Mechanisms of syncopes in arrhythmogenic right ventricular dysplasia–cardiomyopathy beyond monomorphic ventricular tachycardia

Abstract

Syncopes appear in 10–20% in arrhythmogenic right ventricular dysplasia–cardiomyopathy (ARVD/C). In the majority of cases sustained or non-sustained monomorphic ventricular tachycardias represent the underlying mechanism of syncope. In other cases the mechanism remains unclear. In 37 patients (23 females, mean age 43.6 ± 12.8 years) without detectable and inducible monomorphic ventricular tachycardia, a diagnostic algorithm including repeat ECG, holter monitoring, telemetry, electrophysiological examination, ajmaline challenge, tilt table testing and neurological work-up (EEG, cranial computer tomography) was used in order to identify the mechanism of syncopes. Constant AV block 3° could be found in 3 patients (2 males). Intermittant AV block 2° or 3° could be identified in 3 females. Four males had abnormal Wenckebach point during rapid atrial stimulation, 3 males demonstrate isolated HV interval prolongation. Rapid polymorphic VT and VF could be induced in a young female with ARVD/C. Eight patients (7 females) presented with recurrent syncopes and provocable right precordial ST elevation and right bundle branch block during ajmaline challenge. Three patients had abnormal tilt table testing as the only pathological finding. In one female with intermittent AV block 2° tilt table testing and ajmaline challenge was positive. One female had the diagnosis of focal epilepsia after neurological work-up. In 11 cases the mechanism of syncopes remained unclear. In patients with ARVD/C and syncopes beyond detectable or inducible monomorphic VT, several mechanisms of syncopes could be identified with conduction disease as the predominant finding. These results may help in identifying rare mechanisms of syncopes in ARVD/C.