Eva W Cheung1, Marc E Richmond2, Mariel E Turner2, Emile A Bacha3, Alejandro J Torres2. 1. Department of Pediatrics, Division of Critical Care Medicine, Columbia University, New York, New York. Electronic address: ec2335@columbia.edu. 2. Department of Pediatrics, Division of Cardiology, Columbia University, New York, New York. 3. Department of Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, Columbia University, New York, New York.
Abstract
BACKGROUND: We investigated the influence of coronary artery abnormalities on outcome in patients with pulmonary atresia/intact ventricular septum (PA-IVS) for planned single-ventricle palliation. METHODS: Catheterization and medical records were reviewed in patients with PA-IVS for planned single-ventricle palliation at our institution between 2000 and 2012. Primary outcome was death or transplantation. Patients with confirmed or strong suspicion of stenosis in 2 or more main coronary arteries or coronary ostial atresia were defined as having right ventricle-dependent coronary circulation (RVDCC); those with stenosis of 1 main vessel or normal anatomy were defined as having non-RVDCC. RESULTS: Of 58 patients with PA-IVS, 17 (30%) underwent single-ventricle palliation. Ten (59%) had RVDCC (3 with ostial atresia) and 7 (41%) had non-RVDCC. Median follow-up time was 8.2 years (0 months-11.3 years), with 1 patient in each group lost to follow-up. Five patients with RVDCC died, including the 3 patients with ostial atresia, and 1 underwent transplantation at 6 months of life. No deaths occurred after second-stage palliation. Three of the 4 surviving patients with RVDCC completed a Fontan operation, and 2 of these patients had evidence of cardiac ischemia on follow-up. No deaths occurred among patients with non-RVDCC. Kaplan-Meier analysis demonstrated significantly better survival in patients with non-RVDCC (100%) than in patients with RVDCC (40%) (p = 0.026). CONCLUSIONS: In patients with PA-IVS undergoing single-ventricle palliation, RVDCC is associated with high early mortality, especially with coronary ostial atresia. There should be early consideration of transplantation in neonates with RVDCC. Patients with non-RVDCC undergoing single-ventricle palliation have excellent long-term outcomes, with no mortality seen in this series.
BACKGROUND: We investigated the influence of coronary artery abnormalities on outcome in patients with pulmonary atresia/intact ventricular septum (PA-IVS) for planned single-ventricle palliation. METHODS: Catheterization and medical records were reviewed in patients with PA-IVS for planned single-ventricle palliation at our institution between 2000 and 2012. Primary outcome was death or transplantation. Patients with confirmed or strong suspicion of stenosis in 2 or more main coronary arteries or coronary ostial atresia were defined as having right ventricle-dependent coronary circulation (RVDCC); those with stenosis of 1 main vessel or normal anatomy were defined as having non-RVDCC. RESULTS: Of 58 patients with PA-IVS, 17 (30%) underwent single-ventricle palliation. Ten (59%) had RVDCC (3 with ostial atresia) and 7 (41%) had non-RVDCC. Median follow-up time was 8.2 years (0 months-11.3 years), with 1 patient in each group lost to follow-up. Five patients with RVDCC died, including the 3 patients with ostial atresia, and 1 underwent transplantation at 6 months of life. No deaths occurred after second-stage palliation. Three of the 4 surviving patients with RVDCC completed a Fontan operation, and 2 of these patients had evidence of cardiac ischemia on follow-up. No deaths occurred among patients with non-RVDCC. Kaplan-Meier analysis demonstrated significantly better survival in patients with non-RVDCC (100%) than in patients with RVDCC (40%) (p = 0.026). CONCLUSIONS: In patients with PA-IVS undergoing single-ventricle palliation, RVDCC is associated with high early mortality, especially with coronary ostial atresia. There should be early consideration of transplantation in neonates with RVDCC. Patients with non-RVDCC undergoing single-ventricle palliation have excellent long-term outcomes, with no mortality seen in this series.
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