Arrhythmogenic right ventricular cardiomyopathy as lethal complication factor after cardiac surgery.

In patients with arrhythmogenic right ventricular dysplasia (ARVD), the right ventricular myocardium histologically discloses atrophy paralleled by fibrofatty or fatty replacement. Apoptosis is believed to be a putative major pathogenetic mechanism. Altogether, our knowledge of genetics, etiology and pathophysiology of ARVD has increased impressively in the last few years, and effective genetic tests now principally would be possible. Nevertheless, due to often uncharacteristic or even lacking symptoms, clinical diagnosis may be very difficult and could not be made during lifetime of patient presented here, partly due to additional, independent cardiac problems. The question of an effective preoperative diagnostic regimen for cardiosurgical interventions remains and seems to be currently open.