title: Outcomes of the rehabilitative procedure for patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries beyond the infant period.
abstract: OBJECTIVE
  To determine the effect and safeness of the right ventricle to pulmonary artery connection with occlusion of major aortopulmonary collaterals and pulmonary artery angioplasty to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect beyond the infant period.
  
  
  METHODS
  From December 2009 to August 2012, 37 consecutive patients (mean age 1.9 ± 1.7 years, range 0.6-7.2 years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index 90.9 ± 42.6 mm(2)/m(2); McGoon ratio 1.0 ± 0.2) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, during which most of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was 1.6 ± 0.8 years (range 0.6-3.3 years). Continuous variables are expressed as means ± standard deviation.
  
  
  RESULTS
  There were no early deaths, but one patient died of myocarditis 1 year after the rehabilitation. Significant pulmonary artery growth was obtained (Nakata index 215.1 ± 95.1 mm(2)/m(2), P < 0.001; McGoon ratio 1.6 ± 0.5, P < 0.001) in all of the 37 patients, and among them, 17 patients (45.9%) whose pulmonary growth was considered adequate obtained a complete repair without perforation of the ventricular septal defect. The preoperative McGoon ratio might be a good predictor for adequate pulmonary growth. There was one early death after anatomical repair. At the last visit, all survivors who underwent anatomical repair were in New York Heart Association class I-II with satisfactory haemodynamics.
  
  
  CONCLUSIONS
  Connection of the right ventricle to the pulmonary artery is safe and effective to promote the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, this strategy allows complete repair in the majority of patients beyond infancy.
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Yonghui
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Zhongdong
authors.initials: Z
authors.lastname: Hua
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Keming
authors.initials: K
authors.lastname: Yang
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Hao
authors.initials: H
authors.lastname: Zhang
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Yan
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Xu
authors.initials: X
authors.lastname: Wang
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Junmin
authors.initials: J
authors.lastname: Chu
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors.forename: Kai
authors.initials: K
authors.lastname: Ma
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China drlishoujun@yahoo.com.
authors.forename: Shoujun
authors.initials: S
authors.lastname: Li
mesh_terms: D017130:Angioplasty / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D010166:Palliative Care; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*; D018633:Pulmonary Atresia / Q000453:epidemiology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Major aortopulmonary collaterals; Pulmonary atresia; Right ventricle to pulmonary artery connection; Ventricular septal defect