Pulmonary stenosis etiology and anatomy

Etiology

 * Congenital pulmonic stenosis is most common.


 * Rheumatic involvement is rare, is usually part of multivalvular involvement, rarely leads to serious deformity.


 * Carcinoid plaques can be present in the carcinoid syndrome. These result in constriction of the pulmonic valve ring, retraction and fusion of the valve cusps.

Anatomy

 * Typically the valve is domed shaped with fused commissures.


 * If the foramen ovale is patent, then right to left shunting can occur at the atrial level.


 * If there is pulmonary atresia with an intact ventricular septum then these patients die soon after birth.