Sudden cardiac death in hypertrophic cardiomyopathy: Improved risk stratification strategies are needed

Publication date: December 2019Source: Revista Portuguesa de Cardiologia (English Edition), Volume 38, Issue 12Author(s): Inês Cruz
Source: Revista Portuguesa de Cardiologia - Category: Cardiology Source Type: research

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Abstract The histone demethylase JMJD family is involved in various physiological and pathological functions. However, the roles of JMJD1A in the cardiovascular system remain unknown. Here, we studied the function of JMJD1A in cardiac hypertrophy. The mRNA and protein levels of JMJD1A were significantly downregulated in the hearts of human patients with hypertrophic cardiomyopathy and the hearts of C57BL/6 mice underwent cardiac hypertrophy induced by transverse aortic constriction (TAC) surgery or isoproterenol (ISO) infusion. In neonatal rat cardiomyocytes (NRCMs), siRNA-mediated JMJD1A knockdown facilitated ISO...
Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
AbstractHypertrophic cardiomyopathy (HCM) is a prevalent cardiomyopathy in children, with variable etiologies, phenotypes, and associated syndromic genetic disorders (GD). The spectrum of evaluation in this heterogeneous population has not been well described. We aimed to describe mortality and medical management in the pediatric HCM population, and compare HCM pediatric patients with GD to those without GD. Children (
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
Source: Journal of Echocardiography - Category: Cardiology Source Type: research
Condition:   Hypertrophic Cardiomyopathy Intervention:   Device: Cardioverter-defibrillator Sponsor:   Poznan University of Medical Sciences Completed
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
by Sathiya N. Manivannan, Sihem Darouich, Aida Masmoudi, David Gordon, Gloria Zender, Zhe Han, Sara Fitzgerald-Butt, Peter White, Kim L. McBride, Maher Kharrat, Vidu Garg Hypertrophic cardiomyopathy (HCM) is characterized by the thickening of the ventricular muscle without dilation and is often associated with dominant pathogenic variants in cardiac sarcomeric protein genes. Here, we report a family with two infants diagnosed with infantile-onset HCM and mitral val ve dysplasia that led to death before one year of age. Using exome sequencing, we discovered that one of the affected children had a homozygous frameshift vari...
Source: PLoS Genetics - Category: Genetics & Stem Cells Authors: Source Type: research
Condition:   Hypertrophic Cardiomyopathy Intervention:   Device: Cardioverter-defibrillator Sponsor:   Poznan University of Medical Sciences Completed
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Hypertrophic cardiomyopathy (HCM) severity greatly varies among patients even with the same HCM gene mutations. This variation is largely regulated by modifier gene(s), which, however, remain largely unknown. The current study is aimed to identify modifier genes using BXD strains, a large murine genetic reference population (GRP) derived from crosses between C57BL/6  J (B6) and D2 DBA/2 J (D2) mice. D2 mice is a natural model that carry the genetic basis and phenotypes of HCM.
Source: Journal of Molecular and Cellular Cardiology - Category: Cytology Authors: Source Type: research
Although it is the most common inherited monogenic cardiac disorder, affecting up to 0.5% of the population, there is a gap in the recognition of this condition.ACC.org
Source: Medscape Cardiology Headlines - Category: Cardiology Tags: Cardiology Article Source Type: news
ConclusionsIn high ‐risk HCM patients, the occurrence of polymorphic NSVT and of NSVT with multiple morphologies carries a high risk for ICD interventions. Sustained VTs tend to recur with the same morphology of previous long‐lasting NSVTs.This article is protected by copyright. All rights reserved.
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ARTICLES Source Type: research
This article is protected by copyright. All rights reserved.
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: EDITORIAL ‐ INVITED Source Type: research
More News: Cardiology | Cardiomyopathy | Heart | Hypertrophic Cardiomyopathy