ROMANO-WARD-SYNDROM

Abstract

Syncopal attacks caused by cardiac arrhythmias have been frequently reported. In one family we found female members of three generations affected by characteristic prolongation and alteration of the QT-interval in ECG. All patients suffered from attacks with loss of consciousness which could not be defined as epileptic fits. Ventricular arrhythmias have to be considered as reasons for the attacks. This combination of symptoms without additional malformations (i.e. congenital deafness) is well known in literature as Romano Ward Syndrome. Mode of inheritance, pathogenetic mechanisms and possibilities of therapy are discussed. Therapy with a beta blocking medication (propranolol) made our youngest patient free of attacks, the other two patients have had no complaints for two years, without any therapy.