BACKGROUND: One anatomic variant of @entity1146 with @entity1757 and major aortopulmonary collaterals (PA/ @entity1757 /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 @entity1 undergoing repair of PA/ @entity1757 /MAPCAs with absent pulmonary arteries. METHODS: This was a retrospective review of 35 @entity1 who underwent surgical repair of PA/ @entity1757 /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 @entity1 underwent unifocalization of MAPCAs, with an average of 3.5     1.4 MAPCAs per @entity1 . RESULTS: Twenty-eight of the 35 @entity1 (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, @entity1757 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 @entity1 (20%) were not deemed suitable candidates for @entity1757 closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative @entity204 and 1 interim @entity204 . Three @entity1 have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment. CONCLUSIONS: The majority of @entity1 with PA/ @entity1757 /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.

Surgical Repair of @entity1146 With XXXX With Absent Intrapericardial Pulmonary Arteries.

@entity1146 :: ('MESH:D018633', 'Disease') :: ['pulmonary atresia'] @entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity1757 :: ('MESH:D006345', 'Disease') :: ['VSD', 'ventricular septal defect']
@entity1757 :: (MESH:D006345,Disease) :: ['Ventricular Septal Defect and Major Aortopulmonary Collaterals']
