John P. Breinholt, John A. Hawkins, LuAnn Minich, Lloyd Y. Tani, Garth S. Orsmond, Saskia Ritter, Robert E. Shaddy
Primary Children’s Medical Center and University of Utah.
United States
Annals of Thoracic Surgery
Ann Thorac Surg 1999; 68: 164-168
DOI: 10.1016/s0003-4975(99)00311-2
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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 1999
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No