加载中

    Does septal myectomy reduce risk of sudden cardiac death in patients with hypertrophic cardiomyopathy? Schaff Hartzell V,Nguyen Anita The Journal of thoracic and cardiovascular surgery 10.1016/j.jtcvs.2018.04.082
    Surgical relief of left ventricular outflow obstruction in pediatric hypertrophic cardiomyopathy: The need for a tailored approach. Karamichalis John M,Aguib Heba,Anastasopulos Alexandra,Yacoub Magdi The Journal of thoracic and cardiovascular surgery 10.1016/j.jtcvs.2018.08.015
    Surgical results for hypertrophic obstructive cardiomyopathy: More than an institutional niche. Hui Dawn S,Lee Richard The Journal of thoracic and cardiovascular surgery 10.1016/j.jtcvs.2018.09.061
    Surgical management of left ventricular outflow tract obstruction in a specialized hypertrophic obstructive cardiomyopathy center. Hodges Kevin,Rivas Carlos Godoy,Aguilera José,Borden Robert,Alashi Alaa,Blackstone Eugene H,Desai Milind Y,Smedira Nicholas G The Journal of thoracic and cardiovascular surgery OBJECTIVES:This study evaluates operative approach and contemporary surgical outcomes in the management of left ventricular outflow tract obstruction by a single surgeon at a high-volume, specialized hypertrophic cardiomyopathy center. METHODS:This is a retrospective review of 1559 consecutive operations for left ventricular outflow tract obstruction from 2005 to 2015. Demographic profiles, echocardiogram-derived ventricular morphology and hemodynamics, operative data, and in-hospital outcomes were analyzed. RESULTS:Of the 1559 operations, 586 were isolated septal myectomies, 522 were myectomies with mitral valve or subvalvular apparatus intervention, 422 were myectomies with another concomitant procedure, and 29 were isolated mitral valve interventions without myectomy. Common mitral valve interventions included anterior leaflet shortening (16%), chordae tendineae resection (9.8%), papillary muscle resection (7.2%), and papillary muscle reorientation (7.5%). Ninety-two patients underwent mitral valve replacement, 42 for left ventricular outflow tract obstruction and 50 for intrinsic mitral valve pathology. Patients undergoing mitral interventions had thinner septums (18 ± 0.4 mm vs 22 ± 0.5 mm, P < .001) and less myocardium removed (6.2 ± 3.5 g vs 8.8 ± 3.8 g, P < .001) than patients without a mitral intervention. Prevalence of in-hospital permanent pacemaker insertion was 4.2% (n = 1334) for complete heart block and 1.1% (n = 464) for isolated septal myectomy with normal preoperative conduction. Overall, there were 2 postoperative ventricular septal defects (0.13%) and none for isolated myectomies. Operative mortality was 0.38%. CONCLUSIONS:Septal myectomy can be performed safely with excellent outcomes when the procedure is performed by a highly experienced surgeon in a high-volume, specialized center. A mitral valve intervention is a useful adjunct in patients with moderate hypertrophy. 10.1016/j.jtcvs.2018.11.148
    Sex differences in hypertrophic cardiomyopathy: Time to tailor risk stratification and therapy? Constantine Andrew,Dimopoulos Konstantinos,Rafiq Isma,Vazir Ali European journal of preventive cardiology 10.1177/2047487319890996
    Predictors of long-term outcome after septal myectomy in symptomatic hypertrophic obstructive cardiomyopathy patients with previous alcohol septal ablation and residual obstruction. Zhu Changsheng,Tang Bing,Cui Hao,Wang Shengwei,Xiao Minghu,Chen Zixian,Meng Yanhai,Zhao Shihua,Song Yunhu,Yu Qinjun,Wang Shuiyun Journal of cardiac surgery BACKGROUND AND AIM:Recently alcohol septal ablation (ASA) has emerged as an alternative treatment for drug-refractory hypertrophic obstructive cardiomyopathy (HOCM) and a subgroup of HOCM patients with previous ASA may need myectomy. However, subsequent outcome and mechanism of residual obstruction has not been determined. This study aims to determine outcome after myectomy and mechanism of residual obstruction in HOCM patients with previous ASA. METHODS:From February 2009 to June 2017, 38 HOCM patients with previous ASA underwent surgical septal myectomy at our institution. Seventy-six patients who underwent surgical septal myectomy initially were included as the comparison group through one-to-two propensity score matching method. RESULTS:Fourteen available cardiac magnetic resonance images revealed inferior location and small area of infarcted myocardium induced by ASA in 12 patients and outside targeted location in two patients. During follow-up (median, 2.4; maximum, 7.8 years), event-free survival at 7 years was 83.2% in the previous ASA group and 94.6% in the comparison group, respectively (P = 0.0378). Multivariable analysis indicated previous ASA (hazard ratio, 4.28; 95% confidence intervals [CI], 1.20-15.26; P = 0.025) and postoperative left ventricular end-diastolic diameter (hazard ratio, 1.14; 95% CI, 1.05-1.23; P = 0.002) were independent predictors of adverse events. CONCLUSIONS:This study demonstrated that uncontrollable extent and location of infarcted myocardium induced by ASA may attribute to residual obstruction after previous ASA, and the long-term event-free survival after myectomy was inferior. It may provide special precaution to patient selection and the increased number of ASA practiced worldwide. 10.1111/jocs.14072
    Different Histopathologic Diagnoses in Patients With Clinically Diagnosed Hypertrophic Cardiomyopathy After Surgical Myectomy. Alashi Alaa,Desai Ria M,Khullar Tamanna,Hodges Kevin,Rodriguez E Rene,Tan Carmela,Popovic Zoran B,Thamilarasan Maran,Wierup Per,Lever Harry M,Smedira Nicholas G,Desai Milind Y Circulation 10.1161/CIRCULATIONAHA.119.040129
    Conduction Abnormalities and Long-Term Mortality Following Septal Myectomy in Patients With Obstructive Hypertrophic Cardiomyopathy. Cui Hao,Schaff Hartzell V,Nishimura Rick A,Geske Jeffrey B,Dearani Joseph A,Lahr Brian D,Ommen Steve R Journal of the American College of Cardiology BACKGROUND:Conduction abnormalities including bundle branch block are recognized sequelae following septal myectomy in patients with hypertrophic cardiomyopathy, but their incidence and effect on long-term mortality have not been well studied. OBJECTIVES:This study was conducted to determine the impact of conduction abnormalities on mortality in patients with obstructive hypertrophic cardiomyopathy following myectomy. METHODS:The outcomes of 2,482 patients with obstructive hypertrophic cardiomyopathy who underwent transaortic septal myectomy from 1961 to 2016 were analyzed. Pre-operative and early post-operative electrocardiograms were reviewed to determine conduction status. The study endpoint was all-cause mortality. RESULTS:Mean age at operation was 54.2 ± 14.6 years, and 1,370 patients (55.2%) were men. Pre-operatively, 2,159 patients (87.0%) had normal conduction, of whom 38.8% developed left bundle branch block, 1.1% developed right bundle branch block (RBBB), and 0.6% had complete heart block (CHB) after myectomy. Among 112 patients with baseline RBBB, 34.8% developed CHB post-operatively. Overall, only 2.3% of patients developed CHB. Over a median follow-up period of 8.6 years, overall mortality differed among post-operative conduction groups after adjustment for age, sex, and concomitant procedures (p = 0.015). Specifically, mortality in those with paced rhythm after myectomy was significantly increased relative to those with normal conduction (hazard ratio: 1.57; 95% confidence interval: 1.15 to 2.14; p = 0.005), although no significant difference was observed in left bundle branch block and RBBB (vs. normal) groups. CONCLUSIONS:Left bundle branch block is a common sequela after septal myectomy but does not influence post-operative mortality. Myectomy-related CHB is rare in patients with baseline normal conduction, while pre-existing RBBB greatly increases the risk for CHB. Persistent paced rhythm after operation is an independent predictor of mortality. 10.1016/j.jacc.2019.05.053
