Chest radiograph reveals dilatation of the main pulmonary artery with relatively normal-sized right and left pulmonary arteries. The aorta is left sided.
Facts: Pulmonary Valvular Stenosis
- Common congenital heart defects, approximately 10% of all cases
- Classified as subvalvular, valvular and supravalvular stenosis (based on level of obstruction) and as mild, moderate and severe (based on pressure gradient across stenosis). It can occur in branch pulmonary arteries as well
- May occur in isolation (as in our case) or be associated with other complex congenital heart defects
- In severe cases, physical and ECG findings of right axis deviation, right ventricular hypertrophy would be apparent
- Cardiac ultrasound: obstruction at right ventricular outflow tract (RVOT), pulmonary valve (PV), main pulmonary artery, right and left pulmonary arteries, abnormal pulmonary valve annulus, abnormal pressure gradients across RVOT, PV and pulmonary arteries
- Radiography: enlargement of the main pulmonary artery, right ventricular hypertrophy. Radiographic differential diagnoses are pulmonary hypertension, idiopathic dilatation of the pulmonary trunk
Our case - pulmonary valvular stenosis in a young woman who had the diagnosis since birth and had undergone valvulotomy.
1. Heiden K. Congenital heart defects, simplified. 2009
2. Castaner E, Gallardo X, Rimola F, et al. Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics 2006;26:349-371.