Pulmonary stenosis etiology and anatomy

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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.