title: Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.
abstract: BACKGROUND
  A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation.
  
  
  METHODS
  The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals.
  
  
  RESULTS
  Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%).
  
  
  CONCLUSIONS
  A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia.
authors.forename: Jerome
authors.initials: J
authors.lastname: Soquet
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia.
authors.forename: Matthew
authors.initials: M
authors.lastname: Liava'a
authors.affiliation: Department of Cardiology, the Royal Children's Hospital, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia.
authors.forename: Lucas
authors.initials: L
authors.lastname: Eastaugh
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia; Department of Pediatrics, University of Melbourne, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia.
authors.forename: Johann
authors.initials: J
authors.lastname: Brink
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia; Department of Pediatrics, University of Melbourne, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia; Department of Pediatrics, University of Melbourne, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D000792:Angiography; D003097:Collateral Circulation; D006345:Heart Septal Defects, Ventricular / Q000534:rehabilitation / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011651:Pulmonary Artery / Q000002:abnormalities / Q000601:surgery; D018633:Pulmonary Atresia / Q000534:rehabilitation / Q000601:surgery*; D016896:Treatment Outcome