<unk>  <unk> , also known as<unk>  right ventricular cardiomyopathy or ARVC) is a type of nonischemic cardiomyopathy that involves primarily the right ventricle.  It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.<unk>  is an important cause of ventricular arrhythmias in children and young adults.  It is seen predominantly in males, and 30-50% of cases have a familial distribution.  It is usually inherited in an autosomal dominant pattern, with variable expression.  The penetrance is 20-35% in general, but significantly higher in Italy.  Seven gene loci have been implicated in<unk> .  However, about 50% of families that express<unk>  that undergo genetic screening do not show linkage with any of the known  chromosomal  loci.  It is unclear whether the pathogenesis varies with the different loci involved.  A standard genetic screening test is not available.
Naxos disease is an autosomal recessive variant of<unk> , described initially on the Greek island of Naxos.  There, the penetrance is >90%.  It involves the gene that codes for plakoglobin (a protein that is involved in cellular adhesion), on chromosome 17p.  Naxos disease is described as a triad of<unk> , palmoplantar keratosis, and wooly hair.  The signs of Naxos disease are more severe than with autosomal dominant<unk> .<unk>  <unk> , also called<unk>  right ventricular cardiomyopathy (ARVC) or<unk> /cardiomyopathy <unk> /C), is an inherited heart disease.<unk>  is caused by genetic defects of the parts of heart muscle (also called myocardium or cardiac muscle) known as desmosomes, areas on the surface of heart muscle cells which link the cells together. The desmosomes are composed of several proteins, and many of those proteins can have harmful mutations.
The disease is a type of nonischemic cardiomyopathy that involves primarily the right ventricle.  It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.<unk>  is often found in association with diffuse palmoplantar keratoderma, and woolly hair, because their genes are nearby and often inherited together :513
ARVC/D is an important cause of ventricular arrhythmias in children and young adults.  It is seen predominantly in males, and 30-50% of cases have a familial distribution.
Arrhythmogenic right ventricular dysplasia
Arrhythmogenic right ventricular cardiomyopathy
Atrial septal defect migraine
Right ventricular outflow tract tachycardia
Annuloaortic ectasia
Familial atrial fibrillation
Interrupted aortic arch
Intraventricular conduction delay