Agrawal Hitesh, Mery Carlos M, Day Patrick E, Sexson Tejtel S Kristen, McKenzie E Dean, Fraser Charles D, Qureshi Athar M, Molossi Silvana
Coronary Anomalies Program, Texas Children's Hospital, Houston, Texas.
The Lillie Frank Abercrombie Section of Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.
Congenit Heart Dis. 2017 Sep;12(5):610-614. doi: 10.1111/chd.12511. Epub 2017 Jul 2.
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.
冠状动脉异常起源（AAOCA）是美国年轻运动员心源性猝死的第二大主要原因。迄今为止，这些患者的长期预后数据尚缺乏。对于如何最佳治疗这些患者，人们了解不足，且治疗方式并不统一。
向198名心脏医疗服务提供者发送了一份关于AAOCA治疗的包含15个问题的在线调查问卷。目的是确定知识差距，以证明有必要设立一个专门的科学论坛来讨论AAOCA。进行了描述性统计。
共有91名提供者（46%）完成了调查，包括儿科心脏病专科医生（40%）、普通儿科心脏病医生（24%）、心血管（CV）外科医生（22%）、成人心脏病医生（10%）、执业护士（8%）、心脏病学研究员（3%）和CV麻醉医生（1%）。48%的人从业超过15年，28%的人处于执业的前5年。52%的提供者诊治成人患者，93%的人诊治儿童/青少年患者。88%的人隶属于学术机构。除一名提供者外，所有提供者均在美国执业，62%的人在得克萨斯州执业。一半的参与者（50%）对管理AAOCA患者非常有信心，36%的人有一定信心。提供者使用各种成像检查来确认解剖结构，包括计算机断层扫描血管造影88%、心脏磁共振成像70%、心导管检查60%、超声心动图12%、血管内超声2%和心肌灌注扫描1%。大多数人在为家属提供咨询方面感到得心应手，并认为根据病变类型，这些患者应接受手术转诊（85%）而非临床随访（67%），并限制运动（65%）。
目前AAOCA患者的评估和治疗方式存在异质性。即使是来自学术机构的参与者也存在知识差距。采用明确的治疗方法获取长期数据可能有助于改善预后，避免不必要的运动限制或手术。
