Can preload-reducing therapy prevent disease progression in arrhythmogenic right ventricular cardiomyopathy? Experimental evidence and concept for a clinical trial

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@article{6f8c8c32decf4566bee6d08c5e57f1f6,
title = "Can preload-reducing therapy prevent disease progression in arrhythmogenic right ventricular cardiomyopathy?: Experimental evidence and concept for a clinical trial",
abstract = "Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy and a leading cause of sudden cardiac death in a young population. ARVC is especially common in young athletes. Mutations in different desmosomal genes have been identified causing dysfunctional cell-cell contacts. Reduced myocardial expression of plakoglobin in cell-cell contact complexes appears to associate with disease manifestation in patients harbouring mutations within other cell-cell contact genes. Experimental data suggest that preload reduction may be a simple and effective intervention to prevent disease progression and ventricular arrhythmias in ARVC. This review discusses the potential effects of this innovative approach and describes the design of the first controlled trial of preload-reducing therapy in patients with ARVC.",
keywords = "Animals, Arrhythmogenic Right Ventricular Dysplasia, Clinical Trials as Topic, Disease Progression, Heart, Humans",
author = "Larissa Fabritz and Lisa Fortm{\"u}ller and Yu, {Ting Y} and Matthias Paul and Paulus Kirchhof",
note = "Copyright {\textcopyright} 2012 Elsevier Ltd. All rights reserved.",
year = "2012",
month = sep,
day = "5",
doi = "10.1016/j.pbiomolbio.2012.08.010",
language = "English",
volume = "110",
pages = "340--6",
journal = "Progress in Biophysics and Molecular Biology",
issn = "0079-6107",
publisher = "Elsevier",
number = "2-3",

}

RIS

TY - JOUR

T1 - Can preload-reducing therapy prevent disease progression in arrhythmogenic right ventricular cardiomyopathy?

T2 - Experimental evidence and concept for a clinical trial

AU - Fabritz, Larissa

AU - Fortmüller, Lisa

AU - Yu, Ting Y

AU - Paul, Matthias

AU - Kirchhof, Paulus

N1 - Copyright © 2012 Elsevier Ltd. All rights reserved.

PY - 2012/9/5

Y1 - 2012/9/5

N2 - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy and a leading cause of sudden cardiac death in a young population. ARVC is especially common in young athletes. Mutations in different desmosomal genes have been identified causing dysfunctional cell-cell contacts. Reduced myocardial expression of plakoglobin in cell-cell contact complexes appears to associate with disease manifestation in patients harbouring mutations within other cell-cell contact genes. Experimental data suggest that preload reduction may be a simple and effective intervention to prevent disease progression and ventricular arrhythmias in ARVC. This review discusses the potential effects of this innovative approach and describes the design of the first controlled trial of preload-reducing therapy in patients with ARVC.

AB - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy and a leading cause of sudden cardiac death in a young population. ARVC is especially common in young athletes. Mutations in different desmosomal genes have been identified causing dysfunctional cell-cell contacts. Reduced myocardial expression of plakoglobin in cell-cell contact complexes appears to associate with disease manifestation in patients harbouring mutations within other cell-cell contact genes. Experimental data suggest that preload reduction may be a simple and effective intervention to prevent disease progression and ventricular arrhythmias in ARVC. This review discusses the potential effects of this innovative approach and describes the design of the first controlled trial of preload-reducing therapy in patients with ARVC.

KW - Animals

KW - Arrhythmogenic Right Ventricular Dysplasia

KW - Clinical Trials as Topic

KW - Disease Progression

KW - Heart

KW - Humans

U2 - 10.1016/j.pbiomolbio.2012.08.010

DO - 10.1016/j.pbiomolbio.2012.08.010

M3 - Article

C2 - 22944071

VL - 110

SP - 340

EP - 346

JO - Progress in Biophysics and Molecular Biology

JF - Progress in Biophysics and Molecular Biology

SN - 0079-6107

IS - 2-3

ER -