Clinical Presentation and Therapy of Semilunar Valve and Aortic Arch Anomalies

Abstract

There are three types of congenital aortic stenosis. The most common is aortic valve stenosis and this comprises approximately 5 % of congenital heart defects. The aortic valve cusps or leaflets are congenitally malformed. They may be thickened and the raphe may be fused. Usually these valves are bicuspid. The second type of aortic stenosis is supravalvar aortic stenosis. This involves a narrowing of the ascending aorta just above the sino-tubular junction. The third type of aortic stenosis is subvalvar aortic stenosis. In this condition, there is a fibromuscular ridge or tunnel that causes obstruction a short distance below the valve.

Coarctation of the aorta consists of a ledge-shaped protrusion of tissue into the aorta immediately opposite the insertion of the ductus or ligamentum arteriosus. Frequently, there is an associated bicuspid aortic valve.

Pulmonary valve stenosis (with intact ventricular septum) usually occurs sporadically. Isolated pulmonary valve insufficiency is rare. It can accompany idiopathic dilation of the pulmonary artery and pulmonary vascular obstructive disease. Most cases of pulmonary insufficiency result from surgical or balloon procedures to treat pulmonary valve stenosis.