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https://www.readbyqxmd.com/read/28912182/hypertrophic-obstructive-cardiomyopathy-surgical-myectomy-and-septal-ablation
#1
REVIEW
Rick A Nishimura, Hubert Seggewiss, Hartzell V Schaff
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...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28874324/complex-phenotype-linked-to-a-mutation-in-exon-11-of-the-lamin-a-c-gene-hypertrophic-cardiomyopathy-atrioventricular-block-severe-dyslipidemia-and-diabetes
#2
Ana Rita G Francisco, Inês Santos Gonçalves, Fátima Veiga, Mónica Mendes Pedro, Fausto J Pinto, Dulce Brito
The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Herein we describe a new phenotype associated with a mutation in exon 11 of the LMNA gene: hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes...
September 2, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28856513/emerging-pharmacologic-and-structural-therapies-for-hypertrophic-cardiomyopathy
#3
REVIEW
Daniel J Philipson, Eugene C DePasquale, Eric H Yang, Arnold S Baas
Hypertrophic cardiomyopathy is the most common inherited heart disease. Although it was first described over 50 years ago, there has been little in the way of novel disease-specific therapeutic development for these patients. Current treatment practice largely aims at symptomatic control using old drugs made for other diseases and does little to modify the disease course. Septal reduction by surgical myectomy or percutaneous alcohol septal ablation are well-established treatments for pharmacologic-refractory left ventricular outflow tract obstruction in hypertrophic cardiomyopathy patients...
August 31, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#4
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
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...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28762919/-effectiveness-of-extended-myectomy-in-patients-with-hypertrophic-cardiomyopathy-with-midventricular-obstruction
#5
A V Bogachev-Prokophiev, S I Zheleznev, M S Fomenko, R M Sharifulin, A V Afanasyev, O Yu Malakhova, A M Karaskov
PURPOSE: to assess effectiveness and safety of extended myectomy performed in patients with hypertrophic obstructive cardiomyopathy (HOCM) with midventricular obstruction. MATERIAL AND METHODS: Between 2010 and 2013 185 HOCM patients were operated for left ventricular outflow tract (LVOT) obstruction. Among these patients 32 had midventricular obstruction. Their age was 22-74 (mean 51.9+/-14.2) years. Mean peak gradient across LVOT was 89.1+/-20.4 mm Hg, thickness of interventricular septum was 26...
May 2017: Kardiologiia
https://www.readbyqxmd.com/read/28728695/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of-balance
#6
REVIEW
Paul Sorajja
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...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28728694/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of%C3%A2-endorsement
#7
REVIEW
Max Liebregts, Pieter A Vriesendorp, Jurrien M Ten Berg
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...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28681495/underappreciated-occurrence-of-discrete-subaortic-membranes-producing-left-ventricular-outflow-obstruction-in-hypertrophic-cardiomyopathy
#8
Muhil Kannappan, Barry J Maron, Hassan Rastegar, Natesa G Pandian, Martin S Maron, Ethan J Rowin
Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms...
August 2017: Echocardiography
https://www.readbyqxmd.com/read/28642712/investigations-into-the-sarcomeric-protein-and-ca-2-regulation-abnormalities-underlying-hypertrophic-cardiomyopathy-in-cats-felix-catus
#9
Andrew E Messer, Jasmine Chan, Alex Daley, O'Neal Copeland, Steven B Marston, David J Connolly
Hypertrophic cardiomyopathy (HCM) is the most common single gene inherited cardiomyopathy. In cats (Felix catus) HCM is even more prevalent and affects 16% of the outbred population and up to 26% in pedigree breeds such as Maine Coon and Ragdoll. Homozygous MYBPC3 mutations have been identified in these breeds but the mutations in other cats are unknown. At the clinical and physiological level feline HCM is closely analogous to human HCM but little is known about the primary causative mechanism. Most identified HCM causing mutations are in the genes coding for proteins of the sarcomere...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28609808/resection-of-subaortic-membrane-for-discrete-subaortic-stenosis
#10
Sachin Talwar, Abhishek Anand, Saurabh Kumar Gupta, Sivasubramanian Ramakrishnan, Shyam Sunder Kothari, Anita Saxena, Rajnish Juneja, Shiv Kumar Choudhary, Balram Airan
BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death...
