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Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review

Jorge Romero1, Eliany Mejia-Lopez, Carlos Manrique

  • 1Division of Cardiology, Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.

Clinical Medicine Insights. Cardiology
|June 14, 2013
PubMed

Insights

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition affecting the right ventricle, potentially leading to sudden cardiac death in young athletes. Diagnosis requires integrated criteria, with ICDs and heart transplantation as key treatments.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiac condition.
  • It primarily impacts the right ventricle, but can affect both ventricles, leading to heart failure, arrhythmias, and sudden cardiac death.
  • ARVC/D accounts for a significant percentage of sudden cardiac death in young athletes.

Purpose of the Study:

  • To provide an overview of Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).
  • To highlight diagnostic challenges and current diagnostic criteria.
  • To outline current therapeutic strategies for ARVC/D.

Main Methods:

  • Review of pathological characteristics including myocardial atrophy, fibrofatty replacement, and chamber dilation.
  • Application of consensus diagnostic criteria integrating electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI), and myocardial biopsy.
  • Emphasis on early detection, family screening, and risk stratification.

Main Results:

  • Diagnosis is complicated by nonspecific symptoms and varied presentations.
  • Integrated diagnostic approaches improve detection rates.
  • Therapeutic options include implantable cardioverter-defibrillators (ICDs), ablative procedures, and heart transplantation.

Conclusions:

  • ARVC/D is a significant cause of sudden cardiac death, particularly in young individuals.
  • Accurate diagnosis relies on a combination of clinical, imaging, and histological findings.
  • Management focuses on risk stratification and timely intervention to prevent adverse outcomes.

Keywords:
Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)cardiomyopathysudden cardiac deathtachyarrhythmias

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