arrhythmogenic right ventricular dysplasia/cardiomyopathy

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A range of autosomal dominant disorders in which there is arrhythmia of the right ventricle of the heart and replacement of muscle with fibrous tissue; they are an inherited cause of juvenile sudden death. The mutated gene product in ARVD1 is TGFβ3; in ARVD2, ryanodine receptor 2; in ARVD5, transmembrane protein 43; in ARVD8, desmoplakin; in ARVD9, plakophilin 2, in ARVD10 desmoglein 2; in ARVD11 desmocollin 2; in ARVD12, γ-*catenin.

Subjects: Medicine and Health.

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