Arrhythmogenic Right Ventricular Cardiomyopathy

Julia H. Indik, Frank I. Marcus

Research output: Chapter in Book/Report/Conference proceedingChapter


Arrhythmogenic right ventricular cardiomyopathy is a disorder of myocyte cell-to-cell adhesion related to desmosomal proteins, with the most common mutation being in plakophilin. Ventricular arrhythmias commonly precede structural abnormalities, which predominantly affect the subtricuspid and basal anterior wall of the right ventricle. Clinical disease in the child generally begins after age 10, and a strategy of evaluation and management for the child is needed. The goals of management for the child are to prevent or slow progression of clinical disease. Beta blockade may be of benefit. An implantable cardioverter defibrillator may be considered based on an individualized assessment of risk of sudden cardiac death, but data for primary prevention in a pediatric population are lacking. Of utmost importance is to restrict exercise, particularly for those who already manifest abnormalities.

Original languageEnglish (US)
Title of host publicationHeart Failure in the Child and Young Adult
Subtitle of host publicationFrom Bench to Bedside
Number of pages6
ISBN (Electronic)9780128023938
ISBN (Print)9780128026137
StatePublished - Jan 1 2017


  • ARVC
  • Desmosomal protein
  • Exercise restriction
  • Genetic testing
  • Sudden cardiac death

ASJC Scopus subject areas

  • General Medicine


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