Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.

OBJECTIVES: The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs.

METHODS: We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n  = 24) or pulmonary artery rehabilitation ( n  = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves.

RESULTS: During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P  <   0.01] and the presence of native pulmonary artery (HR = 0.06; 95% CI: 0.10, 0.35; P  <   0.01) as independent positive predictors of better survival. The area under the ROC curve for PVI was 0.79 ( P  <   0.01), and a cut-off point of 438 mm 2 /m 2 was deemed the significant threshold for survival (sensitivity 92%, specificity 72%).

CONCLUSIONS: Preoperational high PVI and native pulmonary artery presence were significant morphologic predictors of a positive survival advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.