July 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28483207/usefulness-of-preoperative-transforming-growth-factor-beta-to-predict-new-onset-atrial-fibrillation-after-surgical-ventricular-septal-myectomy-in-patients-with-obstructive-hypertrophic-cardiomyopathy
#11
Ying Guo, Xi Wu, Xinxin Zheng, Jie Lu, Shuiyun Wang, Xiaohong Huang
Postoperative atrial fibrillation (AF) occurs frequently after cardiac surgery and contributes significantly to mortality. Transforming growth factor-beta (TGF-β) is associated with postoperative AF after coronary artery bypass grafting and valve surgery. We performed a prospective study to evaluate the role of TGF-β as a predictor of AF after myectomy. A total of 109 consecutive obstructive hypertrophic cardiomyopathy patients without previous AF who underwent myectomy were identified. We measured plasma TGF-β levels before surgery, monitored heart rhythm until discharge, and followed patients for a mean of 36 ± 10 months...
July 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28396501/mechanism-of-progressive-heart-failure-and-significance-of-pulmonary-hypertension-in-obstructive-hypertrophic-cardiomyopathy
#12
Michele Covella, Ethan J Rowin, Nicholas S Hill, Ioana R Preston, Alberto Milan, Alexander R Opotowsky, Barry J Maron, Martin S Maron, Bradley A Maron
BACKGROUND: There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure. METHODS AND RESULTS: To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status...
April 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28363351/comparison-of-ventricular-septal-measurements-in-hypertrophic-cardiomyopathy-patients-who-underwent-surgical-myectomy-using-multimodality-imaging-and-implications-for-diagnosis-and-management
#13
COMPARATIVE STUDY
Dermot Phelan, Brett W Sperry, Paaladinesh Thavendiranathan, Patrick Collier, Zoran B Popović, Harry M Lever, Nicholas G Smedira, Milind Y Desai
Accurate and reproducible quantification of ventricular septal (VS) thickness in hypertrophic cardiomyopathy (HC) is essential for diagnosis, surgical planning, and risk stratification. We sought to compare VS thickness measurements using transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR) in patients with HC. Prospectively reported measurements of VS thickness were compared using analysis of variance and Bland-Altman plots in 90 consecutive patients with HC who underwent a TTE, TEE, and CMR within 3 months...
May 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28329292/surgical-septal-myectomy-or-alcohol-septal-ablation-which-approach-offers-better-outcomes-for-patients-with-hypertrophic-obstructive-cardiomyopathy
#14
Shi Sum Poon, Mark Field, Dhiraj Gupta, Duke Cameron
A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether surgical septal myectomy (SM) is more beneficial than alcohol septal ablation (ASA) in patients with hypertrophic obstructive cardiomyopathy. Altogether 218 articles were found using the reported search, of which 15 studies represented the best evidence to answer the clinical question. There were 14 observational studies and 1 meta-analysis study. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these articles are tabulated...
June 1, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329110/mid-term-outcomes-of-concomitant-surgical-ablation-of-atrial-fibrillation-in-patients-undergoing-cardiac-surgery-for-hypertrophic-cardiomyopathy%C3%A2
#15
Elisabetta Lapenna, Alberto Pozzoli, Michele De Bonis, Giovanni La Canna, Teodora Nisi, Simona Nascimbene, Luca Vicentini, Stefania Di Sanzo, Benedetto Del Forno, Davide Schiavi, Ottavio Alfieri
OBJECTIVES: Atrial fibrillation (AF) is common in patients with hypertrophic cardiomyopathy (HCM) and it is often poorly tolerated because of loss of atrial contraction and reduced filling time with rapid ventricular rates. Restoring sinus rhythm is of great clinical benefit to HCM patients. Very few data exist regarding surgical ablation of concomitant AF in this setting. The aim of this study was to evaluate the mid-term outcome of surgical AF ablation in patients who underwent cardiac surgery due to HCM...