    A Floating Stitch on the Anterior Mitral Leaflet Can Eliminate Systolic Anterior Motion in Hypertrophic Obstructive Cardiomyopathy. Mutsuga Masato,Narita Yuji,Usui Akihiko Seminars in thoracic and cardiovascular surgery Reducing the systolic anterior motion is a key way of resolving left ventricular outflow tract obstruction in surgery for hypertrophic obstructive cardiomyopathy. We developed a new technique using a floating stitch on the anterior mitral leaflet (AML). It is applied just to the middle of the tip of the AML and fixed to the annuloplasty ring at the middle of the posterior annulus. The AML is prevented from touching the interventricular septum and systolic anterior motion can theoretically be solved. 10.1053/j.semtcvs.2019.09.006
    In-Hospital Postoperative Atrial Fibrillation Indicates a Poorer Clinical Outcome after Myectomy for Obstructive Hypertrophic Cardiomyopathy. Tang Bing,Song Yunhu,Cheng Sainan,Cui Hao,Ji Keshan,Zhao Shihua,Wang Shuiyun Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia OBJECTIVES:This study aims to investigate the risk factors of in-hospital postoperative atrial fibrillation (POAF) and the impact of POAF on the clinical outcome in hypertrophic cardiomyopathy (HCM) patients who underwent myectomy. METHODS:Data from a total of 494 obstructive HCM patients, who had undergone preoperative cardiac magnetic resonance (CMR) testing and who underwent myectomy at Fuwai Hospital from June 2011 to June 2016, were collected. RESULTS:Multivariate logistic regression analysis showed that old age (odds ratio [OR], 4.326; 95% confidence interval [CI], 2.248-8.325; p <0.001), maximal left atrium volume (LAV) (OR, 1.137; 95% CI, 1.075-1.202; p <0.001), and hypertension (OR, 2.754; 95% CI, 1.262-6.007; p = 0.011) were associated with the incidence of POAF. In the patients without preoperative AF, Cox regression analysis demonstrated that POAF (p = 0.002), decreased left atrium (LA) ejection fraction (LAEF) (p = 0.036), concomitant procedure (p = 0.039), and postoperative residual moderate or severe mitral valve regurgitation (p = 0.030) were independent predictors of composite cardiovascular events. CONCLUSIONS:POAF indicated a poorer clinical outcome after myectomy for obstructive HCM patients, which was similar to those with preoperative AF. Elevated LAV was independently related to POAF onset in HCM patients who underwent myectomy. 10.5761/atcs.oa.19-00108
    Childhood Hypertrophic Obstructive Cardiomyopathy and Its Relevant Surgical Outcome. Zhu Changsheng,Wang Shuiyun,Ma Yi,Wang Shengwei,Zhou Zhou,Song Yunhu,Yan Jun,Meng Yanhai,Nie Changrong The Annals of thoracic surgery BACKGROUND:Contemporary experiences regarding childhood hypertrophic obstructive cardiomyopathy are limited. This study aimed to describe the clinical presentation of childhood hypertrophic obstructive cardiomyopathy and its relevant surgical outcome. METHODS:In all, 117 consecutive children with hypertrophic obstructive cardiomyopathy aged 0.6 to 17.5 years who underwent septal myectomy at our institution between February 2009 and December 2018 were included. Medical records and other patient-related data were reviewed. RESULTS:In the present study, the anatomic and physiologic characteristics of childhood hypertrophic obstructive cardiomyopathy were highly heterogeneous, with simultaneous right ventricular outflow tract obstruction in 22 patients (18.8%), coronary myocardial bridging in 25 patients (21.4%), and intraventricular anatomic abnormalities in 61 patients (52.1%). The mean peak left or right ventricular outflow tract gradient, interventricular septal thickness, and degree of mitral regurgitation significantly decreased after surgery. One early death was noted in the study. During follow-up, three sudden cardiac deaths were noted. The overall survival rates at follow-up were 100% at 1 year and 96.5% at 3 years. The overall survival rates free from reoperation were 99.1% at 1 year and 98.0% at 3 years. CONCLUSIONS:In our cohort of children with hypertrophic obstructive cardiomyopathy undergoing septal myectomy, biventricular obstruction, myocardial bridging, and intraventricular anatomic abnormalities are frequent phenotypic components. Despite the complexity of childhood hypertrophic obstructive cardiomyopathy, surgical treatment results in a favorable outcome in carefully selected patients. 10.1016/j.athoracsur.2019.10.016
    Preoperative NT-proBNP Predicts Midterm Outcome After Septal Myectomy. Song Changpeng,Wang Shengwei,Guo Ying,Zheng Xinxin,Lu Jie,Fang Xiaonan,Wang Shuiyun,Huang Xiaohong Journal of the American Heart Association Background The prognostic value of N-terminal pro-brain natriuretic peptide ( NT -pro BNP ) in patients with hypertrophic cardiomyopathy who underwent septal myectomy has not been well studied. Methods and Results We retrospectively evaluated NT -pro BNP levels in 758 patients (46.1±13.8 years; median follow-up, 936 days) who underwent septal myectomy in our center between March 2011 and April 2018. The median NT -pro BNP level was 1450.5 (interquartile range 682.6-2649.5) pg/mL. Overall, 22 (2.9%) patients died during follow-up; of these, 86.4% were cardiovascular deaths. The 3-year survival free from all-cause mortality by tertile was 95.2% (95% CI 91.1% to 97.4%; NT -pro BNP >2080 pg/mL), 98.3% (95% CI 94.6% to 99.5%; NT -pro BNP , 947-2080 pg/mL), and 99.2% (95% CI , 94.4% to 99.9%; NT -pro BNP <947 pg/mL). The 3-year survival rate free from cardiovascular mortality by tertiles was 95.2% in the highest tertile, 98.8% in the middle tertile, and 99.2% in the lowest tertile. Cox regression analysis indicated that Ln( NT -pro BNP ) was a significantly independent predictor of all-cause mortality (hazard ratio 2.380, 95% CI 1.356-4.178, P=0.003) and cardiovascular mortality (hazard ratio 2.788, 95% CI 1.450-5.362, P=0.002). In addition, concomitant coronary artery bypass grafting for coronary artery disease was also an independent predictor of cardiovascular mortality (hazard ratio 5.178, 95% CI 1.597-16.789, P=0.006). Conclusions Increased preoperative NT -pro BNP level is a strong predictor of midterm mortality in patients undergoing septal myectomy. 10.1161/JAHA.118.011075
