title: Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals.
abstract: BACKGROUND
  Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.
  
  
  METHODS
  This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.
  
  
  RESULTS
  There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.
  
  
  CONCLUSIONS
  The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA. mainwaring@stanford.edu
authors.forename: Richard D
authors.initials: RD
authors.lastname: Mainwaring
authors.forename: V Mohan
authors.initials: VM
authors.lastname: Reddy
authors.forename: Stanton B
authors.initials: SB
authors.lastname: Perry
authors.forename: Lynn
authors.initials: L
authors.lastname: Peng
authors.forename: Frank L
authors.initials: FL
authors.lastname: Hanley
mesh_terms: D000015:Abnormalities, Multiple / Q000401:mortality / Q000601:surgery*; D000792:Angiography / Q000379:methods; D001028:Aortopulmonary Septal Defect / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D015331:Cohort Studies; D003097:Collateral Circulation / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods; D011182:Postoperative Care; D018633:Pulmonary Atresia / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D011652:Pulmonary Circulation / Q000502:physiology; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D016896:Treatment Outcome