Concomitant reentrant tachycardias from concealed accessory atrioventricular bypass tract and atrioventricular nodal reentry in a patient with Williams syndrome

Williams syndrome is characterized by a constellation of features including mental retardation and supravalvular aortic stenosis. Other cardiovascular abnormalities including arrhythmias contributing to sudden death have been described in these patients. In this report we describe a case of a 49-year-old female with Williams syndrome who presented with severe symptomatic supraventricular tachycardia. Cardiac electrophysiology study identified a left posteroseptal concealed accessory bypass tract responsible for atrioventricular reentrant tachycardia and a concomitant typical atrioventricular nodal tachycardia. Such unusual association of combination of two different types of supraventricular tachycardia and Williams syndrome has not been previously reported. Radiofrequency ablation was successfully performed to cure these arrhythmias.