Atrioventricular pacemaker placement in romano-ward syndrome and recurrent torsades de pointes

Abstract

T h e Romano-Ward syndrome 1 is a heritable disorder predominantly affecting young women and characterized by a marked propensity for syncope and sudden death. The electrocardiogram heralds the underlying electrical instability by showing a delayed repolarization interval with notched or asymmetric T waves. The devastating clinical events associated with this electrocardiographic pattern are the result of sudden paroxysms of polymorphic ventricular tachycardia (VT). This arrhythmia tends to occur sporadically and in a spontaneous manner, but may be precipitated by intense auditory stimuli, physical activity or emotional stress. 1,2 Left untreated, patients with the RomanoWard syndrome and associated syncope have a reported mortality rate of at least 5% per year during an estimated average follow-up of 5 years. 2 Two treatment modalities, fl-blocker therapy and left stellate ganglionectomy, had significant beneficial effects on outcome in that each was associated with a reduction in the frequency of syncope and sudden death. 1 Nevertheless, some patients with this dramatic clinical picture continue to have syncope despite a combination of the 2 forms of therapy.