Author + information
- Received October 7, 2016
- Revision received December 5, 2016
- Accepted January 3, 2017
- Published online March 20, 2017.
- Michael K. Cheezum, MDa,b,∗ (, )
- Richard R. Liberthson, MDc,
- Nishant R. Shah, MD, MPH, MScd,
- Todd C. Villines, MDe,
- Patrick T. O’Gara, MDa,
- Michael J. Landzberg, MDf and
- Ron Blankstein, MDa
- aDepartments of Medicine and Radiology, Cardiovascular Division, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
- bDepartment of Medicine, Cardiology Service, Fort Belvoir Community Hospital, Ft. Belvoir, Virginia
- cDepartment of Medicine, Division of Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
- dLifespan Cardiovascular Institute, Division of Cardiovascular Medicine, Department of Medicine, Brown University Alpert School of Medicine, Providence, Rhode Island
- eDepartment of Medicine, Cardiology Service, Walter Reed National Military Medical Center, Bethesda, Maryland
- fDepartment of Cardiology, Boston Children’s Hospital, Boston, Massachusetts
- ↵∗Address for correspondence:
Dr. Michael K. Cheezum, Brigham and Women's Hospital, Harvard Medical School, Departments of Medicine and Radiology (Cardiovascular Division), 75 Francis Street, Boston, Massachusetts 02115.
Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prevalence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients. Observational data suggests that interarterial ALCA is rare (weighted prevalence = 0.03%; 95% confidence interval [CI]: 0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence = 0.23%; 95% CI: 0.17% to 0.31%). Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge gaps in management.
The opinions and assertions herein are those of the authors alone, and do not represent the views of the U.S. Army, Office of the Surgeon General, Department of Defense, or the U.S. Government. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received October 7, 2016.
- Revision received December 5, 2016.
- Accepted January 3, 2017.