June 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28301315/time-and-age-dependent-decrease-of-nt-probnp-after-septal-myectomy-in-hypertrophic-obstructive-cardiomyopathy
#16
Hao Cui, Xi Wu, Shuiyun Wang, Bing Tang, Changsheng Zhu, Haibo Chen, Xiaohui Zhou, Rong Wu, Qinjun Yu, Xiaohong Huang
BACKGROUND: NT-proBNP level is a predictive factor in hypertrophic cardiomyopathy patients, in which left ventricular outflow tract obstruction contributes to an increased NT-proBNP level. However, studies regarding the influence of septal myectomy on NT-proBNP level in hypertrophic obstructive cardiomyopathy are lacking. METHODS: One hundred and eighty-two patients who underwent septal myectomy in Fuwai Hospital from May 2011 to March 2016 and were included in the present study...
August 28, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28275421/treating-a-structural-heart-disease-using-a-non-structural-approach-role-of-cardiac-pacing-in-hypertrophic-cardiomyopathy
#17
Bernard Benjamin P Albano, Erdie C Fadreguilan, Jeffrey M Chua, James Ho, Ana Beatriz Medrano
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease characterized by a thickened non-dilated ventricle in the absence of another cardiac or systemic condition. Its most important hemodynamic consequence is left ventricular outflow tract (LVOT) obstruction. The primary management strategy of this condition is surgical septal myectomy, but an acceptable alternative treatment in patients who are not suitable for (or who refuse) surgery is alcohol septal ablation (ASA). However, in patients with unfavorable coronary anatomy which precludes ASA (i...
February 2017: Cardiology Research
https://www.readbyqxmd.com/read/28268009/why-we-need-more-septal-myectomy-surgeons-an-emerging-recognition
#18
EDITORIAL
Barry J Maron, Joseph A Dearani, Martin S Maron, Steve R Ommen, Hassan Rastegar, Rick A Nishimura, Daniel G Swistel, Mark V Sherrid, Anthony Ralph-Edwards, Harry Rakowski, Nicholas G Smedira, Ethan J Rowin, Milind Y Desai, Harry M Lever, Paolo Spirito, Paolo Ferrazzi, Hartzell V Schaff
No abstract text is available yet for this article.
February 1, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28262912/hypertrophic-angulation-deformity-of-the-basal-interventricular-septum-combined-with-abnormality-of-the-papillary-muscle-and-chordae-tendineae
#19
Yi Wang, Luwei Ye, Lixue Yin, Jie Zeng
A Chinese woman was admitted to our hospital because of syncope. Transthoracic echocardiography revealed a hypertrophic basal interventricular septum of 15 mm with a sharp angle protruding into the left ventricular outflow tract. Moreover, an anomalous anterolateral papillary muscle (maximum width of 11 mm) was inserted into the left ventricular outflow tract, with short chordae tendineae connecting both basal interventricular septum and anterior leaflet of the mitral valve. All of these abnormalities resulted in a left ventricular outflow gradient of 136 mmHg...
January 23, 2017: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/28247219/concomitant-septal-myectomy-during-minimally-invasive-aortic-valve-replacement-through-a-right-mini-thoracotomy-for-the-treatment-of-aortic-stenosis-with-systolic-anterior-motion-of-the-mitral-valve
#20
Toshinori Totsugawa, Kota Suzuki, Arudo Hiraoka, Kentaro Tamura, Hidenori Yoshitaka, Taichi Sakaguchi
This paper reports concomitant septal myectomy during minimally invasive aortic valve replacement through a right mini-thoracotomy. A 76-year-old woman was diagnosed as having aortic stenosis with systolic anterior motion of the mitral valve. The bulging septum was exposed by pulling up a traction suture placed at the annulus of the right coronary cusp; the anomalous chordae and muscle bundles attached to the septum were also resected. By setting an adequate intercostal thoracotomy and placing a traction suture, concomitant myectomy was safely performed even through a right mini-thoracotomy...
March 1, 2017: General Thoracic and Cardiovascular Surgery
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