Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome

Eur J Cardiothorac Surg. 2012 Aug;42(2):235-40; discussion 240-1. doi: 10.1093/ejcts/ezr310. Epub 2012 Mar 7.


Objectives: Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.

Methods: This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).

Results: There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.

Conclusions: The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.

MeSH terms

  • Alagille Syndrome / surgery*
  • Blood Vessel Prosthesis Implantation / methods
  • Collateral Circulation / physiology
  • Female
  • Heart Septal Defects, Ventricular / diagnostic imaging
  • Heart Septal Defects, Ventricular / physiopathology
  • Heart Septal Defects, Ventricular / surgery*
  • Heart Ventricles / surgery
  • Humans
  • Infant
  • Length of Stay
  • Male
  • Pulmonary Artery / surgery
  • Pulmonary Atresia / diagnostic imaging
  • Pulmonary Atresia / physiopathology
  • Pulmonary Atresia / surgery*
  • Radiography
  • Retrospective Studies
  • Treatment Outcome