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hypertrophic obstructive cardiomyopathy

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https://www.readbyqxmd.com/read/28427417/novel-trigenic-cacna1c-des-mypn-mutations-in-a-family-of-hypertrophic-cardiomyopathy-with-early-repolarization-and-short-qt-syndrome
#1
Yanhong Chen, Hector Barajas-Martinez, Dongxiao Zhu, Xihui Wang, Chonghao Chen, Ruijuan Zhuang, Jingjing Shi, Xueming Wu, Yijia Tao, Weidong Jin, Xiaoyan Wang, Dan Hu
BACKGROUND: Hypertrophic cardiomyopathy (HCM) patients with early repolarization (ER) pattern are at higher risk of ventricular arrhythmia, yet the genetic background of this situation has not been well investigated. Here we report novel trigenic mutations detected in a Chinese family of obstructive HCM with ER and short QT syndrome (SQTS). METHODS: Proband and family members underwent detailed medical assessments. DNAs were extracted from peripheral blood leukocytes for genetic screening with next generation method...
April 20, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28424545/efficacy-and-safety-of-alcohol-septal-ablation-in-patients-over-65-years-old-with-obstructive-hypertrophic-cardiomyopathy
#2
Laila Cheddadi, Olivier Lairez, Thibault Lhermusier, Francisco Campelo-Parada, Michel Galinier, Didier Carrié, Nicolas Boudou
BACKGROUND: The performance of alcohol septal ablation (ASA) in elderly symptomatic patients with drug-refractory obstructive hypertrophic cardiomyopathy is still to be confirmed. The objective of this study was to compare the efficacy and safety of ASA in patients under and over 65 years old. METHODS AND RESULTS: Fifty-one consecutive patients with obstructive hypertrophic cardio-myopathy who underwent ASA were retrospectively included and reviewed for in-hospital major acute cardiac events and follow-up...
2017: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/28396501/mechanism-of-progressive-heart-failure-and-significance-of-pulmonary-hypertension-in-obstructive-hypertrophic-cardiomyopathy
#3
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/28393803/echocardiography-for-hypertrophic-obstructive-cardiomyopathy
#4
Kalpna Irpachi, K Ashok Kumar, Poonam Malhotra Kapoor
No abstract text is available yet for this article.
April 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28391581/contribution-of-mitral-valve-leaflet-length-and-septal-wall-thickness-to-outflow-tract-obstruction-in-patients-with-hypertrophic-cardiomyopathy
#5
Kareem Morant, Yoko Mikami, Immaculate Nevis, David McCarty, John Stirrat, David Scholl, Martin Rajchl, Peter Giannoccaro, Louis Kolman, Bobby Heydari, Carmen Lydell, Andrew Howarth, Andrew Grant, James A White
We sought to examine whether elongation of the mitral valve leaflets in patients with hypertrophic cardiomyopathy (HCM) is synergistic to septal wall thickness (SWT) in the development of left ventricular outflow tract obstruction (LVOTO). HCM is a common genetic cardiac disease characterized by asymmetric septal hypertrophy and predisposition towards LVOTO. It has been reported that elongation of the mitral valve leaflets may be a primary phenotypic feature and contribute to LVOTO. However, the relative contribution of this finding versus SWT has not been studied...
April 8, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28379420/improvements-in-left-ventricular-twist-mechanics-following-myectomy-for-hypertrophic-cardiomyopathy-with-mid-ventricular-obstruction
#6
Srilakshmi M Adhyapak, Prahlad G Menon, V Rao Parachuri
We aimed to evaluate left ventricle twist mechanics in mid-ventricular obstructive and apical type of hypertrophic cardiomyopathy and changes induced by myectomy. We studied 3 consecutive patients by cardiac magnetic resonance preoperatively and 6 weeks after myectomy. We calculated the apical and basal rotations at the base and apex respectively. All 3 patients underwent myectomy by the standard described technique. The basal rotations remained the same, while there was an improvement in the maximal apical rotation from 0...
March 31, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28378353/reduction-of-left-ventricular-outflow-tract-obstruction-with-transcatheter-mitral-valve-repair
#7
Megan Coylewright, Elizabeth S O'Neill, John F Robb, Jock N McCullough, Cassie M Tighe, Joshua M Callahan, Timothy A Beaver
Many patients with severe mitral regurgitation cannot undergo conventional mitral valve surgery due to prohibitive surgical risk and are candidates for transcatheter repair with an edge-to-edge technique. Prior reports suggest efficacy with this approach for mitral regurgitation due to hypertrophic cardiomyopathy with left ventricular outflow obstruction. We present a case report of transcatheter mitral valve repair for posterior leaflet prolapse with concomitant left ventricular outflow tract obstruction due to systolic anterior motion of the mitral valve in the absence of hypertrophic cardiomyopathy...
