Repair of congenital and acquired pulmonary vein stenosis

Background. Congenital pulmonary vein stenosis is a rare cause of obstruction of pulmonary venous blood flow with a high mortality. Acquired pulmonary vein stenosis is an equally serious condition. Methods. Eight patients (age range, 3 months to 43 years; median age, 1.5 years) underwent surgical relief of pulmonary vein stenosis. Two had congenital pulmonary vein stenosis, 5 had pulmonary vein stenosis that was acquired after surgical treatment of total anomalous pulmonary venous connection, and 1 had pulmonary vein stenosis associated with idiopathic mediastinal fibrosis and calcification. Results. One infant died 2 months after correction of acquired pulmonary vein stenosis. At follow-up extending to 16 years (median follow-up, 6.5 years), 6 patients are in New York Heart Association functional class I, and 1 patient is in class II. Conclusions. In view of the dismal natural history of untreated pulmonary vein stenosis, prompt surgical relief of the stenosis may be a rewarding undertaking.