Hu Fan, Wang Xinyue, Wan Jiaojiao, Li Yifei, Wang Tao, Zhou Kaiyu, Shi Xiaoqing, Liu Zhongqiang, Fang Jie, Hua Yimin
Key Laboratory of Birth Defects and Related Diseases of Women and Children of MOE, State Key Laboratory of Oral Diseases, Department of Pediatrics, National Clinical Research Center for Oral Diseases, West China Second University Hospital, West China Hospital of Stomatology, Sichuan University, Chengdu, China.
Front Cardiovasc Med. 2022 Jun 9;9:918832. doi: 10.3389/fcvm.2022.918832. eCollection 2022.
An anomalous aortic origin of a coronary artery (AAOCA) has been considered as a dominant cause of sudden cardiac death (SCD) among young age children. Therefore, it is critical to identify AAOCA timely to avoid lethal events. Recently, accumulating cases of right or left coronary arteries originating from inappropriate locations at the sinus of Valsalva have been identified. Here, we report a rare case of AAOCA with an intra-arterial wall course pretending normal migration on imaging screening in a patient who suffered from syncope.
A 7-year-old male without a previous history of cardiovascular and cerebrovascular diseases suddenly suffered from sharp chest pain and syncope after intensive exercise. The electrocardiogram showed that the ST segment of multiple leads was depressed by more than 0.05 mV, and biomarkers indicated severe myocardial injuries. The left ventricular ejection fraction (LVEF) decreased dramatically to 23%. Fulminant myocarditis and cardiomyopathy were therefore excluded. However, a relatively normal coronary artery origin, which arose from the left coronary sinus, presented on echocardiography and cardiac CT angiography (CTA). It is difficult to draw an association between severe clinical manifestations and slight malformations on echocardiography and CTA. Furthermore, selective coronary angiography revealed that an anomalous left coronary artery arose from the superior margin of the inappropriate sinus, developed an intramural wall course and finally exits the left sinus of Valsalva and migrated between the aorta and the pulmonary artery, which induced severe myocardial infarction during exercise. Then, the patient received surgical correction with a modified unroofing procedure. After 2 months of intensive treatment, the patient was discharged and remained asymptomatic through 18 months of follow-up.
AAOCA, especially anomalous left coronary artery (ALCA), represents a major potential risk of SCD. We reported an atypical manifestation of ALCA arising from the inappropriate sinus of Valsalva and merging into the intra-arterial wall to develop a strange course and then sprout between the aorta and the pulmonary artery. The diversity of AAOCA might present as a relatively normal course under non-invasive radiological imaging scanning.
冠状动脉异常起源（AAOCA）被认为是幼儿心脏性猝死（SCD）的主要原因。因此，及时识别AAOCA以避免致命事件至关重要。最近，已发现越来越多的右冠状动脉或左冠状动脉起源于主动脉窦不适当位置的病例。在此，我们报告一例罕见的AAOCA病例，该病例在影像学筛查中表现为动脉壁内走行，看似正常迁移，患者有晕厥症状。
一名7岁男性，既往无心血管和脑血管疾病史，剧烈运动后突然出现剧烈胸痛和晕厥。心电图显示多个导联ST段压低超过0.05 mV，生物标志物提示严重心肌损伤。左心室射血分数（LVEF）急剧降至23%。因此排除了暴发性心肌炎和心肌病。然而，超声心动图和心脏CT血管造影（CTA）显示冠状动脉起源相对正常，起自左冠状窦。很难将严重的临床表现与超声心动图和CTA上的轻微畸形联系起来。此外，选择性冠状动脉造影显示，异常的左冠状动脉起源于不适当窦的上缘，走行于动脉壁内，最终从主动脉左窦穿出并在主动脉和肺动脉之间迁移，这在运动时导致了严重心肌梗死。随后，患者接受了改良的去顶术进行手术矫正。经过2个月的强化治疗，患者出院，随访18个月无症状。
AAOCA，尤其是异常左冠状动脉（ALCA），是SCD的主要潜在风险。我们报告了ALCA的一种非典型表现，其起源于不适当的主动脉窦，汇入动脉壁内形成奇特走行，然后在主动脉和肺动脉之间穿出。AAOCA的多样性在非侵入性放射学成像扫描下可能表现为相对正常的走行。
