,Yu Jinchao,Lu Minjie,Ling Jian,Zhang Yan,Yan Chaowu,Liu Qiong,Li Shiguo,Jin Lixin,Jerecic Renate,He Zuoxiang The international journal of cardiovascular imaging To analyze cardiac magnetic resonance imaging (CMR) characteristics in patients with isolated left ventricular noncompaction (IVNC) and assess its value in the diagnosis of IVNC in a Chinese adult Han population. We collected a consecutive series of 30 patients with IVNC from January 1, 2007, to December 31, 2008. During the same period, we prospectively included patients drawn from groups given a potential differential diagnosis for IVNC. All magnetic resonance images were analyzed using 17-segment model. Left ventricular ejection fraction was significantly lower for patients with DCM (16.2 ± 5.2%, P < 0.001) and higher in AR (47.6 ± 16.2%, P = 0.009), AS (54.6 ± 21.1%, P = 0.001) and HHD (62.4 ± 6.8%, P < 0.001) compared with IVNC (33.0 ± 14.1%). The two-layered structure was most frequently seen at the apical segments, followed by the mid-cavity and basal segments in patients with INVC. The anterior and lateral walls were more commonly involved in patients with IVNC. The number of noncompacted segments and end-diastolic ratio of non-compacted to compacted myocardium (NC/C ratio) was greater in patients with IVNC than in other five groups. The end-diastolic NC/C ratio of >2.5 had 96.4% sensitivity and 97.4% specificity for identifying patients with IVNC. CMR provides an accurate and reliable evaluation of the localization and extent of noncompacted myocardium at end-diastole. The end-diastolic NC/C ratio of >2.5 had high diagnostic accuracy for IVNC in a Chinese adult Han population. 10.1007/s10554-010-9741-x
    Meta-Analysis of the Prognostic Role of Late Gadolinium Enhancement and Global Systolic Impairment in Left Ventricular Noncompaction. Grigoratos Chrysanthos,Barison Andrea,Ivanov Alexander,Andreini Daniele,Amzulescu Mihaela-Silvia,Mazurkiewicz Lukasz,De Luca Antonio,Grzybowski Jacek,Masci Pier Giorgio,Marczak Magdalena,Heitner John F,Schwitter Juerg,Gerber Bernhard L,Emdin Michele,Aquaro Giovanni Donato JACC. Cardiovascular imaging OBJECTIVES:The objective of this meta-analysis was to assess the predictive value of late gadolinium enhancement (LGE) and global systolic impairment for future major adverse cardiovascular events in left ventricular noncompaction (LVNC). BACKGROUND:The prognosis of patients with LVNC, with and without left ventricular dysfunction and LGE, is still unclear. METHODS:A systematic review of published research and a meta-analysis reporting a combined endpoint of hard (cardiac death, sudden cardiac death, appropriate defibrillator firing, resuscitated cardiac arrest, cardiac transplantation, assist device implantation) and minor (heart failure hospitalization and thromboembolic events) events was performed. RESULTS:Four studies with 574 patients with LVNC and 677 with no LVNC and an average follow-up duration of 5.2 years were analyzed. In patients with LVNC, LGE was associated with the combined endpoint (pooled odds ratio: 4.9; 95% confidence interval: 1.63 to 14.6; p = 0.005) and cardiac death (pooled odds ratio: 9.8; 95% confidence interval: 2.44 to 39.5; p < 0.001). Preserved left ventricular systolic function was found in 183 patients with LVNC: 25 with positive LGE and 158 with negative LGE. In LVNC with preserved ejection fraction, positive LGE was associated with hard cardiac events (odds ratio: 6.1; 95% confidence interval: 2.1 to 17.5; p < 0.001). No hard cardiac events were recorded in patients with LVNC, preserved ejection fraction, and negative LGE. CONCLUSIONS:Patients with LVNC but without LGE have a better prognosis than those with LGE. When LGE is negative and global systolic function is preserved, no hard cardiac events are to be expected. Currently available criteria allow diagnosis of LVNC, but to further define the presence and prognostic significance of the disease, LGE and/or global systolic impairment must be considered for better risk stratification. 10.1016/j.jcmg.2018.12.029
