Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia With Ventricular Septal Defect

• Published in: Journal of the American College of Cardiology 2006-04, Vol.47 (7), p.1448-1456
• Main Authors: Amark, Kerstin M., Karamlou, Tara, O’Carroll, Aoife, MacDonald, Cathy, Freedom, Robert M., Yoo, Shi-Joon, Williams, William G., Van Arsdell, Glen S., Caldarone, Christopher A., McCrindle, Brian W.
• Format: Article
• Language: English
• Subjects: Adolescent; Age; Angiography; Aortography; aortopulmonary collateral arteries; Biological and medical sciences; Bronchi - blood supply; Cardiac and Cardiovascular Systems; Cardiology; Cardiology. Vascular system; Child; Child, Preschool; Collateral Circulation; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; construction; Data analysis; Estimates; experience; fallot; Female; Heart; Heart Septal Defects, Ventricular - complications; Heart Septal Defects, Ventricular - diagnostic imaging; Heart Septal Defects, Ventricular - mortality; Heart Septal Defects, Ventricular - surgery; Humans; infancy; Infant; Infant, Newborn; Kardiologi; life; Lung - blood supply; Male; Medical sciences; Mortality; Patients; Pulmonary arteries; Pulmonary Artery - diagnostic imaging; Pulmonary Atresia - complications; Pulmonary Atresia - diagnostic imaging; Pulmonary Atresia - mortality; Pulmonary Atresia - surgery; Reoperation - statistics & numerical data; Risk Factors; surgical-management; Survival Analysis; tetralogy; Treatment Outcome; unifocalization; Veins & arteries
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2005.10.068

Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia with Ventricular Septal Defect Kerstin M. Amark, Tara Karamlou, Aoife O’Carroll, Cathy MacDonald, Robert M. Freedom, Shi-Joon Yoo, William G. Williams, Glen S. Van Arsdell, Christopher A. Caldarone, Brian W. McCrindle We determined the prevalence of end states and their associated risk factors for patients with pulmonary atresia and ventricular septal defect (PAVSD). Important improvement in outcomes occurred over time associated with a shift in treatment paradigm, emphasizing primary complete repair for those with simple PAVSD and an individualized approach for those with MAPCAs tailored to the adequacy of the pulmonary arterial bed. Surgical decisions should be matched to well-defined bronchopulmonary anatomy. We described morphologic characteristics, particularly pulmonary anatomy, and determined the prevalence of definitive end states and their determinants in children with pulmonary atresia associated with ventricular septal defect (PAVSD). Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes. From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review (n = 171) characterized bronchopulmonary segment arterial supply. A total of 185 patients underwent surgery, and repair was definitive in 75%. Initial operations included systemic-pulmonary artery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review, 118 patients had simple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arteries (MAPCAs). Overall survival from initial operation was 71% at 10 years. Risk factors for death after initial operation included younger age at repair, earlier birth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteries, and initial placement of a systemic-pulmonary artery shunt. Competing-risks analysis for initially palliated patients predicted that after 10 years, 68% achieved complete repair (with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22% died without repair, and 10% remained alive without repair. Reoperations after complete repair occurred in 38 children (27%), with risk factors including older age at pal