19.2 Mechanisms of syncope in arrhythmogenic right ventricular dysplasia-cardiomyopathy

Abstract

In patients with arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) syncopes are frequent events with in most cases a bad prognosis. In most cases the cause of syncope is non-sustained monomorphic VT. In 27 patients (9 males, mean age 46.3 years) with ISFC/ESC criteria of ARVD/C without documented or inducible monomorphic VT several other features could be found. Permanent AV Block IIIo in 3 cases, an intermittant AV block IIIo in 2 cases, an isolated HV interval of 80 msec in one case and isolated pathological atrioventricular conduction (<100 bpm) in 4 cases. In a young female patient a short polymorphic VT with degeneration into VF was inducible. In four patients positive Brugada phenomenon was provocable by ajmaline testing. Tilt table testing was done in 7 cases; neurocardiogenic syncope was found in 2 cases. Conclusions: Besides non-sustained monomorphic VT other mechanisms of syncope must be discussed in ARVD/C. There seems to be an association of ARVD/C to progressive conduction disease, catecholaminergic polymorphic ventricular tachycardia and Brugada syndrome.