Scimitar syndrome and left pulmonary vein stenosis: A serious and rare association

Abstract

Scimitar syndrome is a rare constellation of cardiovascular anomalies consisting of partial right anomalous pulmonary venous drainage to the inferior vena cava, hypoplasia of the right lung, cardiac dextroposition, and in many cases, one or more aortopulmonary collaterals from the descending aorta to the hypoplastic lung. The “infantile form” commonly presents with significant heart failure and pulmonary hypertension. Stenosis of the left pulmonary veins associated with scimitar syndrome is a rare but well-described combination, usually associated with a poor prognosis. We describe two patients with this association in whom the left pulmonary vein stenosis manifested months after the initial diagnosis as a progressive lesion that complicated the course of the disease due to severe pulmonary hypertension. Both patients were successfully treated with a combination of surgical, percutaneous, and hybrid treatment.

Keywords

• pulmonary hypertension
• pulmonary vein stenosis
• scimitar syndrome