Arrhythmogenic right ventricular cardiomyopathy/dysplasia

Hugh Calkins, Frank Marcus

Research output: Chapter in Book/Report/Conference proceedingChapter


Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy characterized clinically by ventricular arrhythmias, sudden death, and structural abnormalities of the right ventricle. Although structural involvement of the right ventricle predominates, a left dominant form (LDAC) has been described. Structural involvement of both ventricles is common in advanced disease. Due to significant heterogeneity in its manifestation, the diagnosis of ARVC/D is challenging and requires a multifaceted approach to patient evaluation. The recently published Revised Task Force Criteria provide clinicians with a validated model to improve the diagnosis of ARVC/D. The management of ARVC/D is primarily aimed at reducing the burden of symptomatic arrhythmias and decreasing the incidence of sudden cardiac death. Automatic implantable cardioverter-defibrillators (AICDs) significantly reduce mortality in patients with ARVC/D. However, accurate risk assessment is needed to avoid exposing low-risk patients to the long-term complications of AICDs. Strenuous exertion increases the rate of disease progression and increases the risk of sudden death.

Original languageEnglish (US)
Title of host publicationElectrical Diseases of the Heart
Subtitle of host publicationVolume 2: Diagnosis and Treatment, Second Edition
PublisherSpringer-Verlag London Ltd
Number of pages14
ISBN (Electronic)9781447149781
ISBN (Print)9781447149774
StatePublished - Jan 1 2013


  • Arrhythmogenic cardiomyopathy
  • Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)
  • Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
  • Arrhythmogenic right ventricular dysplasia (ARVD)
  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)

ASJC Scopus subject areas

  • General Medicine


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