title: Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome.
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.
authors.affiliation: Department of Pediatrics, Division of Critical Care Medicine, Columbia University, New York, New York. Electronic address: ec2335@columbia.edu.
authors.forename: Eva W
authors.initials: EW
authors.lastname: Cheung
authors.affiliation: Department of Pediatrics, Division of Cardiology, Columbia University, New York, New York.
authors.forename: Marc E
authors.initials: ME
authors.lastname: Richmond
authors.affiliation: Department of Pediatrics, Division of Cardiology, Columbia University, New York, New York.
authors.forename: Mariel E
authors.initials: ME
authors.lastname: Turner
authors.affiliation: Department of Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, Columbia University, New York, New York.
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.affiliation: Department of Pediatrics, Division of Cardiology, Columbia University, New York, New York.
authors.forename: Alejandro J
authors.initials: AJ
authors.lastname: Torres
mesh_terms: D017023:Coronary Angiography; D003326:Coronary Circulation; D003330:Coronary Vessel Anomalies / Q000150:complications* / Q000401:mortality / Q000503:physiopathology; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome