title: 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.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Catheterization Lab, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.|Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Qianjun
authors.initials: Q
authors.lastname: Jia
authors.affiliation: Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Jianzheng
authors.initials: J
authors.lastname: Cen
authors.affiliation: Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Zhuang
authors.affiliation: Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Xiaomei
authors.initials: X
authors.lastname: Zhong
authors.affiliation: Department of Cardiovascular Disease Epidemiology, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Xiaoqing
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Catheterization Lab, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Jiahua
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Changhong
authors.initials: C
authors.lastname: Liang
authors.affiliation: Department of Catheterization Lab, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
authors.forename: Meiping
authors.initials: M
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000328:Adult; D019540:Area Under Curve; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology*; D000072226:Computed Tomography Angiography; D005260:Female; D006343:Heart Septal Defects / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011651:Pulmonary Artery / Q000502:physiology*; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D011667:Pulmonary Veins / Q000502:physiology*; D012372:ROC Curve; D012189:Retrospective Studies; D055815:Young Adult
keywords: Computed tomography; Congenital heart defect; Pulmonary arterial index; Pulmonary atresia; Pulmonary vein index