title: Anomalous aortic origin of a coronary artery: toward a standardized approach.
abstract: Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients.
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas. Electronic address: cmmery@texaschildrens.org.
authors.forename: Carlos M
authors.initials: CM
authors.lastname: Mery
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Division of Pediatric Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.
authors.forename: Silvana M
authors.initials: SM
authors.lastname: Lawrence
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; E. B. Singleton Department of Pediatric Radiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Krishnamurthy
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Division of Pediatric Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.
authors.forename: S Kristen
authors.initials: SK
authors.lastname: Sexson-Tejtel
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Outcomes & Impact Service, Texas Children's Hospital, Houston, Texas.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Carberry
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.
authors.forename: E Dean
authors.initials: ED
authors.lastname: McKenzie
authors.affiliation: Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.
authors.forename: Charles D
authors.initials: CD
authors.lastname: Fraser
mesh_terms: D000465:Algorithms; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality / Q000592:standards*; D002309:Cardiology / Q000592:standards*; D003330:Coronary Vessel Anomalies / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D019091:Critical Pathways / Q000592:standards; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D003952:Diagnostic Imaging / Q000592:standards; D006801:Humans; D011237:Predictive Value of Tests; D015995:Prevalence; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
keywords: anomalous aortic origin of a coronary artery; coronary anomalies; sudden cardiac death