Plotselinge dood bij jonge mensen door aritmogene rechterventrikeldysplasie

A previously healthy boy aged 14 developed persistent ventricular tachycardia while engaged in sports; ultrasonography revealed arrhythmogenic right ventricular dysplasia (ARVD). He was treated with a class III antiarrhythmic drug but nevertheless died 4 years later during recreational activities. An uncle of the boy was found to have died suddenly on a playing field, 25 years previously at the age of 20. In ARVD, fibrolipomatous areas in the right ventricle lead to dilations of the wall where (sometimes fatal) re-entrant tachycardias may develop. An autosomal dominant heredity with variable expression and penetrance is considered probable, while the genetic defect was located recently. Examination and, if necessary, pharmacotherapy of relatives of an ARVD patient may reduce the risk of a fatal arrhythmia.