Fixed subaortic stenosis revisited. congenital abnormalities in 72 new cases and review of the literature.

Seventy-two patients aged 2-65 years (mean 20.2) with fixed subaortic stenosis (FSS) are reported. All patients underwent echocardiography and angiography prior to surgical intervention. Sixty-four (89%) had discrete subaortic stenosis (DSS) and 8 (11%) tunnel type stenosis (TSS). Patients with DSS aged 2-65 years (mean 29) while patients with TSS aged 10-34 years (mean 19) and had a distinct female preponderance (7:1). Twenty-five patients (39%) with DSS had other congenital abnormalities of which aortic stenosis associated with bicuspid aortic valve was slightly more common than ventricular septal defect (7:6). Five patients with TSS had a small aortic annulus and hypoplastic aorta. Our data and those in the literature indicate that TSS although related to DSS has certain features that may set it apart as a distinct entity. In addition DSS appears to be associated with aortic stenosis and bicuspid aortic valve as frequently as with ventricular septal defect. Copyright 2000 S. Karger AG, Basel