Management of Congenital and Acquired Pulmonary Vein Stenosis

Background Pulmonary vein (PV) stenosis, whether congenital or after repair of total anomalous pulmonary venous connection (TAPVC), continues to carry a poor prognosis. Methods A retrospective review identified 36 patients who underwent repair of PV stenosis between December 1989 and June 2003. Fourteen with congenital PV stenosis underwent scar excision and primary repair (n = 2), intraoperative stent placement (n = 4), or sutureless pericardial marsupialization (n = 8). Twenty-two with acquired PV stenosis after TAPVC repair underwent anastomotic revision and/or vein repair (n = 11) or sutureless pericardial marsupialization (n = 11). Follow-up ranged from 1 month to 14 years (median, 30 months). Results Among the 14 patients with congenital PV stenosis, 8 died (3 early deaths, 4 late deaths with restenosis, and 1 late noncardiac death). Among the six survivors, five (4 after marsupialization) have not developed restenosis. Among 11 of 22 patients with acquired PV stenosis undergoing anastomotic revision or vein repair, there were 5 deaths (2 early, 2 late with restenosis, and 1 late noncardiac death) and 1 of the six survivors has developed restenosis. Of the remaining 11 undergoing marsupialization, there was one late death (with restenosis) and 10 survivors have no restenosis. Congenital etiology, use of marsupialization technique, presence of associated defect, and extent of disease were identified as risk factors for poor outcome. Conclusions Patients with pulmonary vein stenosis continue to have a guarded prognosis. Sutureless pericardial marsupialization was associated with satisfactory midterm results and appears superior to other conventional techniques.