    Left ventricular characteristics of noncompaction phenotype patients with good ejection fraction measured with cardiac magnetic resonance. Kiss Anna Reka,Gregor Zsófia,Furak Adam,Tóth Attila,Horváth Márton,Szabo Liliana,Czimbalmos Csilla,Dohy Zsofia,Merkely Bela,Vago Hajnalka,Szucs Andrea Anatolian journal of cardiology OBJECTIVE:We describe left ventricular (LV) volumes, myocardial and trabeculated muscle mass and strains with Cardiac magnetic resonance of a large cohort (n=81) who fulfilled the morphologic criteria of left ventricular noncompaction (LVNC) and had good ejection fraction (EF >55%) and compare them with healthy controls (n=81). Male and female patients were compared to matched controls and to each other. We also investigated the LV trabeculated muscle mass cutoff in male and female patients with LVNC. METHODS:81 participants with LVNC and 81 healthy controls were included. Male and female patients were compared to matched controls and to each other. We also investigated the left ventricular trabeculated muscle mass cut-off in male and female LVNC patients. RESULTS:The LV parameters of the LVNC population were normal, but they had significantly higher volumes, myocardial and trabeculated muscle mass, and a significantly smaller EF than the controls. Similar differences were observed after stratifying by sex. The optimal LV trabeculated muscle mass cutoffs were 25.8 g/m2 in men (area under the curve: 0.81) and 19.0 g/m2 in women (area under the curve: 0.87). The patients had normal global strains but a significantly worse global circumferential strain (patients vs controls: -29.9±4.9 vs. -35.8±4.7%, p<0.05) and significantly higher circumferential mechanical dispersion than the controls (patients vs. controls: 7.6±4.2 vs. 6.1±2.8%; p<0.05). No disease-related strain differences were noted between men and women. CONCLUSION:The LV functional and strain characteristics of the LVNC cohort differed significantly from those of healthy participants; this might be caused by increased LV trabeculation, and its clinical relevance might be questionable. The LV trabeculated muscle mass was very different between men and women; thus, the use of sex-specific morphologic diagnostic criteria should be considered. 10.5152/AnatolJCardiol.2021.25905
    Does Employing a Flowchart Improve the Diagnostic Performance of Cardiac Magnetic Resonance Imaging in Left Ventricular Noncompaction? Alis Deniz,Bagcilar Omer,Asmakutlu Ozan,Topel Cagdas,Bagcilar Yeseren Deniz,Sahin Anil,Gurbak Ismail,Karaarslan Ercan Acta Cardiologica Sinica Background:To test the hypothesis that making a diagnosis of left ventricular noncompaction (LVNC) on cardiac magnetic resonance imaging (CMRI) using a noncompacted-to-compacted (NC/C) myocardium ratio > 2.3 would yield significant errors, and also to test a diagnostic flowchart in patients who undergo CMRI and have clinical and echocardiographic findings suggesting LVNC could improve the diagnosis of LVNC. Methods:A total of 84 patients with LVNC and 162 controls consisting of patients with other diseases and healthy participants who had CMRI and echocardiograms were selected. The diagnostic flowchart of the study involved the use of CMRI with all available sequences for patients with a high pre-test probability of LVNC. Two blinded independent cardiologists evaluated echocardiograms, and patients with suggestive echocardiographic and clinical findings for LVNC were enrolled in the high pre-test probability of LVNC group. Two independent blinded radiologists established the diagnosis of LVNC based on NC/C ratio > 2.3 on CMRI, and they were allowed to re-assess the patients following the diagnostic flowchart. Results:An NC/C ratio > 2.3 identified 83 of 84 LVNC patients, yet incorrectly classified 48 of the 162 controls as having LVNC. Radiologists changed their decision in 23 of 48 patients with incorrect diagnoses, resulted in improved specificity (70.4% to 84.6%). The use of the CMRI diagnostic flowchart in the high pre-test probability group yielded a high specificity (97.2%) and accuracy (95.9%). Conclusions:LVNC diagnosed by CMRI based on the NC/C criterion can lead to overdiagnosis, whereas only using CMRI in patients with a high pre-test probability of LVNC with all available sequences may improve the diagnostic performance. 10.6515/ACS.202103_37(2).20201012A
    The mitral regurgitation effects of cardiac structure and function in left ventricular noncompaction. Zou Qing,Xu Rong,Li Xiao,Xu Hua-Yan,Yang Zhi-Gang,Wang Yi-Ning,Fan Hai-Ming,Guo Ying-Kun Scientific reports This study evaluated the effects of mitral regurgitation (MR) on cardiac structure and function in left ventricular noncompaction (LVNC) patients. The clinical and cardiovascular magnetic resonance (CMR) data for 182 patients with noncompaction or hypertrabeculation from three institutes were retrospectively included. We analyzed the difference in left ventricular geometry, cardiac function between LVNC patients with and without MR. The results showed that patients with MR had a worse New York Heart Association (NYHA) class and a higher incidence of arrhythmia (P < 0.05). MR occurred in 48.2% of LVNC patients. Compared to LVNC patients without MR, the two-dimensional sphericity index, maximum/minimum end-diastolic ratio and longitudinal shortening in LVNC patients with MR were lower (P < 0.05), and the peak longitudinal strain (PLS) of the global and segmental myocardium were obviously reduced (P < 0.05). No significant difference was found in strain in LVNC patients with different degree of MR; end diastolic volume, end systolic volume, and global PLS were statistically associated with MR and NYHA class (P < 0.05), but the non-compacted to compacted myocardium ratio had no significant correlation with them. In conclusion, the presence of MR is common in LVNC patients. LVNC patients with MR feature more severe morphological and functional changes. Hypertrabeculation is not an important factor affecting structure and function at the heart failure stage. 10.1038/s41598-021-84233-6
    Echocardiographic and clinical markers of left ventricular ejection fraction and moderate or greater systolic dysfunction in left ventricular noncompaction cardiomyopathy. Arenas Ivan A,Mihos Christos G,DeFaria Yeh Doreen,Yucel Evin,Elmahdy Hany M,Santana Orlando Echocardiography (Mount Kisco, N.Y.) BACKGROUND:Left ventricular noncompaction (LVNC) is associated with progressive LV systolic dysfunction and dilated cardiomyopathy. We aimed to investigate the echocardiographic and clinical characteristics associated with LV ejection fraction (LVEF) and moderate or greater systolic dysfunction in patients with LVNC. METHODS:Our institutional echocardiography database was retrospectively reviewed between 2008 and 2014, and 62 patients with LVNC were identified. Forty-three (69%) had moderate or greater LV systolic dysfunction (LVEF ≤ 40%) and were compared with 19 (31%) patients with preserved or mildly reduced LVEF (>40%). Linear regression analyses were utilized to identify markers associated with LVEF. RESULTS:The mean age was 63 ± 17 years and noncompacted-to-compacted ratio was 2.3 ± 0.5, and was larger in patients with LVEF ≤ 40% (2.4 vs 2.1; P = .02). Patients with LVEF ≤ 40% were older, had more congestive heart failure, significant QRS interval prolongation, and greater LV remodeling and worse mean global longitudinal strain (GLS). Multivariate regression analysis revealed increased age (standardized regression coefficient (β) = -0.17; P = .04) and QRS duration (β = -0.13; P = .08), congestive heart failure (β = -0.18; P = .04), and worsened GLS (β = -0.40; P = .001) were independently associated with decreased LVEF in the cohort (overall model fit R = 0.71; P < .0001). Increased age (β = -0.49; P = .01) and QRS duration (β = -0.50; P = .002), and worsened GLS (β = -0.33; P = .04), were also associated with a lower LVEF in patients with LVEF > 40%. CONCLUSIONS:The independent markers associated with LVEF and moderate or greater LV systolic dysfunction in patients with LVNC, in particular GLS and QRS duration, may detect high-risk candidates for more aggressive clinical surveillance and medical therapy. 10.1111/echo.13873
    Improvement of the Diagnosis of Left Ventricular Noncompaction Cardiomyopathy After Analyzing Both Advantages and Disadvantages of Echocardiography and CMRI. Xu Yifeng,Liu Xiaoxiao,Li Hongli Progress in cardiovascular diseases 10.1016/j.pcad.2018.05.006
    Evaluation of myocardial deformation pattern of left ventricular noncompaction by cardiac magnetic resonance tissue tracking. Pu Cailing,Hu Xiuhua,Ye Yang,Lv Sangying,Fei Jingle,Albaqali Samar Mohamed Abdulla Ali Maki,Hu Hongjie Kardiologia polska 10.33963/KP.15133
