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

• Published in: Congenital heart disease Vol. 12; no. 5; p. 610
• Main Authors: Agrawal, Hitesh, Mery, Carlos M, Day, Patrick E, Sexson Tejtel, S Kristen, McKenzie, E Dean, Fraser, Jr, Charles D, Qureshi, Athar M, Molossi, Silvana
• Format: Journal Article
• Language: English
• Published: United States 01.09.2017
• Subjects: Adolescent; Aorta, Thoracic - abnormalities; Aorta, Thoracic - diagnostic imaging; Aorta, Thoracic - surgery; Cardiac Catheterization; Child; Computed Tomography Angiography; Coronary Angiography; Coronary Vessel Anomalies - diagnosis; Coronary Vessel Anomalies - surgery; Coronary Vessels - diagnostic imaging; Coronary Vessels - surgery; Disease Management; Echocardiography; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging, Cine; Male; Retrospective Studies; Surveys and Questionnaires; Vascular Surgical Procedures - methods; congenital heart disease; evaluation and management; survey; anomalous coronary arteries
• ISSN: 1747-0803
• DOI: 10.1111/chd.12511

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. 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. 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%). 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.