Genetic Arrhythmias Complicating Patients with Dilated Cardiomyopathy
Sudden cardiac death due to malignant arrhythmias is a common cause of death in dilated cardiomyopathy (DCM). Whether genetic variants increase the risk of arrhythmias in DCM is unknown.
Conclusions: We report the identification of a novel mutation in RBM20 by WES in a large pedigree with DCM. PMID: 31583969 [PubMed - as supplied by publisher]
Abstract The deletion of Arginine 14 of the phosholamban gene (PLN p.R14del) is associated with the pathogenesis of an inherited form of cardiomyopathy with prominent arrhythmias. Patients carrying the PLN R14del mutation are at risk of developing dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy. Although the genetic etiology is well defined, the molecular mechanism underlying the pathogenesis of PLN R14del-cardiomyopathy is unknown. Our CURE PLaN network, funded by the Foundation Leducq, will bring together leading scientists, clinicians, and patients to elucidate the genotype-phenotype r...
In this study we aimed to investigate left atrial (LA) function, measured from routine cine cardiovascular magnetic resonance imaging, to determine its value for the prediction of sudden cardiac death (SCD) or appropriate implantable cardioverter defibrillator (ICD) shock in patients who received primary prevention ICD implantation.MethodsWe studied 203 patients with ischemic or idiopathic nonischemic dilated cardiomyopathy who underwent cardiovascular magnetic resonance imaging before primary prevention ICD implantation. LA volumes were measured at end-diastole and end-systole from 4- and 2-chamber cine images, and LA emp...
Dilated cardiomyopathy (DCM) is the most common cardiomyopathy in children. Patients with severe cardiac dysfunction are thought to be at risk for sudden cardiac arrest (SCA). Following diagnosis, a period of medical optimization is recommended prior to permanent implantable cardioverter-defibrillator (ICD) implantation. Wearable cardioverter defibrillators (WCD) provide an option for arrhythmia protection as an outpatient during this optimization.
Dilated cardiomyopathy (DCM) is the most common cardiomyopathy in children. Patients with severe cardiac dysfunction are thought to be at risk of sudden cardiac arrest (SCA). After diagnosis, a period of medical optimization is recommended before permanent implantable cardioverter-defibrillator (ICD) implantation. Wearable cardioverter-defibrillators (WCDs) provide an option for arrhythmia protection as an outpatient during this optimization.
ConclusionCatheter ablation of VF through „De‐Networking” of the Purkinje system in patients without overt arrhythmia substrate or trigger appears safe and effective and will require further study in a larger patient cohort.This article is protected by copyright. All rights reserved
Publication date: Available online 15 August 2019Source: American Heart JournalAuthor(s): Fatima Ali-Ahmed, Frederik Dalgaard, Sana M. Al-KhatibAbstractMyocarditis is a major cause of sudden cardiac death (SCD) and dilated cardiomyopathy (DCM) in young adults. Cardiac magnetic resonance (CMR) is the established tool for the diagnosis of myocarditis, and late gadolinium enhancement (LGE) detected on CMR imaging is the strongest independent predictor of SCD, all-cause mortality, and cardiac mortality. Several other factors have been associated with SCD or cardiac transplantation including New York Heart Association functiona...
Background and Aims: Duchenne Muscular Dystrophy (DMD) is a progressive muscle-wasting disease. Besides skeletal muscle degeneration, important sources of morbidity and mortality are dilated cardiomyopathy, arrhythmias and vascular dysfunction. There is substantial evidence that Tenascin C (TN-C) plays role in left ventricular (LV) remodelling and its serum levels are associated with the severity of LV dysfunction in patients with heart failure. Additionally, recent studies demonstrate that endothelial dysfunction may contribute to the progression of dilated cardiomyopathy.
Conclusions: The clinical course of patients with ICM and DCM treated with CRT-D differs significantly during long-term follow-up, with increased mortality and incidence of ICD therapies in ICM patients. PMID: 31360179 [PubMed]
Ventricular tachycardia (VT) and VF occur mainly in people with impaired cardiac function and/or ischaemic heart disease, and account for the majority of sudden cardiac deaths worldwide.1 Treatment with anti-arrhythmic drugs such as amiodarone may be at best neutral in terms of mortality and carries significant long-term risks.2,3 While ICDs significantly improve survival for patients with significantly impaired left ventricular ejection fraction (LVEF), the devices also carry risk