European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
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Eur J Cardiothorac Surg · Aug 2012
Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome.
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. ⋯ 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.