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

John P. Breinholt, John A. Hawkins, Luann Minich, Lloyd Y. Tani, Garth S. Orsmond, Saskia Ritter, Robert E. Shaddy

Research output: Contribution to journalArticlepeer-review

76 Scopus citations

Abstract

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. Copyright (C) 1999 The Society of Thoracic Surgeons.

Original languageEnglish (US)
Pages (from-to)164-168
Number of pages5
JournalAnnals of Thoracic Surgery
Volume68
Issue number1
DOIs
StatePublished - Jul 1999

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

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