    Effect of Septal Myectomy on Obstructive Sleep Apnoea Syndrome in Patients With Hypertrophic Obstructive Cardiomyopathy. Wang Shengwei,Cui Hao,Meng Liukun,Wu Rong,Tang Bing,Zhu Changsheng,Yu Qinjun,Huang Xiaohong,Wang Shuiyun Heart, lung & circulation BACKGROUND:The prevalence of obstructive sleep apnoea (OSA) is high in patients with hypertrophic cardiomyopathy. The effect of septal myectomy on OSA is not clear. This study aimed to examine the association between hypertrophic obstructive cardiomyopathy and OSA before and after septal myectomy. METHOD:We included 85 consecutive patents with a confirmed diagnosis of hypertrophic obstructive cardiomyopathy who underwent septal myectomy. Polysomnography was performed in all patients before and 3 months after the surgery. RESULTS:Of the 85 patients, 49 (58%) were diagnosed with OSA. Patients with OSA were significantly older than those without OSA. The incidence of atrial fibrillation significantly increased during the perioperative period in patients with OSA (p = 0.03). The severity of OSA significantly increased 3 months after surgery, as determined by the apnoea-hypopnoea index (AHI; p < 0.001), obstructive apnoea index (p = 0.024), and hypopnoea index (p = 0.003), whereas central apnoea index was decreased (p = 0.008). In the multivariate linear regression analysis, mean oxygen desaturation and time% with SpO <90% during sleep before surgery were significantly associated with increased AHI, independently of body mass index and sex (p = 0.026 and p = 0.007, respectively; adjusted R = 0.365). CONCLUSIONS:The severity of OSA significantly increased 3 months after septal myectomy as determined by AHI, obstructive apnoea index, and hypopnoea index. Mean oxygen saturation and time% with SpO <90% during sleep before surgery were independently associated with the increase of AHI. However, the specific mechanism of such deterioration of OSA after septal myectomy needs to be determined in detail. 10.1016/j.hlc.2019.05.190
    Surgical Approaches to Hypertrophic Cardiomyopathy and Implications for Perioperative Management. Herrera Elizabeth Lee,Lawrie Gerald M International anesthesiology clinics 10.1097/AIA.0000000000000203
    Surgical Myectomy: Subaortic, Midventricular, and Apical. Nguyen Anita,Schaff Hartzell V Cardiology clinics Surgical septal myectomy is the preferred method of septal reduction for most patients with obstructive hypertrophic cardiomyopathy whose symptoms do not respond to medical management. Transaortic extended septal myectomy has low operative mortality and provides durable relief of symptoms. Surgical treatment is possible for patients with less common phenotypes, such as complex long-segment septal hypertrophy, midventricular obstruction, or apical hypertrophic cardiomyopathy. For these anatomic subtypes, transapical myectomy can be used alone or combined with transaortic myectomy. This article describes both of these surgical techniques and discusses preoperative considerations and postoperative management for patients with hypertrophic cardiomyopathy. 10.1016/j.ccl.2018.08.006
    Clinical Profile of Nonresponders to Surgical Myectomy with Obstructive Hypertrophic Cardiomyopathy. Wells Sophie,Rowin Ethan J,Boll Griffin,Rastegar Hassan,Wang Wendy,Maron Martin S,Maron Barry J The American journal of medicine BACKGROUND:Surgical myectomy reverses heart failure symptoms in the vast majority of obstructive hypertrophic cardiomyopathy patients. However, a small subgroup fails to experience sustained postoperative improvement despite relief of obstruction. Clinical profile of such patients has not been well defined. METHODS:Consecutive obstructive hypertrophic cardiomyopathy patients undergoing myectomy at Tufts Medical Center for drug-refractory New York Heart Association III/IV heart failure symptoms, 2004 to 2017, were followed postoperatively for 2.5 ± 2.8 years and assessed for outcome. RESULTS:Of the 503 patients, there were 4 postoperative deaths (0.8%); 480 patients (96%) had sustained improvement to New York Heart Association classes I or II (responders), but 19 (3.8%) developed advanced symptoms (classes III or IV) in the absence of obstruction (nonresponders). Compared with responders, nonresponders were younger (40 ± 13 vs 53 ± 14 years; P < .001) and had greater septal thickness (25 ± 9 vs 20 ± 4 mm; P < .001). Massive hypertrophy (≥30 mm) was 5-fold more common in nonresponders (P < .01). Seven nonresponders developed systolic dysfunction (ejection fraction 20%-47%), 2 days to 6.1 years postoperatively. Four nonresponders underwent heart transplant 3.4 to 9.2 years after myectomy, and 2 others have been listed. CONCLUSIONS:Surgical myectomy is highly effective at reversing heart failure symptoms in the vast majority of patients with obstructive hypertrophic cardiomyopathy. However, a small minority experience persistent functional limitation despite surgical relief of outflow obstruction. Predictors of adverse postoperative course were substantial/massive septal thickness and youthful age. Patients who failed to respond symptomatically to myectomy were considered for advanced heart failure treatment, including heart transplantation. 10.1016/j.amjmed.2017.12.031
    Surgical myectomy improves pulmonary hypertension in obstructive hypertrophic cardiomyopathy. Geske Jeffrey B,Konecny Tomas,Ommen Steve R,Nishimura Rick A,Sorajja Paul,Schaff Hartzell V,Ackerman Michael J,Gersh Bernard J European heart journal AIMS:Characterization of pulmonary hypertension (PH) and the effects of myectomy in hypertrophic cardiomyopathy (HCM) remain poorly defined. The aim of the study was to investigate the effect of myectomy on PH in HCM. METHODS AND RESULTS:This is a retrospective analysis of 306 consecutive symptomatic HCM patients (70% NYHA class III-IV) with evaluation of echocardiographic right ventricular systolic pressure (RVSP) both preceding (median 3 days) and following (median 4 days) myectomy. Compared with patients without PH (RVSP <35 mmHg, n = 145, 47%), patients with moderate or severe PH (RVSP ≥50 mmHg, n = 51, 17%) were older, predominantly female, had a greater prevalence of atrial fibrillation, higher natriuretic peptide levels, higher left ventricular outflow tract gradient, higher E velocity, and larger left atria. Reduction of RVSP post-myectomy was evident in patients with moderate or severe PH [59 (IQR 54-71) to 50 (IQR 39-62) mmHg, P < 0.0001] and in all patients with PH [RVSP ≥ 35 mmHg, n = 161, 43 (IQR 39-54) to 41 (IQR 35-52) mmHg, P < 0.0001]. In a subgroup of patients with long-term data, PH continued to decline during follow-up. Clinical variables associated with improvement in PH in these patients were higher left atrial volume index (R = 0.43, P = 0.0069) and moderate or severe mitral regurgitation (R = 0.33, P = 0.038). CONCLUSION:Surgical myectomy is associated with improvement in PH, most pronounced in moderate or severe PH. These data provide insight into pulmonary haemodynamics following obstruction relief and can help to guide therapeutic expectations. 10.1093/eurheartj/eht537
    Transapical myectomy for severe mid-ventricular obstructive hypertrophic cardiomyopathy. Ong Kevin C,Geske Jeffrey B,Schaff Hartzell V,Nishimura Rick A European heart journal 10.1093/eurheartj/ehu146
