[Etiopathogenesis of arrhythmogenic right ventricular dysplasia].

Arhythmogenic right ventricular dysplasia (ARVD) is a genetically determined cardiomyopathy with a dominant transmission mode and variable penetrance. Transdifferenciation of cardiomyocytes into adipocytes is likely to explain massive replacement of right ventricular and to a lesser extent left ventricular myocardium by adipose tissue. This phenomenon starts in the mediomural layers and extends into the epicardium.

Prenatal echocardiographic appearance of arrhythmogenic right ventricle dysplasia: a case report.

We report a case of arrhythmogenic right ventricular dysplasia (ARVD) diagnosed prenatally by echocardiography at 24 weeks gestation. The 4-chamber view showed a large outpouched area extending from below the tricuspid valve to the insertion of the moderator band; the affected wall appeared thin and akinetic, with absence of flow at color Doppler investigation and no evidence of cardiovascular failure. The size of the outpouched area was unchanged at subsequent controls (25 and 26 weeks gestation) when frequent extrasystoles occurred, probably of a ventricular origin.