TY - CHAP M1 - Book, Section TI - Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals A1 - MacDonald, Malcolm J. A1 - Hanley, Frank L. A2 - Yuh, David D. A2 - Vricella, Luca A. A2 - Yang, Stephen C. A2 - Doty, John R. Y1 - 2014 N1 - T2 - Johns Hopkins Textbook of Cardiothoracic Surgery AB - EpidemiologyPulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with great morphologic variability that represents approximately 2 percent of congenital heart defects, with a prevalence of 0.07 per 1000 live births.MorphologyThis particular malformation entails the association of atresia of the pulmonary valve, a tetralogy-type VSD, and pulmonary collateral vessels that usually originate from the descending thoracic aorta.Clinical featuresNeonates born with PA, VSD, and MAPCAs may have very unpredictable presentation owing to the anatomic variability of the lesion. Patients may be minimally symptomatic or severely cyanotic, or may develop congestive heart failure (CHF) from pulmonary overcirculation.DiagnosisDiagnostic evaluation includes echocardiography, cardiac catheterization, and, in selected cases, computed tomographic angiography (CTA) to clarify the anatomy of the MAPCAs.TreatmentDefinitive treatment is surgical. The ultimate goal of surgical therapy is creation of separated, in-series pulmonary and systemic circulations. Achievement of this goal requires unifocalization of the vascular systems, VSD closure, and reconstruction of the right ventricular outflow tract (RVOT). “Single-stage” unifocalization with or without concurrent VSD closure is favored by the authors.OutcomesIn over 460 patients managed by the authors’ protocol, complete unifocalization via median sternotomy was achieved in 76 percent of patients. Intracardiac repair (VSD closure) was possible at initial operation in 56 percent. Ninety-five percent of patients were completely repaired by 5 years of age. Recent operative mortality was 2.3 percent, with a 5-year actuarial survival of 85.5 percent. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesssurgery.mhmedical.com/content.aspx?aid=1104596357 ER -