    Long-Term Survival of Patients With Left Ventricular Noncompaction. Vaidya Vaibhav R,Lyle Melissa,Miranda William R,Farwati Medhat,Isath Ameesh,Patlolla Sri Harsha,Hodge David O,Asirvatham Samuel J,Kapa Suraj,Deshmukh Abhishek J,Foley Thomas A,Michelena Hector I,Connolly Heidi M,Melduni Rowlens M Journal of the American Heart Association Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end-systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end-diastolic trough of trabeculation-to-epicardium (X):peak of trabeculation-to-epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end-diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow-up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02-1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17-4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21-3.68) were associated with all-cause mortality. Compared with the expected survival for age- and sex-matched US population, patients with LVNC had reduced overall survival (<0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age- and sex-matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population. 10.1161/JAHA.119.015563
    Association Between Left Ventricular Noncompaction and Vigorous Physical Activity. de la Chica Jose A,Gómez-Talavera Sandra,García-Ruiz Jose M,García-Lunar Ines,Oliva Belén,Fernández-Alvira Juan M,López-Melgar Beatriz,Sánchez-González Javier,de la Pompa José L,Mendiguren Jose M,Martínez de Vega Vicente,Fernández-Ortiz Antonio,Sanz Javier,Fernández-Friera Leticia,Ibáñez Borja,Fuster Valentín Journal of the American College of Cardiology BACKGROUND:Left ventricular (LV) hypertrabeculation fulfilling noncompaction cardiomyopathy criteria has been detected in athletes. However, the association between LV noncompaction (LVNC) phenotype and vigorous physical activity (VPA) in the general population is disputed. OBJECTIVES:The aim of this study was to assess the relationship between LVNC phenotype on cardiac magnetic resonance (CMR) imaging and accelerometer-measured physical activity (PA) in a cohort of middle-aged nonathlete participants in the PESA (Progression of Early Subclinical Atherosclerosis) study. METHODS:In PESA participants (n = 4,184 subjects free of cardiovascular disease), PA was measured by waist-secured accelerometers. CMR was performed in 705 subjects (mean age 48 ± 4 years, 16% women). VPA was recorded as total minutes per week. The study population was divided into 6 groups: no VPA and 5 sex-specific quintiles of VPA rate (Q1 to Q5). The Petersen criterion for LVNC was evaluated in all subjects undergoing CMR. For participants meeting this criterion (noncompacted-to-compacted ratio ≥2.3), 3 more restrictive LVNC criteria were also evaluated (Jacquier, Grothoff, and Stacey). RESULTS:LVNC phenotype prevalence according to the Petersen criterion was significantly higher among participants in the highest VPA quintile (Q5 = 30.5%) than in participants with no VPA (14.2%). The Jacquier and Grothoff criteria were also more frequently fulfilled in participants in the highest VPA quintile (Jacquier Q5 = 27.4% vs. no VPA = 12.8% and Grothoff Q5 = 15.8% vs. no VPA = 7.1%). The prevalence of the systolic Stacey LVNC criterion was low (3.6%) and did not differ significantly between no VPA and Q5. CONCLUSIONS:In a community-based study, VPA was associated with a higher prevalence of CMR-detected LVNC phenotype according to diverse established criteria. The association between VPA and LVNC phenotype was independent of LV volumes. According to these data, vigorous recreational PA should be considered as a possible but not uncommon determinant of LV hypertrabeculation in asymptomatic subjects. 10.1016/j.jacc.2020.08.030
    Long-Term Prognostic Value of Cardiac Magnetic Resonance in Left Ventricle Noncompaction: A Prospective Multicenter Study. Andreini Daniele,Pontone Gianluca,Bogaert Jan,Roghi Alberto,Barison Andrea,Schwitter Juerg,Mushtaq Saima,Vovas Georgios,Sormani Paola,Aquaro Giovanni D,Monney Pierre,Segurini Chiara,Guglielmo Marco,Conte Edoardo,Fusini Laura,Dello Russo Antonio,Lombardi Massimo,Gripari Paola,Baggiano Andrea,Fiorentini Cesare,Lombardi Federico,Bartorelli Antonio L,Pepi Mauro,Masci Pier Giorgio Journal of the American College of Cardiology BACKGROUND:Cardiac magnetic resonance (CMR) is useful for the diagnosis of left ventricular noncompaction (LVNC). However, there are limited data regarding its prognostic value. OBJECTIVES:The goal of this study was to evaluate the prognostic relevance of CMR findings in patients with LVNC. METHODS:A total of 113 patients with an echocardiographic diagnosis of LVNC underwent CMR at 5 referral centers. CMR diagnostic criterion of LVNC (noncompacted/compacted ratio >2.3 in end-diastole) was confirmed in all patients. We performed left ventricular (LV) and right ventricular quantitative analysis and late gadolinium enhancement (LGE) assessments and analyzed the following LVNC diagnostic criteria: left ventricular noncompacted myocardial mass (LV-ncMM) >20% and >25%, total LV-ncMM index >15 g/m, noncompacted/compacted ratio ≥3:1 ≥1 of segments 1 to 3 and 7 to 16 or ≥2:1 in at least 1 of segments 4 to 6 of the American Heart Association model. Outcome was a composite of thromboembolic events, heart failure hospitalizations, ventricular arrhythmias, and cardiac death. RESULTS:At a mean follow-up of 48 ± 24 months, cardiac events (CEs) occurred in 36 patients (16 heart failure hospitalizations, 10 ventricular arrhythmias, 5 cardiac deaths, and 5 thromboembolic events). LV dilation, impaired LV ejection fraction, and LV-ncMM >20% was significantly more frequent in patients with CEs. LV fibrosis was detected by using LGE in 11 cases. CMR predictors of CEs were LV dilation and LGE. LGE was associated with improved prediction of CEs, compared with clinical data and CMR functional parameters in all 3 models. No CEs occurred in patients without dilated cardiomyopathy and/or LGE. CONCLUSIONS:In patients with LVNC evaluated by using CMR, the degree of LV trabeculation seems to have no prognostic impact over and above LV dilation, LV systolic dysfunction, and presence of LGE. 10.1016/j.jacc.2016.08.053
    Changes in strain parameters at different deterioration levels of left ventricular function: A cardiac magnetic resonance feature-tracking study of patients with left ventricular noncompaction. Szűcs Andrea,Kiss Anna Réka,Gregor Zsófia,Horváth Márton,Tóth Attila,Dohy Zsófia,Szabó Liliána Erzsébet,Suhai Ferenc Imre,Merkely Béla,Vágó Hajnalka International journal of cardiology BACKGROUND:There is a lack of cardiac MRI information on left ventricular (LV) strain and rotational parameters of left ventricular noncompaction (LVNC) patients with reduced ejection fraction (EF). Thus, we sought to use feature tracking (FT) to describe these changes at different levels of EF deterioration. METHODS:We included 31 adult LVNC patients with reduced LV EF (Group B, EF < 50%) without any comorbidities or concomitant cardiac diseases, 31 age- and sex-matched LVNC patients with good EF (Group A, EF > 50%) and 31 healthy controls. Group B was divided according to LV EF into two subgroups (Group B-1: EF 35-50%, Group B-2: EF < 35%). Their global longitudinal, circumferential (GCS), and radial (GRS) strains; LV segmental strains; LV apical and basal rotation values; and patterns and degree of LV dyssynchrony were measured. RESULTS:All of the global and mean segmental strain parameters were significantly worse in Groups B, B-1 and B-2 than in Group A and in the controls. The LV mechanical dispersion increased as LV EF decreased. The degree of apical rotation was the highest in the control group, almost the same in Group A and the lowest and in the reverse direction in Group B-2. A rotational pattern, clockwise-directed rigid body rotation (RBR), was found in 39% of the Group B patients, and a counterclockwise-directed RBR was found in 26% of the Group A patients. CONCLUSIONS:The strain values and rotational parameters changed as the EF decreased. These changes affected the global LV, and we did not identify an LVNC-specific strain pattern. 10.1016/j.ijcard.2021.01.072
