GTR Home > Conditions/Phenotypes > Arrhythmogenic right ventricular cardiomyopathy, type 11

Disease characteristics

Excerpted from the GeneReview: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. It primarily affects the right ventricle; with time, it may also involve the left ventricle. The presentation of disease is highly variable even within families, and some affected individuals may not meet established clinical criteria. The mean age at diagnosis is 31 years (±13; range: 4-64 years).

Associated genes

Clinical features

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  • Palpitations
  • Palmoplantar keratoderma
  • Ventricular arrhythmia
  • Sudden cardiac death
  • Syncope
  • Dyspnea
  • Woolly hair
  • Right ventricular cardiomyopathy
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