Author + information
- Received December 15, 2014
- Revision received April 15, 2015
- Accepted April 16, 2015
- Published online November 1, 2015.
- Richard Lorber, MD∗,
- Shubhika Srivastava, MBBS†,
- Travis J. Wilder, MD‡,
- Susan McIntyre, RN‡,
- William M. DeCampli, MD, PhD‡,§∗ ( )(, )
- William G. Williams, MD‡,
- Peter C. Frommelt, MD‖,
- Ira A. Parness, MD†,
- Eugene H. Blackstone, MD∗,‡,¶,
- Marshall L. Jacobs, MD‡,
- Luc Mertens, MD, PhD#,
- Julie A. Brothers, MD∗∗,
- J. René Herlong, MD††,
- AAOCA Working Group of the Congenital Heart Surgeons Society
- ∗Children’s Hospital of San Antonio, Baylor College of Medicine, Houston, Texas
- †Kravis Children’s Hospital at Mount Sinai, New York, New York
- ‡Congenital Heart Surgeons’ Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada
- §Arnold Palmer Hospital for Children, Orlando, Florida
- ‖Children’s Hospital of Wisconsin, Milwaukee, Wisconsin
- ¶Heart and Vascular Institute, The Cleveland Clinic, Cleveland, Ohio
- #Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada
- ∗∗Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
- ††Sanger Heart and Vascular Institute-Charlotte Pediatric Cardiology, Charlotte, North Carolina
- ↵∗Reprint requests and correspondence:
Dr. William M. DeCampli, Arnold Palmer Hospital for Children, University of Central Florida College of Medicine, 92 West Miller Street, MP307, Orlando, Florida 32806.
Objectives 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.
This study was partially funded by the Michael H. Ludwig Memorial Foundation; Children’s Heart Foundation, Chicago; Cardiac Center at The Children’s Hospital of Philadelphia; Cardiovascular and Critical Care Research Center, Children’s Hospitals and Clinics of Minnesota; and the Cardiac Surgery Research and Teaching Fund, Hospital for Sick Children, Toronto. All authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Lorber and Srivastava have contributed equally to this work.
- Received December 15, 2014.
- Revision received April 15, 2015.
- Accepted April 16, 2015.
- American College of Cardiology Foundation