title: Anomalous Aortic Origin of Coronary Arteries in the Young: Echocardiographic Evaluation With Surgical Correlation.
abstract: OBJECTIVE
  This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a "best practice" protocol for imaging and interpreting images in establishing the diagnosis of AAOCA.
  
  
  BACKGROUND
  AAOCA is associated with sudden death in the young. Underlying anatomic risk factors that can cause ischemia-related events include coronary arterial ostial stenosis, intramural course of the proximal coronary within the aortic wall, interarterial course, and potential compression between the great arteries. Consistent protocols for diagnosing and evaluating these features are lacking, potentially precluding the ability to risk stratify patients based on evidence and plan surgical strategy.
  
  
  METHODS
  For a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiographic reports were calculated. For each feature, agreement among institutional echocardiographic reports, ICL review of images, and surgical reports was evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed heuristically to reduce differences between institutional reports and ICL review.
  
  
  RESULTS
  A total of 13%, 33%, and 62% of echocardiograms were missing images enabling diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. There was poor agreement between institutional reports and ICL review for diagnosis of origin of coronary artery, interarterial course, intramural course, and acute angle takeoff (kappa = 0.74, 0.11, -0.03, 0.13, respectively). Surgical findings were also significantly different from those of reports, and to a lesser extent ICL reviews. The resulting protocol contains technical recommendations for imaging each of these features.
  
  
  CONCLUSIONS
  Poor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based risk stratification and surgical planning. Even then, delineation of echocardiographic details in AAOCA will remain imperfect.
authors.affiliation: Children's Hospital of San Antonio, Baylor College of Medicine, Houston, Texas.
authors.forename: Richard
authors.initials: R
authors.lastname: Lorber
authors.affiliation: Kravis Children's Hospital at Mount Sinai, New York, New York.
authors.forename: Shubhika
authors.initials: S
authors.lastname: Srivastava
authors.affiliation: Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Travis J
authors.initials: TJ
authors.lastname: Wilder
authors.affiliation: Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Susan
authors.initials: S
authors.lastname: McIntyre
authors.affiliation: Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada; Arnold Palmer Hospital for Children, Orlando, Florida. Electronic address: william.decampli@orlandohealth.com.
authors.forename: William M
authors.initials: WM
authors.lastname: DeCampli
authors.affiliation: Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.affiliation: Children's Hospital of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Frommelt
authors.affiliation: Kravis Children's Hospital at Mount Sinai, New York, New York.
authors.forename: Ira A
authors.initials: IA
authors.lastname: Parness
authors.affiliation: Children's Hospital of San Antonio, Baylor College of Medicine, Houston, Texas; Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Eugene H
authors.initials: EH
authors.lastname: Blackstone
authors.affiliation: Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Marshall L
authors.initials: ML
authors.lastname: Jacobs
authors.affiliation: Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Julie A
authors.initials: JA
authors.lastname: Brothers
authors.affiliation: Sanger Heart and Vascular Institute-Charlotte Pediatric Cardiology, Charlotte, North Carolina.
authors.forename: J René
authors.initials: JR
authors.lastname: Herlong
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001011:Aorta / Q000002:abnormalities / Q000000981:diagnostic imaging* / Q000601:surgery*; D019985:Benchmarking; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D003331:Coronary Vessels / Q000000981:diagnostic imaging* / Q000601:surgery*; D018618:Echocardiography, Doppler, Color / Q000592:standards*; D017548:Echocardiography, Transesophageal / Q000592:standards*; D005260:Female; D006801:Humans; D059021:Laboratory Proficiency Testing; D008297:Male; D015588:Observer Variation; D011237:Predictive Value of Tests; D012042:Registries; D015203:Reproducibility of Results; D016896:Treatment Outcome; D014481:United States; D054079:Vascular Malformations / Q000000981:diagnostic imaging* / Q000601:surgery*
keywords: anomalous; coronary artery; intraconal AAOCA; intramural; origin interarterial