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A case of arrhythmogenic right ventricular cardiomyopathy presenting with progressive right ventricular failure and recurrent multifocal monomorphic ventricular tachycardia during 15 years of follow-up.

ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a multigenic form of cardiomyopathy characterized by myocardial loss and fibrofatty replacement mainly in the right ventricle. Progressive right ventricular dysfunction, ventricular arrhythmias, and sudden cardiac death are the clinical picture of this disease. Despite its clinical importance as a cause of sudden death, ARVC is likely to be under-recognized. In case reports about ARVC, disease characteristics such as arrhythmias, images, and genes are described in fragments. Little is reported about the long-term course of ARVC in the same patient. In this report, we present a case of a 68-year-old male who was diagnosed with ARVC after his first episode of ventricular tachycardia. Both mechanical and electrical progression were seen during the 15 years of follow-up, requiring the modification of disease management. This report could help improve the understanding of this rare disease, and the way of its management. .

SUBMITTER: Kuwabara K

PROVIDER: S-EPMC6279640 | biostudies-other | 2014 Dec

REPOSITORIES: biostudies-other

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A case of arrhythmogenic right ventricular cardiomyopathy presenting with progressive right ventricular failure and recurrent multifocal monomorphic ventricular tachycardia during 15 years of follow-up.

Kuwabara Kensuke K Hirata Kazuhito K Wake Minoru M Takahashi Takanori T Nakazato Jun J Yagi Nobuhito N Fukuyama Asako A

Journal of cardiology cases 20140926 6

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a multigenic form of cardiomyopathy characterized by myocardial loss and fibrofatty replacement mainly in the right ventricle. Progressive right ventricular dysfunction, ventricular arrhythmias, and sudden cardiac death are the clinical picture of this disease. Despite its clinical importance as a cause of sudden death, ARVC is likely to be under-recognized. In case reports about ARVC, disease characteristics such as arrhythmias, images, ...[more]

PMID: 30534247

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Project description:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient's sudden cardiac death risk is sufficient to justify placement of an implantable cardioverter-defibrillator. The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people and that sudden death may be the first manifestation of the disease. This decision is particularly important because these are often young patients who are expected to live for many years. Although an implantable cardioverter-defibrillator can save lives in individuals with this disease, it is also well recognized that implantable cardioverter-defibrillator therapy is associated with both short- and long-term complications. Decisions about the placement of an implantable cardioverter-defibrillator are based on an estimate of a patient's risk of sudden cardiac death, as well as their preferences and values. The primary purpose of this article is to provide a review of the literature that concerns risk stratification in patients with ARVC and to place this literature in the framework of the 3 authors' considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include electric instability, including the frequency of premature ventricular contractions and sustained ventricular arrhythmia; proband status; extent of structural disease; cardiac syncope; male sex; the presence of multiple mutations or a mutation in TMEM43; and the patient's willingness to restrict exercise and to eliminate participation in competitive or endurance exercise.

Dataset Information

A case of arrhythmogenic right ventricular cardiomyopathy presenting with progressive right ventricular failure and recurrent multifocal monomorphic ventricular tachycardia during 15 years of follow-up.

Publications

A case of arrhythmogenic right ventricular cardiomyopathy presenting with progressive right ventricular failure and recurrent multifocal monomorphic ventricular tachycardia during 15 years of follow-up.

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