Open Access

ARTICLE

Current practices are variable in the evaluation and management of patients with anomalous aortic origin of a coronary artery: Results of a survey

Hitesh Agrawal^1,2, Carlos M. Mery^1,3, Patrick E. Day⁴, S. Kristen Sexson Tejtel^1,2, E. Dean McKenzie^1,3,5, Charles D. Fraser Jr^1,3, Athar M. Qureshi^1,2, Silvana Molossi^1,2

1 Coronary Anomalies Program, Texas Children’s Hospital, Houston, Texas
2 The Lillie Frank Abercrombie Section of Cardiology, Baylor College of Medicine, Texas Children’s Hospital, Houston, Texas
3 Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas
4 McGovern Medical School at The University of Texas Health Science Center at Houston, Houston, Texas
5 Division of Cardiothoracic Surgery, Children’s Healthcare of Atlanta, Atlanta, Georgia

* Corresponding Author: Silvana Molossi, Baylor College of Medicine, Texas Children’s Hospital, 6621 Fannin Street, WT 19345-C, Houston, TX 77030. Email:

Congenital Heart Disease 2017, 12(5), 610-614. https://doi.org/10.1111/chd.12511

Abstract

Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner.
Methods: An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed.
Results: A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty-eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty-two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty-eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%).
Conclusion: There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long-term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery.

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