April 2017: Echocardiography
https://www.readbyqxmd.com/read/28377231/prevalence-and-impact-of-coexistent-bicuspid-aortic-valve-in-hypertrophic-cardiomyopathy
#8
Ratnasari Padang, Bernard J Gersh, Steve R Ommen, Jeffrey B Geske
BACKGROUND: The association between bicuspid aortic valve (BAV) and hypertrophic cardiomyopathy (HCM) has been reported but its true prevalence is unknown. This study investigated the prevalence and clinical impact of coexistent BAV in a large referral HCM population. METHODS: Retrospective analysis of 3765 echocardiograms between 2004 and 2014 in 2640 consecutive patients with HCM was performed to assess for BAV. Patients with coexistent conditions were studied...
March 8, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28375659/cardiopulmonary-exercise-test-in-hypertrophic-cardiomyopathy
#9
Damiano Magri, Caterina Santolamazza
Understanding the functional limitation in hypertrophic cardiomyopathy, the most common inherited heart disease, is challenging. Beside the occurrence of disease-related complications, several factors are potential determinants of exercise limitation, including left ventricular hypertrophy, myocardial fiber disarray, left ventricular outflow tract obstruction, microvascular ischemia, and interstitial fibrosis. Furthermore, drugs commonly used in the daily management of these patients may interfere with exercise capacity, especially those with a negative chronotropic effect...
April 4, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28370331/left-atrial-function-and-phenotypes-in-asymmetric-hypertrophic-cardiomyopathy
#10
Yukari Kobayashi, Matthew Wheeler, Gherardo Finocchiaro, Miyuki Ariyama, Yuhei Kobayashi, Marco V Perez, David Liang, Tatiana Kuznetsova, Ingela Schnittger, Euan Ashley, Francois Haddad
BACKGROUND: Few studies have analyzed changes in left atrial (LA) function associated with different phenotypes of asymmetric hypertrophic cardiomyopathy (HCM). We sought to demonstrate the association of impairments in LA function with disease phenotype in patients with obstructive and nonobstructive HCM. METHODS: From Stanford Cardiomyopathy Registry, we randomly selected 50 age-/sex-matched healthy controls, 35 patients with nonobstructive HCM (HCM 1), 35 patients with obstructive HCM (HCM 2), and 35 patients with obstructive HCM requiring septal reduction therapy (HCM 3)...
April 1, 2017: Echocardiography
https://www.readbyqxmd.com/read/28336204/n-terminal-pro-brain-natriuretic-peptide-is-related-with-coronary-flow-velocity-reserve-and-diastolic-dysfunction-in-patients-with-asymmetric-hypertrophic-cardiomyopathy
#11
Milorad Tesic, Jelena Seferovic, Danijela Trifunovic, Ana Djordjevic-Dikic, Vojislav Giga, Ivana Jovanovic, Olga Petrovic, Jelena Marinkovic, Sanja Stankovic, Jelena Stepanovic, Arsen Ristic, Milan Petrovic, Nebojsa Mujovic, Bosiljka Vujisic-Tesic, Branko Beleslin, Vladan Vukcevic, Goran Stankovic, Petar Seferovic
BACKGROUND: The relations of elevated N-terminal pro-brain natriuretic peptide (NT-pro-BNP) and cardiac ischemia in hypertrophic cardiomyopathy (HCM) patients is uncertain. Therefore we designed the study with the following aims: (1) to analyze plasma concentrations of NT-pro-BNP in various subsets of HCM patients; (2) to reveal the correlations of NT-pro-BNP, myocardial ischemia, and diastolic dysfunction; (3) to assess predictors of the elevated plasma levels of NT-pro-BNP. METHODS AND RESULTS: In 61 patients (mean age 48...
March 20, 2017: 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
#12
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...
