title: Multistage pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary artery.
abstract: OBJECTIVE
  The aim of this study was to determine the effect of multistage pulmonary artery (PA) rehabilitation consisting of right ventricle to pulmonary artery (RV-PA) connection, major aortopulmonary collateral artery (MAPCA) closure and PA angioplasty in patients with pulmonary atresia, ventricular septal defect (VSD) and hypoplastic pulmonary arteries. In addition, the effects of the PA reintervention were reported and risk factors were analysed.
  
  
  METHODS
  This study was a retrospective review of 69 consecutive patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries (mean Nakata index 100.9 ± 57.6 mm(2)/m(2)) who underwent multistage rehabilitation of hypoplastic PA from December 2009 to December 2014.
  
  
  RESULTS
  RV-PA connection was performed at a median age of 1.0 years with 2 hospital deaths in the hybrid operation theatre. Thirty-two patients underwent concomitant pulmonary angioplasty with 28 collateral occlusions. After a mean duration of 15.4 ± 12.7 months, 16 patients had interventional catheterization consisting of 14 balloon dilatations, 12 stent implantations and 16 collateral occlusions. At a mean age of 2.7 ± 1.9 years, complete repair was performed in 31 patients within 1.2 ± 0.6 years of the palliation with 1 hospital death. Twenty-two patients had concomitant PA angioplasty. The estimated complete repair rate was 60.1 ± 7.1% ∼3 years after the palliation by the Kaplan-Meier method. During a mean follow-up of 2.8 ± 1.3 years, 49.3% (33/67) of the patients had PA reintervention. Pulmonary stenosis requiring angioplasty at palliation is associated with PA reintervention (P = 0.003). The actuarial survival rate for the overall population was 93.8 ± 3.0% at 5 years after the placement of an RV-PA connection.
  
  
  CONCLUSIONS
  The multistage strategy consisting of a RV-PA connection, MAPCA closure and PA angioplasty is effective in rehabilitating the hypoplastic PA in patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries. However, PA reinterventions may be required in specific patients.
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Qiuming
authors.initials: Q
authors.lastname: Chen
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Kai
authors.initials: K
authors.lastname: Ma
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Zhongdong
authors.initials: Z
authors.lastname: Hua
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Keming
authors.initials: K
authors.lastname: Yang
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Hao
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Xu
authors.initials: X
authors.lastname: Wang
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Xiaopeng
authors.initials: X
authors.lastname: Hu
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Fuxia
authors.initials: F
authors.lastname: Yan
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Jinping
authors.initials: J
authors.lastname: Liu
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Sen
authors.initials: S
authors.lastname: Zhang
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Lei
authors.initials: L
authors.lastname: Qi
authors.affiliation: Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China drlishoujun@yahoo.com.
authors.forename: Shoujun
authors.initials: S
authors.lastname: Li
mesh_terms: D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011651:Pulmonary Artery / Q000503:physiopathology / Q000601:surgery*; D018633:Pulmonary Atresia / Q000601:surgery*; D012189:Retrospective Studies
keywords: Pulmonary arteries; Pulmonary atresia; Rehabilitation; Right ventricle to pulmonary artery connection