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Section of Cardiac Surgery, Ward Family Center for Congenital Heart Disease, Children's Mercy Hospitals & Clinics, University of Missouri, Kansas City, MO 64108, USA.
Pulmonary atresia with ventricular septal defect and major aorta pulmonary collaterals arteries is a rare and complex congenital cardiac defect. There is considerable variability in the anatomy, morphology, and geometry of the native pulmonary arteries and the collateral vessels. While the ultimate goal of therapy is a biventricular correction with complete unifocalization, establishment of right ventricular to pulmonary arterial continuity, and closure of all intracardiac defects, achieving this endpoint can be frustrating and difficult. A carefully considered approach for each individual patient is required. Patients with appropriate anatomy may undergo a definitive single-stage unifocalization and biventricular correction in early infancy. Patients with less favorable anatomy will require a more eclectic approach. While our knowledge of the genetics of this defect is rudimentary, further advances in genetic understanding and technology hold tremendous promise for the development of future therapies.
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