Bahaaldin Alsoufi1, Brian Schlosser2, Courtney McCracken2, Brian Kogon3, Kirk Kanter3, William Border2, Ritu Sachdeva2. 1. Division of Cardiothoracic Surgery, Department of Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia. Electronic address: balsoufi@hotmail.com. 2. Division of Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia. 3. Division of Cardiothoracic Surgery, Department of Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia.
Abstract
BACKGROUND: Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes. METHODS: Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed. RESULTS: Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3.2 kg (interquartile range, 2.5 to 3.8 kg). Eleven patients (28%) were born prematurely at or before 36 weeks' gestation, and 10 (25%) had genetic/extracardiac malformations. Mean APW size was 0.84 ± 0.28 cm, and by the Mori classification was type I in 17 patients (43%), type II in 18 (45%), and type III in 5 (13%). Twenty-five patients (63%) had simple APW, with no associated cardiac lesions other than atrial septal defect or patent ductus arteriosus, whereas 15 (38%) had complex APW with one or more associated lesions, including interrupted aortic arch (n = 6), ventricular septal defect (n = 6), or other (n = 5). There were no hospital or late deaths. Four patients required cardiac reoperations, 3 of whom had interrupted aortic arch. The 10-year freedom from cardiac reoperation was 100% for simple APW vs 73% for complex APW (p = 0.008), with 75% of reoperations related to aortic obstruction. Age, weight, prematurity, extracardiac anomalies, APW size and type, and APW repair technique were not associated with reoperation risk. CONCLUSIONS: Current outcomes of early repair of APW are excellent, including infants with complex associated cardiac lesions. Compared with historic results, contemporary outcomes are favorable, supporting early and complete repair of APW and associated lesions. Cardiac reoperation can be required in complex APW, mainly with concomitant arch repair, and is usually related to aortic obstruction.
BACKGROUND: Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes. METHODS: Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed. RESULTS: Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3.2 kg (interquartile range, 2.5 to 3.8 kg). Eleven patients (28%) were born prematurely at or before 36 weeks' gestation, and 10 (25%) had genetic/extracardiac malformations. Mean APW size was 0.84 ± 0.28 cm, and by the Mori classification was type I in 17 patients (43%), type II in 18 (45%), and type III in 5 (13%). Twenty-five patients (63%) had simple APW, with no associated cardiac lesions other than atrial septal defect or patent ductus arteriosus, whereas 15 (38%) had complex APW with one or more associated lesions, including interrupted aortic arch (n = 6), ventricular septal defect (n = 6), or other (n = 5). There were no hospital or late deaths. Four patients required cardiac reoperations, 3 of whom had interrupted aortic arch. The 10-year freedom from cardiac reoperation was 100% for simple APW vs 73% for complex APW (p = 0.008), with 75% of reoperations related to aortic obstruction. Age, weight, prematurity, extracardiac anomalies, APW size and type, and APW repair technique were not associated with reoperation risk. CONCLUSIONS: Current outcomes of early repair of APW are excellent, including infants with complex associated cardiac lesions. Compared with historic results, contemporary outcomes are favorable, supporting early and complete repair of APW and associated lesions. Cardiac reoperation can be required in complex APW, mainly with concomitant arch repair, and is usually related to aortic obstruction.