    Diagnostic Cardiovascular Magnetic Resonance Imaging Criteria in Noncompaction Cardiomyopathy and the Yield of Genetic Testing. van Waning Jaap I,Caliskan Kadir,Chelu Raluca G,van der Velde Nikki,Pezzato Andrea,Michels Michelle,van Slegtenhorst Marjon A,Boersma Eric,Nieman Koen,Majoor-Krakauer Danielle,Hirsch Alexander The Canadian journal of cardiology BACKGROUND:Noncompaction cardiomyopathy (NCCM) is characterized by a thickened myocardial wall with excessive trabeculations of the left ventricle, and ∼30% is explained by a (likely) pathogenic variant [(L)PV] in a cardiomyopathy gene. Diagnosing an (L)PV is important because it allows accurate identification of which relatives are at risk and helps predicting prognosis. The goal of this study was to assess which specific clinical and morphologic characteristics of the myocardium may predict an (L)PV and which of the cardiovascular magnetic resonance (CMR) diagnostic criteria for NCCM can best be used for that purpose. METHODS:Sixty-two patients with NCCM, diagnosed by means of echocardiographic Jenni criteria, underwent CMR imaging that was evaluated according the Petersen, Stacey, Jacquier, Captur, and Choi diagnostic CMR criteria for NCCM. Patients also underwent DNA testing and were stratified according to having an (L)PV. RESULTS:Thirty-three patients (53%) with NCCM had an (L)PV. The apical and mid-lateral segments were the dominant locations for meeting Petersen and/or Stacey criteria. Correlation between different CMR criteria varied from moderate to very strong. In multivariate binary logistic regression analysis with CMR and non-CMR parameters, independent positive predictors for an (L)PV were familial cardiomyopathy, trabecular mass, and meeting Petersen criteria in ≥ 2 out of 3 long-axis views, whereas left bundle branch block and hypertension were negative predictors. The receiver operating characteristic curve of this multivariate model had an area under the curve of 0.89 (95% confidence interval 0.82-0.97). CONCLUSIONS:CMR criteria together with family history help to distinguish those patients in whom an (L)PV can be identified, consequently leading to referral for genetic diagnostics and cascade screening. 10.1016/j.cjca.2020.05.021
    Evaluation of isolated left ventricular noncompaction using cardiac magnetic resonance tissue tracking in global, regional and layer-specific strains. Zhang Jiamin,Jiang Mengchun,Zheng Chao,Liu Hui,Guo Yangyu,Xie Xingzhi,Zou ZhiMin,Zhou Xiaoyue,Xia Liming,Luo Meichen,Zeng Mu Scientific reports We used cardiac magnetic resonance tissue tracking (CMR-TT) to quantitatively analyze the global, regional and layer-specific strain of isolated left ventricular noncompaction (ILVNC). Combined with late gadolinium enhancement (LGE), we initially explored the effect of focal myocardial fibrosis on myocardial strain. CMR was performed in 63 patients with ILVNC and 52 patients without ILVNC (i.e., the control group). The ILVNC group was divided into an LGE(+) group (29 patients) and an LGE(-) group (34 patients) according to the presence or absence of late gadalinum enhancement (LGE). CVI42 software was used to measure global and regional (basal, middle, apical) radial strain (RS), circumferential strain (CS), longitudinal strain (LS), subendocardial LS and subepicardial LS. The basal-apical strain gradient was defined as the apical mean strain minus the basal mean strain. We then compared differences between these strain parameters. The subendocardial-subepicardial LS gradient was defined as the maximum subendocardial LS minus the subepicardial LS. Compared with the control group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the ILVNC group were significantly diminished (P < 0.01). Compared with the LGE(-) group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the LGE(+) group were significantly diminished (P < 0.05). In the ILVNC group, the basal-apical CS and LS gradient, and the subendocardial-subepicardial LS gradient were significantly lower than those in the control group (P < 0.01). There were significant differences in myocardial strain between patients with and without ILVNC. ILVNC revealed a specific pattern in terms of strain change. The myocardial strain of the cardiac apex and endocardium was significantly lower than that of the cardiac base and epicardium, respectively. Myocardial strain reduction was more significant in ILVNC patients with focal myocardial fibrosis. 10.1038/s41598-021-86695-0