    Excess anterior mitral leaflet in a patient with hypertrophic obstructive cardiomyopathy and systolic anterior motion. Numata Satoshi,Yaku Hitoshi,Doi Kiyoshi,Yamazaki Sachiko,Yamamoto Tsuneyasu,Kobayashi Takuma,Manabe Kaichiro,Kawazoe Kohei Circulation 10.1161/CIRCULATIONAHA.114.013830
    Myocardial 3-Dimensional Printing for Septal Myectomy Guidance in a Patient With Obstructive Hypertrophic Cardiomyopathy. Yang Dong Hyun,Kang Joon-Won,Kim Namkug,Song Jae-Kwan,Lee Jae-Won,Lim Tae-Hwan Circulation 10.1161/CIRCULATIONAHA.115.015842
    Low Operative Mortality Achieved With Surgical Septal Myectomy: Role of Dedicated Hypertrophic Cardiomyopathy Centers in the Management of Dynamic Subaortic Obstruction. Maron Barry J,Dearani Joseph A,Ommen Steve R,Maron Martin S,Schaff Hartzell V,Nishimura Rick A,Ralph-Edwards Anthony,Rakowski Harry,Sherrid Mark V,Swistel Daniel G,Balaram Sandhya,Rastegar Hassan,Rowin Ethan J,Smedira Nicholas G,Lytle Bruce W,Desai Milind Y,Lever Harry M Journal of the American College of Cardiology 10.1016/j.jacc.2015.06.1333
    Transaortic Chordal Cutting: Mitral Valve Repair for Obstructive Hypertrophic Cardiomyopathy With Mild Septal Hypertrophy. Ferrazzi Paolo,Spirito Paolo,Iacovoni Attilio,Calabrese Alice,Migliorati Katrin,Simon Caterina,Pentiricci Samuele,Poggio Daniele,Grillo Massimiliano,Amigoni Pietro,Iascone Maria,Mortara Andrea,Maron Barry J,Senni Michele,Bruzzi Paolo Journal of the American College of Cardiology BACKGROUND:In severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM) and mild septal hypertrophy, mitral valve (MV) abnormalities may play an important role in MV displacement into the left ventricular (LV) outflow tract. Therefore, isolated myectomy may not relieve outflow obstruction and symptoms, and MV replacement is often the surgical alternative. OBJECTIVES:This study sought to assess the clinical and hemodynamic results of cutting thickened secondary MV chordae combined with a shallow septal muscular resection in severely symptomatic patients with obstructive HCM and mild septal hypertrophy. METHODS:Clinical features were compared before surgery and at most recent clinical evaluation in 39 consecutive patients with obstructive HCM. RESULTS:Over a 23 ± 2 months follow-up, New York Heart Association functional class decreased from 2.9 ± 0.5 pre-operatively to 1.1 ± 1.1 post-operatively (p < 0.001), with no patient in class III at most recent evaluation. The resting outflow gradient decreased from 82 ± 43 mm Hg to 9 ± 5 mm Hg (p < 0.001) and septal thickness decreased from 17 ± 1 mm to 14 ± 2 mm (p < 0.001). No patient had MV prolapse or flail and 1 had residual moderate-to-severe MV regurgitation at most recent evaluation. MV geometry before and after surgery was compared with that of 25 consecutive patients with similar clinical profile and septal thickness that underwent isolated myectomy. After adjustment for differences in pre-operative values between the groups, the post-operative anterior MV leaflet-annulus ratio was 17% greater and tenting area 24% smaller in patients with chordal cutting, indicating that MV apparatus had moved to a more normal posterior position within the LV cavity, preventing MV systolic displacement into the outflow tract and outflow obstruction. CONCLUSIONS:This procedure relieves heart failure symptoms, abolishes LV outflow gradient, and avoids MV replacement in patients with obstructive HCM and mild septal thickness. 10.1016/j.jacc.2015.07.069
    What Is the True Prevalence of Hypertrophic Cardiomyopathy? Baudhuin Linnea M,Kotzer Katrina E,Kluge Michelle L,Maleszewski Joseph J Journal of the American College of Cardiology 10.1016/j.jacc.2015.07.074
    Long-term clinical outcome after alcohol septal ablation for obstructive hypertrophic cardiomyopathy: results from the Euro-ASA registry. Veselka Josef,Jensen Morten Kvistholm,Liebregts Max,Januska Jaroslav,Krejci Jan,Bartel Thomas,Dabrowski Maciej,Hansen Peter Riis,Almaas Vibeke Marie,Seggewiss Hubert,Horstkotte Dieter,Tomasov Pavol,Adlova Radka,Bundgaard Henning,Steggerda Robbert,Ten Berg Jurriën,Faber Lothar European heart journal AIMS:The first cases of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) were published two decades ago. Although the outcomes of single-centre and national ASA registries have been published, the long-term survival and clinical outcome of the procedure are still debated. METHODS AND RESULTS:We report long-term outcomes from the as yet largest multinational ASA registry (the Euro-ASA registry). A total of 1275 (58 ± 14 years, median follow-up 5.7 years) highly symptomatic patients treated with ASA were included. The 30-day post-ASA mortality was 1%. Overall, 171 (13%) patients died during follow-up, corresponding to a post-ASA all-cause mortality rate of 2.42 deaths per 100 patient-years. Survival rates at 1, 5, and 10 years after ASA were 98% (95% CI 96-98%), 89% (95% CI 87-91%), and 77% (95% CI 73-80%), respectively. In multivariable analysis, independent predictors of all-cause mortality were age at ASA (P < 0.01), septum thickness before ASA (P < 0.01), NYHA class before ASA (P = 0.047), and the left ventricular (LV) outflow tract gradient at the last clinical check-up (P = 0.048). Alcohol septal ablation reduced the LV outflow tract gradient from 67 ± 36 to 16 ± 21 mmHg (P < 0.01) and NYHA class from 2.9 ± 0.5 to 1.6 ± 0.7 (P < 0.01). At the last check-up, 89% of patients reported dyspnoea of NYHA class ≤2, which was independently associated with LV outflow tract gradient (P < 0.01). CONCLUSIONS:The Euro-ASA registry demonstrated low peri-procedural and long-term mortality after ASA. This intervention provided durable relief of symptoms and a reduction of LV outflow tract obstruction in selected and highly symptomatic patients with obstructive HCM. As the post-procedural obstruction seems to be associated with both worse functional status and prognosis, optimal therapy should be focused on the elimination of LV outflow tract gradient. 10.1093/eurheartj/ehv693
    The Mitral Valve in Obstructive Hypertrophic Cardiomyopathy: A Test in Context. Sherrid Mark V,Balaram Sandhya,Kim Bette,Axel Leon,Swistel Daniel G Journal of the American College of Cardiology Mitral valve abnormalities were not part of modern pathological and clinical descriptions of hypertrophic cardiomyopathy in the 1950s, which focused on left ventricular (LV) hypertrophy and myocyte fiber disarray. Although systolic anterior motion (SAM) of the mitral valve was discovered as the cause of LV outflow tract obstruction in the M-mode echocardiography era, in the 1990s structural abnormalities of the mitral valve became appreciated as contributing to SAM pathophysiology. Hypertrophic cardiomyopathy mitral malformations have been identified at all levels. They occur in the leaflets, usually elongating them, and also in the submitral apparatus, with a wide array of malformations of the papillary muscles and chordae, that can be detected by transthoracic and transesophageal echocardiography and by cardiac magnetic resonance. Because they participate fundamentally in the predisposition to SAM, they have increasingly been repaired surgically. This review critically assesses imaging and measurement of mitral abnormalities and discusses their surgical relief. 10.1016/j.jacc.2016.01.071
