Nicola Viola, Abdullah A. Alghamdi, Donald G. Perrin, Gregory J. Wilson, John G. Coles, Christopher A. Caldarone
Southampton University Hospital. Hospital for Sick Children.
United Kingdom and Canada
Journal of Thoracic and Cardiovascular Surgery
J Thorac Cardiovasc Surg 2011; 142: 344-350
DOI: 10.1016/j.jtcvs.2010.12.004
Abstract
Background: Primary pulmonary vein stenosis is often associated with relentless restenosis and early death. During the last 2 decades, we have developed a sutureless repair to improve prognosis.
Methods: Hospital records for patients undergoing repair of primary pulmonary vein stenosis from 1989 to 2008 were reviewed. Pulmonary vein stenosis was quantified with a pulmonary vein stenosis score. Survival was determined by Kaplan-Meier analysis.
Results: Twenty-three patients underwent surgical repair. Mean ages at diagnosis and index repair were 23.3 ± 45.6 and 24.1 ± 40.9 months, respectively. Systemic or suprasystemic pulmonary artery pressures were present in 13 of 18 patients (72%). Seven (31%) had single-ventricle circulation. A sutureless technique was used in 19 of 23 cases (83%). Other types of repair were used in 4 of 23 (17%). There were 11 recorded deaths (47%). Survivals were 64%, 47%, and 31% at 1, 5, and 10 years, respectively. Five patients (22%) required 1 reintervention. Surgical repair significantly reduced the total pulmonary vein stenosis score (5.6 ± 2.10 before repair, 2.6 ± 2.72 after repair, P = .0057). The preoperative pulmonary vein stenosis score was the only independent predictor of mortality (hazard ratio, 1.732; P < .01). A preoperative pulmonary vein stenosis score of greater than 4 was a poor prognostic indicator (area under the curve, 0.83).
Conclusions: Mortality and restenosis rates remained high despite the adoption of a sutureless technique. A preoperative pulmonary vein stenosis score of greater than 4 was a strong predictor of poor prognosis.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Diagnostic Testing. Risk Stratification
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2011
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: Yes