Pulmonary vein stenosis with normal connection: Associated cardiac abnormalities and variable outcome

Background. Pulmonary vein (PV) stenosis with anatomically normal connectio n is considered rare, unresponsive to treatment, progressive, and usually f atal. Methods. We reviewed the records of 13 children with this diagnosis at our center since 1990. Results. The number of stenosed PVs ranged from all PVs (n = 5); three PVs (n = 1); two PVs (n = 5); and one PV (n = 2). All patients had associated c ongenital cardiac abnormalities. Operation on PV stenosis was attempted in 7 patients (54%), 2 of whom have done well and 5 of whom have not. Two pati ents underwent heart transplantation for inoperable associated cardiac lesi ons. Significantly more patients with three or four stenosed PVs died (83%) compared with patients with one or two stenosed PVs (0%). Conclusions. (1) Pulmonary vein stenosis with anatomically normal connectio n is associated with other congenital cardiac abnormalities, (2) presentati on and outcome are contingent on the number of stenosed PVs, (3) surgical p alliation may be helpful in some patients, and (4) heart transplantation fo r inoperable associated cardiac abnormalities may be an option in patients with only one or two stenosed PVs. (C) 1999 by The Society of Thoracic Surg eons.