    Management of Atrial Fibrillation in Hypertrophic Cardiomyopathy. MacIntyre Ciorsti,Lakdawala Neal K Circulation 10.1161/CIRCULATIONAHA.115.015085
    The Father of Septal Myectomy for Obstructive HCM, Who Also Had HCM: The Unbelievable Story. Maron Barry J,Roberts William C Journal of the American College of Cardiology 10.1016/j.jacc.2016.05.002
    How Hypertrophic Cardiomyopathy Became a Contemporary Treatable Genetic Disease With Low Mortality: Shaped by 50 Years of Clinical Research and Practice. Maron Barry J,Rowin Ethan J,Casey Susan A,Maron Martin S JAMA cardiology Hypertrophic cardiomyopathy (HCM) is a relatively common genetic heart disease encumbered throughout much of its almost 60-year history by a large measure of misunderstanding and the perception of a grim outcome without effective treatment options. However, it is now apparent that the majority of patients affected with HCM can achieve normal or near-normal life expectancy without disability, and usually do not require major treatment interventions. Nevertheless, for those patients with HCM who are at risk for (or experience) disease-related complications, a constellation of comprehensive nonpharmacologic management strategies have evolved over the last 15 years, altering the natural history and disease course for many, including implantable defibrillators, heart transplant, external defibrillation/therapeutic hypothermia, advances in surgical myectomy, and alcohol ablation. In particular, expanded contemporary risk stratification strategies have led to a more reliable selection of patients likely to achieve primary prevention of sudden death with implantable defibrillators. Most recently, large cohort studies using current management strategies and therapeutic measures have shown that it is now possible to achieve significantly improved survival with a low HCM-related mortality of 0.5% per year across all ages, and including children and young adults characteristically with the most aggressive disease course. These clinical management initiatives, instituted by the practicing cardiology community, have succeeded in preserving life and restoring an active lifestyle for thousands of patients with HCM, while providing many with a measure of reassurance and a reasonable expectation for an extended (if not normal) life span. 10.1001/jamacardio.2015.0354
    Hospital Volume Outcomes After Septal Myectomy and Alcohol Septal Ablation for Treatment of Obstructive Hypertrophic Cardiomyopathy: US Nationwide Inpatient Database, 2003-2011. Kim Luke K,Swaminathan Rajesh V,Looser Patrick,Minutello Robert M,Wong S Chiu,Bergman Geoffrey,Naidu Srihari S,Gade Christopher L F,Charitakis Konstantinos,Singh Harsimran S,Feldman Dmitriy N JAMA cardiology IMPORTANCE:Previous data on septal myectomy (SM) and alcohol septal ablation (ASA) in obstructive hypertrophic cardiomyopathy have been limited to small, nonrandomized, single-center studies. Use of septal reduction therapy and the effect of institutional experience on procedural outcomes nationally are unknown. OBJECTIVE:To examine in-hospital outcomes after SM and ASA stratified by hospital volume within a large, national inpatient database. DESIGN, SETTING, AND PARTICIPANTS:This study analyzed all patients who were hospitalized for SM or ASA in a nationwide inpatient database from January 1, 2003, through December 31, 2011. MAIN OUTCOMES AND MEASURES:Rates of adverse in-hospital events (death, stroke, bleeding, acute renal failure, and need for permanent pacemaker) were examined. Multivariate logistic regression analysis was performed to compare overall outcomes after each procedure based on tertiles of hospital volume of SM and ASA. RESULTS:Of 71 888 761 discharge records reviewed, a total of 11 248 patients underwent septal reduction procedures, of whom 6386 (56.8%) underwent SM and 4862 (43.2%) underwent ASA. A total of 59.9% of institutions performed 10 SM procedures or fewer, whereas 66.9% of institutions performed 10 ASA procedures or fewer during the study period. Incidence of in-hospital death (15.6%, 9.6%, and 3.8%; P < .001), need for permanent pacemaker (10.0%, 13.8%, and 8.9%; P < .001), and bleeding complications (3.3%, 3.8%, and 1.7%; P < .001) after SM was lower in higher-volume centers when stratified by first, second, and third tertiles of hospital volume, respectively. Similarly, there was a lower incidence of death (2.3%, 0.8%, and 0.6%; P = .02) and acute renal failure (6.2%, 7.6%, and 2.4%; P < .001) after ASA in higher-volume centers. The lowest tertile of SM volume among hospitals was an independent predictor of in-hospital all-cause mortality (adjusted odds ratio, 3.11; 95% CI, 1.98-4.89) and bleeding (adjusted odds ratio, 3.77; 95% CI, 2.12-6.70), whereas being in the lowest tertile of ASA by volume was not independently associated with an increased risk of adverse postprocedural events. CONCLUSIONS AND RELEVANCE:In US hospitals from 2003 through 2011, most centers that provide septal reduction therapy performed few SM and ASA procedures, which is below the threshold recommended by the 2011 American College of Cardiology Foundation/American Heart Association Task Force Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy. Low SM volume was associated with worse outcomes, including higher mortality, longer length of stay, and higher costs. More efforts are needed to encourage referral of patients to centers of excellence for septal reduction therapy. 10.1001/jamacardio.2016.0252
    Nonobstructive Hypertrophic Cardiomyopathy: Low Mortality but Not Always Benign. Pozios Iraklis,Abraham Theodore P Journal of the American College of Cardiology 10.1016/j.jacc.2016.04.069
    Mitral Regurgitation in Patients With Hypertrophic Obstructive Cardiomyopathy: Implications for Concomitant Valve Procedures. Hong Joon Hwa,Schaff Hartzell V,Nishimura Rick A,Abel Martin D,Dearani Joseph A,Li Zhuo,Ommen Steve R Journal of the American College of Cardiology BACKGROUND:Incidence and outcome of mitral valve (MV) surgery are unknown in patients with hypertrophic obstructive cardiomyopathy (HOCM) undergoing extended transaortic septal myectomy. OBJECTIVES:This study sought to define indications and suitable operative strategy for mitral regurgitation (MR) in patients with HOCM. METHODS:A total of 2,107 septal myectomy operations performed in adults from January 1993 to May 2014 at Mayo Clinic in Rochester, Minnesota, were retrospectively reviewed. Patients with prior MV operation and apical hypertrophic cardiomyopathy were excluded. Overall, 2,004 operations were performed in 1,993 patients. RESULTS:Pre-operative MR was grade ≥3 (of 4) in 1,152 operations (57.5%). Systolic anterior motion of mitral leaflets caused the MR in most patients. However, intrinsic MV disease was identified pre-operatively in 99 patients, all of whom had MV surgery (with septal myectomy). In 1,905 operations, no intrinsic MV disease was identified pre-operatively; in 1,830 (96.1%), septal myectomy was performed without a direct MV procedure. For 75 patients, intrinsic MV disease discovered intraoperatively led to concomitant MV repair (86.7%) or replacement (13.3%). After isolated septal myectomy, the percentage of patients with MR grade ≥3 decreased from 54.3% to 1.7% (p = 0.001) on early post-operative echocardiography. Among 174 patients with concomitant MV surgery, late survival was superior with MV repair (n = 133 [76.4%]) versus replacement (10-year survival: 80.0% vs. 55.2%; p = 0.002). CONCLUSIONS:In most patients with HOCM, MR related to systolic anterior motion of the MV is relieved through adequate myectomy. Concomitant MV surgery is rarely necessary unless intrinsic MV disease is present. When MV procedures are required, repair is preferred because of improved survival compared with replacement. 10.1016/j.jacc.2016.07.735
