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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

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

Kerstin M. Amark, MD^_*, Tara Karamlou, MD, Aoife O’Carroll, MS, Cathy MacDonald, MD, Robert M. Freedom, MD, Shi-Joon Yoo, MD, William G. Williams, MD, Glen S. Van Arsdell, MD, Christopher A. Caldarone, MD and Brian W. McCrindle, MD, MPH^,_*

^_* Department of Pediatric Cardiology, Göteborg University, The Queen Silvia Children’s Hospital, Göteborg, Sweden
Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Division of Diagnostic Imaging, Department of Radiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

Manuscript received June 1, 2005; revised manuscript received October 4, 2005, accepted October 10, 2005.

^_* Reprint requests and correspondence: Dr. Brian W. McCrindle, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8 (Email: brian.mccrindle{at}sickkids.ca).

OBJECTIVES: 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).

BACKGROUND: Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes.

METHODS: From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review (n = 171) characterized bronchopulmonary segment arterial supply.

RESULTS: 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 palliation, MAPCAs, and more segments supplied by collaterals.

CONCLUSIONS: Outcomes in children with PAVSD have improved over time, and are better in completely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.

Abbreviations and Acronyms   MAPCA = major aortopulmonary collateral arteries   PAVSD = pulmonary atresia associated with ventricular septal defect   RVOT = right ventricular outflow tract

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