Congenital pulmonary valve stenosis

Case contributed by Dr Jayanth Keshavamurthy


Chronic shortness of breath on exertion

Patient Data

Age: 60 years
Gender: Female

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

Preoperative radiograph showing enlarged main and left pulmonary artery with pruning of peripheral pulmonary arteries.

Cardiac MRI to quantify pulmonary regugitation

  1. Left ventricle: normal size and function. Ejection fraction 69%. Normal regional wall motion, however there is septal flattening.
  2. Right ventricle: severe enlargement with end-diastolic volume of 363 ml=L and indexed end-diastolic volume of 192 mL. Preserved systolic function with ejection fraction of 57%.
  3. Valvular function: possibly bicuspid pulmonary valve morphology with pulmonary valve area of 2.3 cm2. Severe pulmonic insufficiency with regurgitant volume greater than 100 mL and regurgitant fraction over 60%.
  4. Profound enlargement of main and left pulmonary artery.

Post operative chest radiographs after a bioprosthetic 29 mm pulmonary valve replacement. Pulmonary artery reduction and plication.

Case Discussion

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.

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