Arrhythmogenic right ventricular cardiomyopathy/dysplasia: An update

Hugh Calkins, Frank Marcus

Research output: Contribution to journalReview articlepeer-review

28 Scopus citations


Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). The diagnosis is based on the International Task Force criteria. Cardiologists may not be aware of these diagnostic criteria for ARVC/D and may place too much importance on the results of MRI imaging of the right ventricle. Patients with ARVC/D usually have an abnormal 12-lead electrocardiogram, abnormal echocardiogram, and ventricular arrhythmias with a left bundle branch block morphology. If noninvasive testing suggests ARVC/D, invasive testing with an RV angiogram, RV biopsy, and electrophysiologic study is recommended. Once a diagnosis of ARVC/D is established, the main treatment decision involves whether to implant an implantable cardioverter-defibrillator. We also recommend treatment with β blockers. Patients with ARVC/D are encouraged to avoid competitive athletics. Recent advances in the understanding of the genetic basis of ARVC/D have revealed that ARVC/D is a disease of desmosomal dysfunction.

Original languageEnglish (US)
Pages (from-to)367-375
Number of pages9
JournalCurrent Cardiology Reports
Issue number5
StatePublished - 2008

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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