    MV Surgery as Adjunct to Surgical Myectomy for Obstructive HCM: Less Is More Than Enough. Weissler-Snir Adaya,Adler Arnon,Rakowski Harry Journal of the American College of Cardiology 10.1016/j.jacc.2016.04.070
    Procedural Volume and Outcomes of Septal Reduction Therapies in Patients With Hypertrophic Obstructive Cardiomyopathy. Panaich Sidakpal S,Chothani Ankit,Badheka Apurva O JAMA cardiology 10.1001/jamacardio.2016.3767
    Hypertrophic obstructive cardiomyopathy. Veselka Josef,Anavekar Nandan S,Charron Philippe Lancet (London, England) Hypertrophic obstructive cardiomyopathy is an inherited myocardial disease defined by cardiac hypertrophy (wall thickness ≥15 mm) that is not explained by abnormal loading conditions, and left ventricular obstruction greater than or equal to 30 mm Hg. Typical symptoms include dyspnoea, chest pain, palpitations, and syncope. The diagnosis is usually suspected on clinical examination and confirmed by imaging. Some patients are at increased risk of sudden cardiac death, heart failure, and atrial fibrillation. Patients with an increased risk of sudden cardiac death undergo cardioverter-defibrillator implantation; in patients with severe symptoms related to ventricular obstruction, septal reduction therapy (myectomy or alcohol septal ablation) is recommended. Life-long anticoagulation is indicated after the first episode of atrial fibrillation. 10.1016/S0140-6736(16)31321-6
    Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm: Implications for Risk Stratification and Management. Rowin Ethan J,Maron Barry J,Haas Tammy S,Garberich Ross F,Wang Weijia,Link Mark S,Maron Martin S Journal of the American College of Cardiology BACKGROUND:A previously under-recognized subset of hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) apical aneurysms is being identified with increasing frequency. However, risks associated with this subgroup are unknown. OBJECTIVES:The authors aimed to clarify clinical course and prognosis of a large cohort of HCM patients with LV apical aneurysms over long-term follow-up. METHODS:The authors retrospectively analyzed 1,940 consecutive HCM patients at 2 centers, 93 of which (4.8%) were identified with LV apical aneurysms; mean age was 56 ± 13 years, and 69% were male. RESULTS:Over 4.4 ± 3.2 years, 3 of the 93 patients with LV apical aneurysms (3%) died suddenly or of heart failure, but 22 (24%) survived with contemporary treatment interventions: 18 experienced appropriate implantable cardioverter-defibrillator discharges, 2 underwent heart transplants, and 2 were resuscitated after cardiac arrest. The sudden death (SD) event rate was 4.7%/year, which includes sudden death, successful resuscitation from cardiac arrest or appropriate ICD interventions triggered by VF or rapid VT. Notably, recurrent monomorphic ventricular tachycardia requiring ≥2 implantable cardioverter-defibrillator shocks occurred in 13 patients, including 6 who underwent successful radiofrequency ablation of the arrhythmic focus without ventricular tachycardia recurrence. Five non-anticoagulated patients experienced nonfatal thromboembolic events (1.1%/year), whereas 13 with apical clots and anticoagulation did not incur embolic events. There was no consistent relationship between aneurysm size and adverse HCM-related events. Rate of HCM-related deaths combined with life-saving aborted disease-related events was 6.4%/year, 3-fold greater than the 2.0%/year event rate in 1,847 HCM patients without aneurysms (p < 0.001). CONCLUSIONS:HCM patients with LV apical aneurysms are at high risk for arrhythmic sudden death and thromboembolic events. Identification of this phenotype expands risk stratification and can lead to effective treatment interventions for potentially life-threatening complications. 10.1016/j.jacc.2016.11.063
    To Add or Not to Add Mitral Valve Surgery to Septal Myectomy in HOCM Patients. Kluin Jolanda,Wilde Arthur A M,Pinto Yigal,van Herwerden Lex A Journal of the American College of Cardiology 10.1016/j.jacc.2016.11.093
    Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm. Abinader Edward G Journal of the American College of Cardiology 10.1016/j.jacc.2017.04.059
    Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy: A Word of Endorsement. Liebregts Max,Vriesendorp Pieter A,Ten Berg Jurrien M Journal of the American College of Cardiology Twenty years after the introduction of alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy, the arrhythmogenicity of the ablation scar appears to be overemphasized. When systematically reviewing all studies comparing ASA with myectomy with long-term follow-up, (aborted) sudden cardiac death and mortality rates were found to be similarly low. The focus should instead shift toward lowering the rate of reinterventions and pacemaker implantations following ASA because, in this area, ASA still seems inferior to myectomy. Part of the reason for this difference is that ASA is limited by the route of the septal perforators, whereas myectomy is not. Improvement may be achieved by: 1) confining ASA to hypertrophic cardiomyopathy centers of excellence with high operator volumes; 2) improving patient selection using multidisciplinary heart teams; 3) use of (3-dimensional) myocardial contrast echocardiography for selecting the correct septal (sub)branch; and 4) use of appropriate amounts of alcohol for ASA. 10.1016/j.jacc.2017.02.080
    Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy: A Word of Balance. Sorajja Paul Journal of the American College of Cardiology The management of drug-refractory symptoms of obstructive hypertrophic cardiomyopathy has long been debated and is primarily centered on the choice between surgical myectomy and alcohol septal ablation. Decision making in these patients requires consideration of procedural risk, expertise and efficacy, and the long-term impact on patients' survival. Although there have been numerous reports on these procedures that may help guide decision making, these data continue to be self-reported and voluntary. Greater insight into the real-world experience for these therapies and how they should be applied in practice would be gained from mandatory reporting, akin to other multidisciplinary, transcatheter-based therapies. 10.1016/j.jacc.2017.06.011
