Surgical management of aortopulmonary window associated with interrupted aortic arch: A Congenital Heart Surgeons Society study Igor E. Konstantinov, MD, PhD, a Tara Karamlou, MD, a William G. Williams, MD, a Jan M. Quaegebeur, MD, b Pedro J. del Nido, MD, c Thomas L. Spray, MD, d Christopher A. Caldarone, MD, b Eugene H. Blackstone, MD, e Brian W. McCrindle, MD, MPH, a for the members of the Congenital Heart Surgeons Society Supplemental material is available online. Objective: The objective was to determine outcomes and risk factors of surgical management of patients with aortopulmonary window associated with interrupted aortic arch. Methods: From 1987 to 1997, 472 neonates with interrupted aortic arch were enrolled prospectively from 33 institutions. Associated aortopulmonary window was present in 20 patients. Competing risk methodology determined the prevalence of reintervention for postrepair pulmonary artery and aortic arch obstruction. Results: Interrupted aortic arch was type A in 17 patients and type B in 3 patients. Aortopulmonary window morphology was type I (n = 10), type II (n = 5), and type III (n = 5). Associated cardiovascular anomalies were common, including atrial septal defect (n = 13) and systemic venous anomalies (n = 3). Overall survival after initial admission was 91%, 86%, and 84% at 1, 5, and 10 years, respectively. Fifteen patients underwent single-stage repair, and 4 patients underwent staged repair. There was an increased prevalence of patch augmentation of the interrupted aortic arch anastomosis in lower-weight infants (2.3 kg vs 3.1 kg, P = .07). Competing risk analysis estimated that 5 years after repair, 51% had initial arch reintervention, 6% had initial pulmonary artery reintervention, and 43% were alive without rein- tervention. Reintervention for arch obstruction was more likely for those with interrupted aortic arch type B (P = .08) and for those with higher weight at initial repair (P = .003). Conclusions: Complete correction of aortopulmonary window in the setting of interrupted aortic arch can be performed with low mortality in the neonatal period. Reinterventions for aortic arch obstruction are the most frequent complication after repair, but pulmonary artery stenosis also occurs. Use of patch augmentation may reduce the need for subsequent arch reintervention. A ortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary trunk, occurring above 2 normally formed semi- lunar valves. 1,2 Isolated APW occurs in 0.2% of cases of congenital heart disease 3,4 and is accompanied by other cardiac anomalies in 52% of cases. 5 Interrupted aortic arch (IAA) is the most frequently associated major lesion. The combination of APW and IAA is rare, and the cumulative experience with this combination of lesions is limited to isolated case reports. 3,4,6 –10 The presence of IAA with APW is a risk factor for death among those with APW. 2 The surgical management of APW and IAA is necessarily complex, yet its rarity has precluded multivariable analysis from which therapeutic inferences could be derived. We sought to identify morphologic and procedural variables that are associated with From the a Hospital for Sick Children, To- ronto, Canada; b Children’s Hospital of New York, New York; c Children’s Hospital of Boston, Boston, Mass; d Children’s Hospital of Philadelphia, Philadelphia, Pa; e Cleve- land Clinic, Cleveland, Ohio. Received for publication Jan 8, 2005; revi- sions received Feb 28, 2005; accepted for publication March 23, 2005. Address for reprints: Brian W. Mc- Crindle, MD, The Hospital for Sick Chil- dren 555 University Avenue, Toronto, Ontario, Canada M5G 1X8 (E-mail: brian.mccrindle@sickkids.ca). J Thorac Cardiovasc Surg 2006;131:1136-41 0022-5223/$32.00 Copyright © 2006 by The American Asso- ciation for Thoracic Surgery doi:10.1016/j.jtcvs.2005.03.051 Surgery for Congenital Heart Disease Konstantinov et al 1136 The Journal of Thoracic and Cardiovascular Surgery ● May 2006 CHD