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Ann Thorac Surg 1999;68:164-168
© 1999 The Society of Thoracic Surgeons

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Original Articles

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

^a Department of Pediatrics, Primary Children’s Medical Center and University of Utah, Salt Lake City, Utah, USA
^b Department of Surgery, Primary Children’s Medical Center and University of Utah, Salt Lake City, Utah, USA

Address reprint requests to Dr Shaddy, Division of Pediatric Cardiology, Primary Children’s Medical Center, Suite 1500, 100 North Medical Dr, Salt Lake City, UT 84113
e-mail: pcrshadd{at}ihc.com

Background. Pulmonary vein (PV) stenosis with anatomically normal connection is considered rare, unresponsive to treatment, progressive, and usually fatal.

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 congenital 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 patients underwent heart transplantation for inoperable associated cardiac lesions. 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 connection is associated with other congenital cardiac abnormalities, (2) presentation and outcome are contingent on the number of stenosed PVs, (3) surgical palliation may be helpful in some patients, and (4) heart transplantation for inoperable associated cardiac abnormalities may be an option in patients with only one or two stenosed PVs.

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