Congenital pulmonary valve stenosis


Pulmonary valvular stenosis (PVS) is a congenital disorder in 95% of cases. In most patients, pulmonic valvular stenosis is an isolated anomaly that does not present until adulthood.

Post-stenotic dilatation of the main pulmonary artery is seen only in pulmonic valve stenosis. The degree of dilatation appears to be independent of the severity of the right ventricular outflow tract obstruction.

A preferential 'jet-streaming' of blood across the stenotic pulmonic valve can result in asymmetric blood flow which is most commonly increased to the left lung (a classic finding in PVS). There will be associated asymmetric enlargement of the left pulmonary artery.

Valve calcification is rare in PVS.

Operative findings:
The ventricular function was significantly depressed and the right ventricle was massively dilated, as well as the main and branch pulmonary arteries, with the maximum diameter measuring 7.2 cm. The pulmonary valve was incompetent with severe pulmonary valve insufficiency.

Patient underwent

1.    Pulmonary valve replacement (29 mm Epic porcine bioprosthesis).
2.    Pulmonary artery reduction arterioplasty with posterior pulmonary artery plication.

Final pathologic diagnosis:

Pulmonary valve leaflets (biopsy):
-    Heart valve with myxoid degeneration and fibrosis.