title: Surgical repair of anomalous aortic origin of a coronary artery.
abstract: OBJECTIVE
  Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA.
  
  
  METHODS
  Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia.
  
  
  RESULTS
  Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms.
  
  
  CONCLUSIONS
  The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA mainwaring@stanford.edu.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Mainwaring
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA.
authors.forename: V Mohan
authors.initials: VM
authors.lastname: Reddy
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA Division of Pediatric Cardiac Surgery, Oakland Children's Hospital, Oakland, CA, USA.
authors.forename: Olaf
authors.initials: O
authors.lastname: Reinhartz
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA Division of Pediatric Cardiac Surgery, Children's Hospital of Central California, Madera, CA, USA.
authors.forename: Edwin
authors.initials: E
authors.lastname: Petrossian
authors.affiliation: Division of Pediatric Cardiology, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Punn
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA Division of Pediatric Cardiac Surgery, Oakland Children's Hospital, Oakland, CA, USA Division of Pediatric Cardiac Surgery, Children's Hospital of Central California, Madera, CA, USA.
authors.forename: Frank L
authors.initials: FL
authors.lastname: Hanley
mesh_terms: D000293:Adolescent; D000328:Adult; D000465:Algorithms; D002637:Chest Pain / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000601:surgery*; D004417:Dyspnea / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D017202:Myocardial Ischemia / Q000209:etiology / Q000601:surgery*; D063868:Patient Outcome Assessment; D011183:Postoperative Complications; D011651:Pulmonary Artery / Q000601:surgery; D012189:Retrospective Studies; D013575:Syncope / Q000209:etiology; D055815:Young Adult
keywords: Congenital heart defect (coronary arteries); Coronary artery disease; Coronary artery imaging; Myocardial ischaemia