    Intraoperative Diagnosis of Anderson-Fabry Disease in Patients With Obstructive Hypertrophic Cardiomyopathy Undergoing Surgical Myectomy. Cecchi Franco,Iascone Maria,Maurizi Niccolò,Pezzoli Laura,Binaco Irene,Biagini Elena,Fibbi Maria Laura,Olivotto Iacopo,Pieruzzi Federico,Fruntelata Ana,Dorobantu Lucian,Rapezzi Claudio,Ferrazzi Paolo JAMA cardiology Importance:Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective:To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations. Design, Setting, and Participants:This retrospective analysis of patients undergoing surgical septal reduction strategies was conducted in 3 European tertiary referral centers for HCM from July 2013 to December 2016. Patients with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were observed for at least 18 months after the procedure (mean [SD] follow-up, 33 [14] months). Main Outcomes and Measures:Etiology of patients with HCM who underwent surgical myectomy. Results:From 2013, 235 consecutive patients with a clinical diagnosis of HCM underwent septal myectomy. The cardiac surgeon suspected a storage disease in 3 patients (1.3%) while inspecting their heart samples extracted from myectomy. The mean (SD) age at diagnosis for these 3 patients was 42 (4) years; all were male. None of the 3 patients presented with extracardiac features suggestive of AFC. All patients showed asymmetrical left ventricular hypertrophy, with maximal left ventricular thickness in the basal septum (19-31 mm), severe basal LVOTO (70-120 mm Hg), and left atrial dilatation (44-57 mm). Only 1 patient presented with late gadolinium enhancement on cardiovascular magnetic resonance at the right ventricle insertion site. The mean (SD) age at surgical procedure was 63 (5) years. On tactile sensation, the surgeon felt a spongy consistency of the surgical samples, different from the usual stony-elastic consistency typical of classic HCM, and this prompted histological examinations. Histology showed evidence of intracellular storage, and genetic analysis confirmed a GLA A gene mutation (p.Asn215Ser) in all 3 patients. Conclusions and Relevance:Screening for AFC should be performed even in the absence of red flags in patients with HCM older than 25 years. 10.1001/jamacardio.2017.2353
    Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation. Nishimura Rick A,Seggewiss Hubert,Schaff Hartzell V Circulation research Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated. 10.1161/CIRCRESAHA.116.309348
    Clinical Profile and Consequences of Atrial Fibrillation in Hypertrophic Cardiomyopathy. Rowin Ethan J,Hausvater Anais,Link Mark S,Abt Patrick,Gionfriddo William,Wang Wendy,Rastegar Hassan,Estes N A Mark,Maron Martin S,Maron Barry J Circulation BACKGROUND:Atrial fibrillation (AF), the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), is capable of producing symptoms that impact quality of life and is associated with risk for embolic stroke. However, the influence of AF on clinical course and outcome in HCM remains incompletely resolved. METHODS:Records of 1558 consecutive patients followed at the Tufts Medical Center Hypertrophic Cardiomyopathy Institute for 4.8±3.4 years (from 2004 to 2014) were accessed. RESULTS:Of the 1558 patients with HCM, 304 (20%) had episodes of AF, of which 226 (74%) were confined to symptomatic paroxysmal AF (average, 5±5; range, 1 to >20), whereas 78 (26%) developed permanent AF, preceded by 7±6 paroxysmal AF episodes. At last evaluation, 277 patients (91%) are alive at 62±13 years of age, including 89% in New York Heart Association class I or II. No difference was found in outcome measures for patients with AF and age- and sex-matched patients with HCM without AF. Four percent of patients with AF died of HCM-related causes (n=11), with annual mortality 0.7%; mortality directly attributable to AF (thromboembolism without prophylactic anticoagulation) was 0.1% per year (n=2 patients). Patients were treated with antiarrhythmic drugs (most commonly amiodarone [n=103] or sotalol [n=78]) and AF catheter ablation (n=49) or the Maze procedure at surgical myectomy (n=72). Freedom from AF recurrence at 1 year was 44% for ablation patients and 75% with the Maze procedure (<0.001). Embolic events were less common with anticoagulation prophylaxis (4/233, 2%) than without (9/66, 14%) (<0.001). CONCLUSIONS:Transient symptomatic episodes of AF, often responsible for impaired quality of life, are unpredictable in frequency and timing, but amenable to effective contemporary treatments, and infrequently progress to permanent AF. AF is not a major contributor to heart failure morbidity or a cause of arrhythmic sudden death; when treated, it is associated with low disease-related mortality, no different than for patients without AF. AF is an uncommon primary cause of death in HCM virtually limited to embolic stroke, supporting a low threshold for initiating anticoagulation therapy. 10.1161/CIRCULATIONAHA.117.029267
    Prediction of Mid-Term Outcomes in Adult Obstructive Hypertrophic Cardiomyopathy After Surgical Ventricular Septum Myectomy. Tang Bing,Song Yunhu,Cui Hao,Ji Keshan,Zhu Changsheng,Zhao Shihua,Huang Xiaohong,Yu Qinjun,Hu Shengshou,Wang Shuiyun Journal of the American College of Cardiology 10.1016/j.jacc.2017.08.032
    Early Outcomes of Repair of Left Ventricular Apical Aneurysms in Patients With Hypertrophic Cardiomyopathy. Nguyen Anita,Schaff Hartzell V,Nishimura Rick A,Dearani Joseph A,Ommen Steve R Circulation 10.1161/CIRCULATIONAHA.117.030593
    Clinical Course and Management of Hypertrophic Cardiomyopathy. Maron Barry J The New England journal of medicine 10.1056/NEJMra1710575
    Percutaneous Intramyocardial Septal Radiofrequency Ablation for Hypertrophic Obstructive Cardiomyopathy. Liu Liwen,Li Jing,Zuo Lei,Zhang Jinzhou,Zhou Mengyao,Xu Bo,Hahn Rebeccca T,Leon Martin B,Hsi David H,Ge Junbo,Zhou Xiaodong,Zhang Jun,Ge Shuping,Xiong Lize Journal of the American College of Cardiology BACKGROUND:In patients with disabling symptoms caused by hypertrophic obstructive cardiomyopathy (HOCM), echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) could be a less invasive treatment option. OBJECTIVES:This study aimed to investigate the safety and efficacy of the PIMSRA for left ventricular outflow tract (LVOT) gradient reduction in HOCM. METHODS:The study enrolled 15 patients with HOCM. These patients underwent electrocardiography, imaging, and blood biochemistry examination over 6 months of follow-up. RESULTS:At 6 months of follow-up, patients showed significant reductions in peak LVOT gradients (resting gradient: from 88.00 [66.00] mm Hg to 11.00 [6.00] mm Hg; p = 0.001; stress-induced gradient: from 117.00 [81.00] mm Hg to 25.00 [20.00] mm Hg; p = 0.005) and interventricular septum (IVS) thickness (anterior IVS: from 25.00 [21.00] mm to 14.00 [12.00] mm; p = 0.001; posterior IVS: from 24.00 [21.00] mm to 14.00 [11.50] mm; p = 0.001). The reductions in IVS thickness and LVOT gradients were associated with improvement in New York Heart Association functional classification (from 3.00 [2.00] to 1.00 [1.00]; p < 0.001), total exercise time (from 6.00 [5.50] min to 9.00 [8.00] min; p = 0.007), and pro B-type natriuretic peptide levels (from 924.00 [370.45] pg/ml to 137.45 [75.73] pg/ml; p = 0.028). No patient had bundle branch block or complete heart block. CONCLUSIONS:PIMSRA is a safe and effective treatment approach for severe, symptomatic HOCM and results in sustained improvement in exercise capacity, persistent reduction in LVOT gradient, and sustained improvement in cardiac function. 10.1016/j.jacc.2018.07.080
    Searching for Surgical Alternatives in Hypertrophic Cardiomyopathy. Sorajja Paul,Katsiyiannis William T,Harris Kevin M Journal of the American College of Cardiology 10.1016/j.jacc.2018.06.077
    Clinical Course and Management of Hypertrophic Cardiomyopathy. Pascoe Steven J The New England journal of medicine 10.1056/NEJMc1812159
    Case for Earlier Surgical Myectomy in Patients With Obstructive Hypertrophic Cardiomyopathy. Maron Martin S,Spirito Paolo,Maron Barry J Circulation 10.1161/CIRCULATIONAHA.118.035484
    Survival After Alcohol Septal Ablation in Patients With Hypertrophic Obstructive Cardiomyopathy. Batzner Angelika,Pfeiffer Barbara,Neugebauer Anna,Aicha Diaa,Blank Christoph,Seggewiss Hubert Journal of the American College of Cardiology BACKGROUND:Alcohol-induced infarction for treatment of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) was discussed as a risk factor for increased cardiac mortality during follow-up. OBJECTIVES:This study sought to report on long-term survival after echo-guided alcohol septal ablation (percutaneous transluminal septal myocardial ablation [PTSMA]) in symptomatic patients with HOCM. METHODS:Between May 2000 and June 2017, PTSMA with alcohol injection was performed in 952 patients (age 55.7 ± 14.9 years; 59.2% men; 73.3% New York Heart Association functional class III or IV; 50.3% syncope; 10.3% sudden cardiac death in family). Clinical follow-up after 6.0 ± 5.0 years was achieved in all patients. RESULTS:We injected 2.1 ± 0.4 cc of alcohol. Maximal creatine kinase rise was 872 ± 489 U/l. Two (0.21%) patients died 3 and 33 days after ablation. Permanent pacemaker was implanted in 100 (10.50%) patients. Echo gradients were acutely reduced from 63.9 ± 38.2 mm Hg to 33.6 ± 29.8 mm Hg at rest and from 104.6 ± 44.0 mm Hg to 56.5 ± 41.0 mm Hg at Valsalva (p < 0.0001, each). During follow-up, 164 (17.2%) patients underwent reablation due to the planned staged procedure, 18 (1.9%) patients underwent surgical myectomy, and 49 (5.10%) patients underwent cardioverter-defibrillator implantation. Seventy patients died: causes of death were identified as noncardiovascular in 50, stroke related in 6, and cardiac in 14 patients. Estimated 5-year survival was 95.8%, estimated 5-year survival free of cardiovascular events was 98.6%, and an estimated 5-year survival free of cardiac events was 98.9%. Corresponding values at 10 years were 88.3%, 96.5%, and 97.0%, and at 15 years were 79.7%, 92.3%, and 96.5%. CONCLUSIONS:In this study, PTSMA could be proofed as a safe procedure with ongoing symptomatic improvement and excellent long-term survival. Therefore, PTSMA is a reasonable alternative to surgical myectomy in HOCM. 10.1016/j.jacc.2018.09.064
    Septal Reduction Therapy for Hypertrophic Obstructive Cardiomyopathy. Fifer Michael A Journal of the American College of Cardiology 10.1016/j.jacc.2018.10.013
    Survival Differences in Women and Men After Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy. Meghji Zahara,Nguyen Anita,Fatima Benish,Geske Jeffrey B,Nishimura Rick A,Ommen Steve R,Lahr Brian D,Dearani Joseph A,Schaff Hartzell V JAMA cardiology Importance:Recent data indicate that women with hypertrophic cardiomyopathy (HCM) are older and more symptomatic at presentation and have worse clinical outcomes than men. However, to our knowledge, there are no large studies of the association of patient sex with outcomes after surgical myectomy. Objective:To analyze preoperative characteristics and overall survival of women and men undergoing septal myectomy for obstructive HCM. Design, Setting, and Participants:This retrospective, single-center study included the clinical data of adult patients who underwent septal myectomy from January 1961 through April 2016. Data analysis occurred from December 2017 to December 2018. Exposures:Septal myectomy. Main Outcomes and Measures:Survival. Results:A total of 2506 adults were included; 1379 patients (55.0%) were men. At the time of surgery, women were older, with median (IQR) age of 59.5 (46.6-68.2) years vs 52.9 (42.9-62.7) years in men (P < .001). Women were more likely to have New York Heart Association class III or IV status at presentation (women, 1023 [90.8%]; men, 1169 [84.8%]; P < .001) and more severe obstructive physiology, as reflected in higher resting left ventricular outflow tract gradients (women, 67.0 [36.0-97.0] mm Hg; men, 50.0 [23.0-81.0] mm Hg; P < .001). Women also had a greater likelihood of having moderate or severe mitral regurgitation (606 [55.2%]) than men (581 [43.1%]; P < .001) and higher right ventricular systolic pressure (women, 36.0 [30.0-46.0] mm Hg; men, 33.0 [28.0-39.0] mm Hg; P < .001). The unadjusted overall survival was lower in women, corresponding to a median 3.9-year shorter survival than men (median [IQR] survival time: women, 18.2 [12.1-27.2] years; men, 22.1 [15.1-32.5] years; P < .001). In a multivariable Cox regression analysis, however, the association between sex and mortality was attenuated and not significant after controlling for other baseline variables (hazard ratio, 0.98 [95% CI, 0.76-1.26]; P = .86). Among the covariates in the model, older age at surgery (adjusted hazard ratio [aHR], 3.09 [95% CI, 2.12-4.52]; P < .001), higher body mass index (aHR, 1.22 [95% CI, 0.90-1.66]; P < .001), greater NYHA class (aHR, 2.31 [95% CI, 1.03-5.15]; P = .04), and presence of diabetes prior to surgery (aHR, 1.57 [95% CI, 1.10-2.24]; P = .01) were each independently associated with increased mortality. Operations performed later in the study period (2013 vs 2004) were associated with decreased mortality (aHR, 0.82 [95% CI, 0.55-1.22]; P = .001). Conclusions and Relevance:In this large cohort of surgical patients with obstructive HCM, we observed significant differences at clinical presentation between women and men, in that women were older and more symptomatic. However, after adjustment for important baseline prognostic factors, there was no survival difference after septal myectomy by sex. Improved care of women with obstructive HCM should focus on early identification of disease and prompt surgical referral of appropriate patients who do not respond to medical treatment. 10.1001/jamacardio.2019.0084
    Hypertrophic Cardiomyopathy in Noonan Syndrome Treated by MEK-Inhibition. Andelfinger Gregor,Marquis Christopher,Raboisson Marie-Josée,Théoret Yves,Waldmüller Stephan,Wiegand Gesa,Gelb Bruce D,Zenker Martin,Delrue Marie-Ange,Hofbeck Michael Journal of the American College of Cardiology 10.1016/j.jacc.2019.01.066