title: Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries.
abstract: BACKGROUND
  One anatomic variant of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is characterized by the absence of intrapericardial pulmonary arteries. This anatomy obviates the possibility of incorporating the pulmonary arteries for reconstruction or palliative procedures. The purpose of this study was to evaluate the surgical results in patients undergoing repair of PA/VSD/MAPCAs with absent pulmonary arteries.
  
  
  METHODS
  This was a retrospective review of 35 patients who underwent surgical repair of PA/VSD/MAPCAs with absent pulmonary arteries between 2007 and 2014. The median age at the time of surgery was 3.4 months, and the median weight was 4.9 kg. All patients underwent unifocalization of MAPCAs, with an average of 3.5 ± 1.4 MAPCAs per patient.
  
  
  RESULTS
  Twenty-eight of the 35 patients (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, VSD closure, and right ventricle to pulmonary artery conduit. After complete repair, the average right ventricular to aortic pressure ratio was 0.33 ± 0.07. There were no deaths in this subgroup. Seven patients (20%) were not deemed suitable candidates for VSD closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative death and 1 interim death. Three patients have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment.
  
  
  CONCLUSIONS
  The majority of patients with PA/VSD/MAPCAs and absent pulmonary arteries can undergo complete single-stage repair with satisfactory postoperative hemodynamics. These results suggest that unifocalization of MAPCAs can provide a reasonable pulmonary vascular bed in the absence of intrapericardial pulmonary arteries.
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: Sergio A
authors.initials: SA
authors.lastname: Carrillo
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California. Electronic address: mainwaring@stanford.edu.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Mainwaring
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: William L
authors.initials: WL
authors.lastname: Patrick
authors.affiliation: Division of Pediatric Cardiology, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: Holly D
authors.initials: HD
authors.lastname: Bauser-Heaton
authors.affiliation: Division of Pediatric Cardiology, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: Lynn
authors.initials: L
authors.lastname: Peng
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: V Mohan
authors.initials: VM
authors.lastname: Reddy
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
authors.forename: Frank L
authors.initials: FL
authors.lastname: Hanley
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006343:Heart Septal Defects / Q000150:complications* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D010496:Pericardium; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*; D018633:Pulmonary Atresia / Q000150:complications* / Q000601:surgery*; D012189:Retrospective Studies; D054079:Vascular Malformations / Q000150:complications* / Q000601:surgery*