Author + information
- Richard Lorber, MD,
- Shubhika Srivastava, MBBS,
- Travis J. Wilder, MD,
- Susan McIntyre, RN,
- William M. DeCampli, MD, PhD∗ (, )
- William G. Williams, MD,
- Peter Frommelt, MD,
- Ira A. Parness, MD,
- Eugene H. Blackstone, MD,
- Marshall Jacobs, MD,
- Luc Mertens, MD, PhD,
- Julie Brothers, MD,
- J. René Herlong, MD,
- AAOCA Working Group of the Congenital Heart Surgeons’ Society
- ↵∗Pediatric Cardiovascular Surgery, Arnold Palmer Hospital for Children, University of Central Florida College of Medicine, 92 West Miller Street, Orlando, Florida 32806
The authors thank Dr. Angelini for his insightful comments about the diagnosis and management of anomalous aortic origin of a coronary artery (AAOCA). As is evident from Dr. Angelini’s letter, agreement on universally accepted nomenclature for this entity remains elusive. AAOCA has been chosen by the International Society for Nomenclature of Paediatric and Congenital Heart Disease; it is used in North America by the Society of Thoracic Surgeons and in Europe by the Association for European Paediatric and Congenital Cardiology. The Congenital Heart Surgeons’ Society (CHSS) has proposed using AAOCA when a coronary artery ostium arises outside the normal sinus of origin, in combination with additional descriptors regarding the proximal course and other important features (1). We recommend that this nomenclature continue to be used for clarity and uniformity.
We sought to address deficiencies in echocardiographic imaging of coronary artery origins and proximal courses. Because such imaging is most often diagnostic in the young, routine imaging of the coronary arteries as part of a pediatric echocardiogram is recommended. Additionally, echocardiographic imaging is the most practical and widely used imaging modality to screen for congenital coronary anomalies. Guidance on performing this imaging seemed warranted, and we provided this based on a review of studies from a large multicenter cohort (2).
There are many controversies in the management of AAOCA patients. The ongoing longitudinal studies from the CHSS AAOCA Registry are the most comprehensive efforts to date to provide data-driven inferences that address controversies regarding diagnosis, treatment, and follow-up. Chief among these is management of asymptomatic right AAOCA, which is more common than interarterial left AAOCA but much less lethal. Because data are lacking regarding risk stratification, management recommendations vary widely. Without a clear evidence-based management strategy, it is hard to justify screening asymptomatic individuals for AAOCA. We propose that the ethics and public health implications of such screening be discussed before championing a certain method of screening.
Dr. Angelini correctly points out the differences in the yield for diagnosing AAOCA with echocardiography in youths compared with adults. It is well known that sudden death risk attributable to these anomalies is greater in the young than in the adult population. Because of this important difference, the CHSS Registry and our study are based on pediatric and young adult patients. Dr. Angelini also correctly points out that evaluating patients with AAOCA in the modern era involves not only echocardiography, but also additional imaging and functional studies. There is a lack of consensus among the congenital heart disease community as to which studies are indicated. This will be the topic of future investigations.
Please note: The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- American College of Cardiology Foundation