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.
van Son
J A
JA
Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Danielson
G K
GK
Puga
F J
FJ
Edwards
W D
WD
Driscoll
D J
DJ
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications; D011667:Pulmonary Veins / Q000002:abnormalities / Q000473:pathology / Q000601:surgery; D011668:Pulmonary Veno-Occlusive Disease / Q000151:congenital / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome