Camille L. Hancock Friesen, David Zurakowski, Ravi R. Thiagarajan, Joseph M. Forbess, Pedro J. del Nido, John E. Mayer, Richard A. Jonas
Children’s Hospital Boston and Harvard Medical School.
United States
Annals of Thoracic Surgery
Ann Thorac Surg 2005; 79: 596-606
DOI: 10.1016/j.athoracsur.2004.07.005
Abstract
Background: Repair of total anomalous pulmonary venous connection (TAPVC) continues to be associated with significant mortality. We reviewed patients undergoing consecutive TAPVC repairs over a 10-year period at Children’s Hospital Boston. The impact of current surgical and perioperative management strategies on short-term outcomes (postrepair pulmonary venous obstruction and mortality) is evaluated.
Methods: All patients with surgically corrected TAPVC from November 1989 to December 2000 were included. Charts were reviewed for patient demographics, operation variables, and postoperative course.
Results: There were 123 patients in the cohort, of which 72 (59%) were male. The median age and weight at operation were 10 days and 3.6 kg, respectively. Sixty-eight (55%) patients presented with pulmonary venous obstruction, and 65 (53%) underwent emergent TAPVC repair. Thirty-nine (32%) had single-ventricle anatomy, and 84 (68%) had two-ventricle anatomy. Thirty patients (24%) died. Kaplan-Meier survival at 1 month was 65% (95% confidence interval [CI], 55% to 75%) for single-ventricle patients versus 90% (95% CI, 90% to 100%) for two-ventricle patients; at 36 months it was 47% (95% CI, 35% to 59%) versus 87% (95% CI, 81% to 93%), respectively. By Cox multivariable regression analysis, a single ventricle (p < 0.001, hazard ratio, 4.8; 95% CI, 2.5 to 9.2) was an independent mortality risk factor. Prerepair pulmonary venous obstruction was a multivariate risk factor for death among single-ventricle patients. Postrepair pulmonary venous obstruction occurred in 11%. If year of operation is used as a predictor, two-ventricle patient survival has significantly improved (p < 0.05).
Conclusions: Despite current interventions, single-ventricle patients continue to have a worse prognosis than two-ventricle patients.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Incidence or Prevalence of Disease
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Influence of Surgical Era on the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2005
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