Arrhythmogenic right ventricular dysplasia causes

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Overview

Arrhythmogenic right ventricular cardiomyopathy is typically inherited as an autosomal dominant pattern with variable penetrance and incomplete expression. Approximately 40% to 50% of ARVC/D patients have a mutation in genes encoding a desmosome protein. The gene is on the chromosome 14q23-q24.There is an autosomal recessive trait variant associated with palmoplantar keratosis and wooly hair named Naxos disease.


Causes

Arrhythmogenic right ventricular cardiomyopathy is cause by genetic inheritance as:[1]

  • An autosomal dominant pattern with variable penetrance and incomplete expression.
  • A mutation in genes encoding a desmosome protein ( 40% to 50% of ARVC/D patients)
  • The gene is on the chromosome 14q23-q24
  • There is an autosomal recessive trait variant associated with palmoplantar keratosis and wooly hair named Naxos disease
  1. Rampazzo A, Nava A, Danieli GA, Buja G, Daliento L, Fasoli G; et al. (1994). "The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23-q24". Hum Mol Genet. 3 (6): 959–62. doi:10.1093/hmg/3.6.959. PMID 7951245.