Li Q R, Zhen Z, Na J, Gao L, Cao Y L, Yuan Y
Department of Cardiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Department of Radiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Zhonghua Er Ke Za Zhi. 2020 Feb 2;58(2):113-117. doi: 10.3760/cma.j.issn.0578-1310.2020.02.009.
To investigate the clinical features and improve the diagnosis and treatment of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course (ALCA-R-IAC) between the ascending aorta and main pulmonary artery in children. A retrospective analysis of the clinical manifestation, laboratory test, radiological feature, treatment and prognosis were conducted in four female children presented with ALCA-R-IAC in Beijing Children's Hospital from November 2015 to June 2018. The four girls with onset age of 7.5-14.7 years were diagnosed with ALCA-R-IAC by CT coronary angiography (CTCA). Four children presented with exercise-induced syncope and clinical manifestations of acute myocardial infarction including 3 patients with acute left heart failure, 1 cardiogenic shock and 1 cardiac arrest. Nervous system involvement was found in one patient. Troponin I increased significantly to 20.65-50.00 μg/L in the four patients. Electrocardiogram (ECG) developed signs of left main coronary artery involvement. Echocardiography revealed reduced left ventricular ejection fraction (LVEF) of 25%-45% in three children and suspected anomalous origin of the left coronary artery in one child. CTCA showed an anomalous left coronary artery originating from the right coronary sinus, which had an interarterial course between the aorta and pulmonary artery leading to a slim left main coronary trunk. Two children underwent unroofing procedure and the other two children in whom physical activities were restricted received conservative managements. During a regular follow-up period of 12-43 months, all the children survived without recurrent symptoms and had good prognosis. ALCA-R-IAC can present as exercise-related syncope and acute myocardial infarction, even sudden death in children and adolescents. CTCA is helpful to clarify the early diagnosis of ALCA-R-IAC. Surgical intervention is the main treatment for ALCA-R-IAC and strenuous physical activities should be avoided.
探讨儿童升主动脉与主肺动脉之间走行于动脉间的右冠状动脉窦起源的左冠状动脉异常（ALCA-R-IAC）的临床特征，以提高其诊断和治疗水平。对2015年11月至2018年6月在北京儿童医院就诊的4例患ALCA-R-IAC的女童的临床表现、实验室检查、影像学特征、治疗及预后进行回顾性分析。这4名发病年龄为7.5 - 14.7岁的女童通过CT冠状动脉造影（CTCA）确诊为ALCA-R-IAC。4例患儿均有运动诱发的晕厥及急性心肌梗死的临床表现，其中3例出现急性左心衰竭，1例发生心源性休克，1例心脏骤停。1例患儿有神经系统受累表现。4例患儿肌钙蛋白I显著升高至20.65 - 50.00μg/L。心电图（ECG）出现左主干冠状动脉受累征象。超声心动图显示3例患儿左心室射血分数（LVEF）降低至25% - 45%，1例患儿怀疑左冠状动脉起源异常。CTCA显示左冠状动脉异常起源于右冠状动脉窦，走行于主动脉和肺动脉之间，导致左主干冠状动脉变细。2例患儿接受了开窗手术，另外2例限制体力活动的患儿接受了保守治疗。在12 - 43个月的定期随访期间，所有患儿均存活，无复发症状，预后良好。ALCA-R-IAC在儿童和青少年中可表现为与运动相关的晕厥和急性心肌梗死，甚至猝死。CTCA有助于早期诊断ALCA-R-IAC。手术干预是ALCA-R-IAC的主要治疗方法，应避免剧烈体力活动。
