Publication date: Available online 25 March 2020Source: IJC Heart &VasculatureAuthor(s): Murillo de Oliveira Antunes, Thiago Luis Scudeler
This study examined the prevalence of WPW and associated outcomes in a cohort of patients with Danon Disease.
Danon Disease (DD) is a rare X-linked disorder due to mutations in the Lysosomal Associated Membrane Protein 2 (LAMP-2) gene. Patients present with cardiac manifestations of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). The goal of this study is to characterize the longitudinal progression of hypertrophic phenotypes and descriptions of left ventricular strain in DD.
The treatment of end stage hypertrophic cardiomyopathy (HCM) is sometimes complicated because some cases of HCM are not suitable for LVAD. Scarce data exist on outcomes of continuous-flow (CF) LVAD support in these challenging patient cohorts. We evaluated the clinical outcomes of LVAD therapy for patients with HCM.
HCM patients may have increased waitlist (WL) mortality. Given the recent change in prioritizing patients with restrictive and hypertrophic cardiomyopathy requiring mechanical support, we sought to assess whether the policy change would affect the waitlist and post-transplant outcomes in HCM pts.
AbstractA 4-year-old boy with Noonan syndrome accompanied by hypertrophic obstructive cardiomyopathy presented with refractory heart failure owing to severe left ventricular outflow obstruction and mitral regurgitation. He underwent mitral valve replacement and trans-mitral myectomy. The pathology of the resected myocardium was consistent with hypertrophic cardiomyopathy, and the mitral valve leaflets were severely degenerated. Compared with a control patient who is a non-syndromic 4-year-old boy with hypertrophic obstructive cardiomyopathy, mitral pathology was much more severe in Noonan syndrome. Regarding trans-mitral m...
Response by Weissler-Snir and Dorian to Letter Regarding Article, "Hypertrophic Cardiomyopathy-Related Sudden Cardiac Death in Young People in Ontario". Circulation. 2020 Mar 31;141(13):e703-e704 Authors: Weissler-Snir A, Dorian P PMID: 32223681 [PubMed - in process]
Letter by Madias Regarding Article, "Hypertrophic Cardiomyopathy-Related Sudden Cardiac Death in Young People in Ontario". Circulation. 2020 Mar 31;141(13):e701-e702 Authors: Madias JE PMID: 32223672 [PubMed - in process]
Abstract Hypertrophic cardiomyopathy (HCM) is an intractable disease that causes heart failure mainly due to unexplained severe cardiac hypertrophy and diastolic dysfunction. HCM, which occurs in 0.2% of the general population, is the most common cause of sudden cardiac death in young people. HCM has been studied extensively using molecular genetic approaches. Genes encoding cardiac β-myosin heavy chain, cardiac myosin-binding protein C, and troponin complex, which were originally identified as causative genes, were subsequently reported to be frequently implicated in HCM. Indeed, HCM has been considered a di...
This article is protected by copyright. All rights reserved. PMID: 32215921 [PubMed - as supplied by publisher]
Publication date: Available online 23 March 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Paul J.M. Wijnker, Jolanda van der Velden