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 steno sis (FSS) are reported. All patients underwent echocardiography and angiogr aphy prior to surgical intervention, Sixty-four (89%) had discrete subaorti c 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 assoc iated 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 a lthough related to DSS has certain features that may set it apart as a dist inct entity. In addition DSS appears to be associated with aortic stenosis and bicuspid aortic valve as frequently as with ventricular septal defect. Copyright (C) 2000 S. Karger AG, Basel.