February 28, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329285/upright-treadmill-vs-semi-supine-bicycle-exercise-echocardiography-to-provoke-obstruction-in-symptomatic-hypertrophic-cardiomyopathy-a-pilot-study
#13
Patricia Reant, Maxence Dufour, Jerome Peyrou, Amelie Reynaud, Caroline Rooryck, Marina Dijos, Cecile Vincent, Claire Cornolle, Raymond Roudaut, Stephane Lafitte
Aims: Recent findings regarding hypertrophic cardiomyopathy (HCM) haemodynamics emphasized the relationship between symptoms, left ventricular outflow tract obstruction (LVOTO), and the preload condition as the venous return level. As various types of exercises have different effects on peripheral vascular beds, this study sought to compare upright treadmill exercise echocardiography (EE) to semi-supine bicycle EE in maximum provoked LVOTO in HCM patients. Methods and results: Semi-supine bicycle and upright treadmill EE were prospectively performed in HCM patients with New York Heart Association functional Class II...
February 17, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28328122/a-novel-patient-with-an-attenuated-costello-syndrome-phenotype-due-to-an-hras-mutation-affecting-codon-146-literature-review-and-update
#14
Annie Ting Gee Chiu, Gordon Ka-Chun Leung, Yoyo Wing-Yiu Chu, Karen W Gripp, Brian Hon-Yin Chung
De novo germline mutations in HRAS cause Costello syndrome, with >95% of the mutations causing Costello syndrome affecting amino acid position 12 (p.Gly12) or 13 (p.Gly13). We report on a patient with de novo missense mutation causing an amino acid change at codon 146 of HRAS, c.436G > C:p.Ala146Pro, who presented with subtle dysmorphic features, failure to thrive, global developmental delay, and hypertrophic obstructive cardiomyopathy. Mutations affecting codon 146 are observed in <1% of patients with Costello syndrome...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28320395/vasoproliferative-process-resembling-pulmonary-capillary-hemangiomatosis-in-a-cat
#15
J A Jaffey, K J Williams, I Masseau, M Krueger, C Reinero
BACKGROUND: Pulmonary capillary hemangiomatosis is a rare, vascular obstructive disorder that uniformly causes pulmonary arterial hypertension. Clinically, pulmonary capillary hemangiomatosis is indistinguishable from primary pulmonary arterial hypertension and histology is required for definitive diagnosis. The distinctive histologic feature of pulmonary capillary hemangiomatosis is non-malignant extensive proliferation of capillaries in the alveolar septae. Vasodilator treatment of humans with primary arterial hypertension due to pulmonary capillary hemangiomatosis can result in fatal acute pulmonary edema...
March 20, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28316173/-radiofrequency-septal-ablation-a-new-treatment-option-for-patients-with-hypertrophic-obstructive-cardiomyopathy
#16
R Chen, Z X Jiang, Q J Shan
No abstract text is available yet for this article.
March 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/28301315/time-and-age-dependent-decrease-of-nt-probnp-after-septal-myectomy-in-hypertrophic-obstructive-cardiomyopathy
#17
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...
March 16, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28290177/left-ventricular-outflow-tract-obstruction-and-systolic-anterior-motion-of-the-mitral-valve-in-the-absence-of-hypertrophic-cardiomyopathy
#18
Natasha Aleksova, Alice Wang, Christopher A Glover, Thierry Mesana, Girish Dwivedi
Systolic anterior motion of the mitral valve is a mechanism for the development of left ventricular outflow tract (LVOT) obstruction. While often associated with left ventricular hypertrophy (LVH), a case is reported of symptomatic LVOT obstruction due to intrinsic mitral valve pathology in the absence of hypertrophy or cardiomyopathy. This case highlights the importance of recognizing isolated mitral valve pathology as a treatable cause of LVOT obstruction.
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28275421/treating-a-structural-heart-disease-using-a-non-structural-approach-role-of-cardiac-pacing-in-hypertrophic-cardiomyopathy
#19
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/28271534/patient-after-renal-transplantation-with-syncope-role-of-echocardiography-in-upright-position
#20
Paweł Petkow Dimitrow, Danuta Sorysz, Rafał Hładij, Bernadeta Chyrchel, Barbara Dudzik, Władysław Sułowicz, Andrzej Surdacki
In Doppler echocardiography, testing left ventricular outflow tract (LVOT) gradient in the supine position (as is done in everyday practice) does not reflect the pathophysiology of this dynamic abnormality during the daily activities that trigger the symptoms (eg, syncope). LVOT obstruction is a dynamic phenomenon, strongly dependent on the left ventricular cavity size, geometric configuration of hypertrophy, load variability, contractility, and mitral apparatus abnormalities. LVOT gradient may develop not only in hypertrophic cardiomyopathy but also in various heart diseases...
March 8, 2017: Journal of Clinical Ultrasound: JCU
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