Sudden Death in Young Patients With Congenital Aortic Stenosis

Abstract

Four patients with congenital valvular and subvalvular aortic stenosis who died suddenly are presented in detail. Each had evidence of severe obstruction and left ventricular "strain" pattern on the electrocardiogram. Symptoms were present in three cases. Survey of the literature was done with particular reference to the usefulness of standard scalar electrocardiography and patient symptomatology in estimating the severity and the progression of the aortic obstruction. Of those who died suddenly with isolated aortic stenosis, 70% had left ventricular strain pattern, 21% had left ventricular hypertrophy alone, and 9% had an apparently normal electrocardiogram. Symptoms of exertional dyspnea, syncope, chest pain, and fatigue were present in almost all cases. Sudden death in the absence of severe ECG abnormalities and/or symptoms is very uncommon.