    Prevalence and Prognostic Significance of Left Ventricular Noncompaction in Patients Referred for Cardiac Magnetic Resonance Imaging. Ivanov Alexander,Dabiesingh Devindra S,Bhumireddy Geetha P,Mohamed Ambreen,Asfour Ahmed,Briggs William M,Ho Jean,Khan Saadat A,Grossman Alexandra,Klem Igor,Sacchi Terrence J,Heitner John F Circulation. Cardiovascular imaging BACKGROUND:Presence of prominent left ventricular trabeculation satisfying criteria for left ventricular noncompaction (LVNC) on routine cardiac magnetic resonance examination is frequently encountered; however, the clinical and prognostic significance of these findings remain elusive. This registry aimed to assess LVNC prevalence by 4 current criteria and to prospectively evaluate an association between diagnosis of LVNC by these criteria and adverse events. METHODS AND RESULTS:There were 700 patients referred for cardiac magnetic resonance: 42% were women, median age was 70 years (range, 45-71 years), mean left ventricular ejection fraction was 51% (±17%), and 32% had late gadolinium enhancement on cardiac magnetic resonance. The cohort underwent diagnostic assessment for LVNC by 4 separate imaging criteria-referenced by their authors as Petersen, Stacey, Jacquier, and Captur, with LVNC prevalence of 39%, 23%, 25% and 3%, respectively. Primary clinical outcome was combined end point of time to death, ischemic stroke, ventricular tachycardia/ventricular fibrillation, and heart failure hospitalization. Secondary clinical outcomes were (1) all-cause mortality and (2) time to the first occurrence of any of the following events: cardiac death, ischemic stroke, ventricular tachycardia/ventricular fibrillation, or heart failure hospitalization. During a median follow-up of 7 years, there were no statistically significant differences in assessed outcomes noted between patients with and without LVNC irrespective of the applied criteria. CONCLUSIONS:Current criteria for the diagnosis of LVNC leads to highly variable disease prevalence in patients referred for cardiac magnetic resonance. The diagnosis of LVNC, by any current criteria, was not associated with adverse clinical events on nearly 7 years of follow-up. Limited conclusions can be made for Captur criteria due to low observed prevalence. 10.1161/CIRCIMAGING.117.006174
    Value of Cardiac Magnetic Resonance Fractal Analysis Combined With Myocardial Strain in Discriminating Isolated Left Ventricular Noncompaction and Dilated Cardiomyopathy. Zheng Tian,Ma Xiaohai,Li Shuhao,Ueda Takuya,Wang Zheng,Lu Aijia,Zhou Wei,Zou Hongye,Zhao Lei,Gong Lianggeng Journal of magnetic resonance imaging : JMRI BACKGROUND:Excessive trabeculation is present in isolated left ventricular noncompaction (LVNC) and dilated cardiomyopathy (DCM), which sometimes makes the differentiation between these two difficult. Fractal dimension (FD) is a unitless measure value of how completely the object fills space, which can assess the extent of myocardial trabeculae quantitatively. PURPOSE:To compare the trabeculae features and myocardial strain derived from cardiac MR between LVNC and DCM. STUDY TYPE:Respective case-control series. POPULATION:In all, 35 LVNC patients and 30 DCM patients were enrolled, and 20 healthy volunteers were selected as a control group. FIELD STRENGTH/SEQUENCE:5 T with 8-channel phased-array cardiac receiver coil including steady-state free precession cine imaging. ASSESSMENT:The degree of left ventricular trabeculation was evaluated by a semiautomatic tool based on fractal analysis. Myocardial deformation was assessed by feature tracking. STATISTICAL TESTS:Independent samples Student's t-test, Mann-Whitney U-test, receiver operating characteristics (ROC) curves, and Spearman's rank coefficient were conducted. RESULTS:Max apical FD and mean global FD were higher in the LVNC group than in the DCM group (1.433 ± 0.074 vs. 1.341 ± 0.062, P < 0.001; 1.323 ± 0.036 vs. 1.267 ± 0.041, P < 0.001, respectively). For diagnosing LVNC, max apical FD was 1.392 (area under the curve [AUC] = 0.881, 95% confidence interval [CI]: 0.804-0.957), and the cutoff value of mean global FD was 1.283 (AUC = 0.895, 95% CI: 0.828-0.961). The global peak longitudinal strain value of the left ventricle (GPLS) showed significant differences between the LVNC group and DCM group [-6.49 (-11.41, -4.90) vs. -4.61 (-5.87, -3.61), P = 0.006]. The diagnostic accuracy for LVNC is highest when using FDs in coordination with GPLS (AUC = 0.93, 95% CI: 0.86-0.98, P < 0.001). DATA CONCLUSION:Fractal analysis provides a quantitative measurement of myocardial trabeculation. The combination of fractal analysis with myocardial strain provides a novel biomarker in distinguishing LVNC from DCM. LEVEL OF EVIDENCE:3 Technical Efficacy Stage: 2 J. Magn. Reson. Imaging 2019;50:153-163. 10.1002/jmri.26616
