Familial incidence of late ventricular potentials and electrocardiographic abnormalities in arrhythmogenic right ventricular dysplasia.

Familial forms of arrhythmogenic right ventricular dysplasia (ARVD) have been described. Signal-averaged electrocardiograms (SAECGs) and standard electrocardiograms have been used to detect ARVD. The purpose of this prospective study, for a given family member, was to evaluate the risk of having ARVD or only belonging to an affected family. To address these issues, we assessed the incidence of late ventricular potentials and electrocardiographic (ECG) abnormalities in the families of our patients with ARVD. SAECGs and electrocardiograms were recorded in 101 eligible family members and compared with those recorded in ARVD patients with sustained ventricular tachycardia (13 patients in 12 families), and in 37 control subjects with a normal electrocardiogram. The incidence of late ventricular potentials was significantly higher in family members than in control subjects (16% vs 3%, p <0.05). The incidence of ECG abnormalities was 34% in family members. When the incidence of late ventricular potentials and/or ECG abnormalities were added up, results were 38% abnormal findings in family members. Late ventricular potentials and/or ECG abnormalities were found in members of all 7 families; these abnormalities were initially thought to be sporadic forms, and thereafter were classified as familial forms. Thus, SAECGs and standard ECG recordings in ARVD family members showed 38% abnormal findings, and that all cases of ARVD could be classified as familial forms. The incidence of familial forms of ARVD was greater than was previously believed, which is highly suggestive of a genetic transmission of the disease in our geographic area.