    Echocardiography fails to detect left ventricular noncompaction in a cohort of patients with noncompaction on cardiac magnetic resonance imaging. Diwadkar Sachin,Nallamshetty Leelakrishna,Rojas Carlos,Athienitis Alexia,Declue Chris,Cox Chad,Patel Aarti,Chae Sanders H Clinical cardiology BACKGROUND:Left ventricular noncompaction (LVNC) is a rare disorder characterized by increased left ventricular trabeculation, deep intertrabecular recesses, and a thin compacted myocardial layer with associated clinical sequelae. Cardiac imaging with echocardiogram and cardiac magnetic resonance (CMRI) can detect variable myocardial morphology including excessive trabeculations. Multiple CMRI and echocardiographic criteria have been offered that attempt to identify LVNC morphology. The aim of this study was to assess the utility of echocardiogram in identifying LVNC in a cohort of patients with LVNC detected on CMRI. HYPOTHESIS:Echocardiography fails to identify LVNC morphology in a large proportion of patients with LVNC/hypertrabeculation detected on CMRI. METHODS:There were 1060 CMRI studies collected from 2009 to 2015 at 2 institutions. The patients included in this study (n = 37) met the criteria for LVNC on CMRI and had complete CMRI and echocardiogram images Clinical and imaging data were retrospectively reviewed. RESULTS:Of the 37 patients with LVNC on CMRI, only 10 patients (27%) had LVNC identified on echocardiogram (P < 0.0001, 95% confidence interval: 25.7%-66.2%). Echocardiography and CMRI were also significantly different in terms of identification of distribution of LVNC. Although 21 of 37 patients (57%) had evidence of LVNC in either the anterior or lateral walls on CMRI, there were 0 patients with LVNC detected in the anterior or lateral walls on echocardiogram (P = 0.019). CONCLUSIONS:Echocardiogram fails to detect LVNC morphology/hypertrabeculation in a significant number of a cohort of patients with LVNC on CMRI. LVNC may be missed if echocardiogram is the only imaging modality performed in a cardiac evaluation. 10.1002/clc.22669
    The value of cardiac magnetic resonance imaging and programmed ventricular stimulation in patients with ventricular noncompaction and ventricular arrhythmias. Gunda Sampath,Ghannam Michael,Liang Jackson J,Attili Anil,Sharaf Dabbagh Ghaith,Cochet Hubert,Lathkar-Pradhan Sangeetha,Latchamsetty Rakesh,Jongnarangsin Krit,Morady Fred,Bogun Frank Journal of cardiovascular electrophysiology INTRODUCTION:Left ventricular noncompaction (LVNC) is associated with ventricular arrhythmias (VA) including premature ventricular complexes, and ventricular tachycardia (VT). The value of imaging with delayed enhancement cardiac magnetic resonance (DE-CMR) and programmed ventricular stimulation (PVS) for risk stratification in patients with VA and LVNC is unknown. The purpose of this study was to determine whether DE-CMR and PVS are beneficial for risk stratification and whether CMR helps to identify VA target sites. METHODS AND RESULTS:Consecutive patients with LVNC undergoing ablation for VAs were included, all patients had preprocedure DE-CMR. A total of 23 patients (7 women, 46 ± 14 years, ejection fraction 35 ± 14) were included and followed for 2.9 ± 2.2 years. DE-CMR scar was present in 12/23 patients (52%). PVS was performed in 20/23 patients, 8/10 patients (80%) with scar were inducible for VT compared to 0/10 (0%) patients without scar (p < .001). VA target sites in patients with scarring were located adjacent to areas of scarring in all but 1 patient and ablation was successful in 15/23 patients (65%). Patients with scar had worse survival free of VT than those without scar (log rank p = .01) and patients with inducible VT had worse survival free of VT than those who were noninducible (log rank p < .001). CONCLUSIONS:The presence of CMR defined scar in patients with LVNC was associated with inducible VT and worse outcomes. Inducibility for VT was associated with VT recurrence. Furthermore, CMR is beneficial in localizing the arrhythmogenic substrate in LVNC and therefore can aid in procedural planning. 10.1111/jce.14884