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儿童左冠状动脉异常起源于右冠状窦致心原性晕厥的临床案例分析

Clinical analysis of children with cardiac syncope caused by anomalous origin of the left coronary artery from the right sinus

摘要:

目的:分析左冠状动脉异常起源于右冠状窦(ALCA-R)致心原性晕厥患儿的临床特点并总结诊治经验。方法:回顾性研究,入选2015年11月至2018年6月在首都医科大学附属北京儿童医院确诊为ALCA-R致心原性晕厥的住院患儿,对其临床表现、心肌损伤标志物、影像学特征及治疗转归进行分析。结果:共纳入4例女童,发病年龄7~14岁。临床症状为运动性晕厥伴急性心肌梗死(4例)、急性左心衰竭(3例)、心跳骤停(1例)。4例患儿心肌酶谱及肌钙蛋白均明显升高;心电图异常均表现为左主干闭塞;超声心动图仅1例提示左冠状动脉异常起源可能;全部经冠状动脉CT血管造影确诊为ALCA-R,左主干走行于主动脉与肺动脉之间并受压狭窄(即主肺动脉间型);2例患儿经心脏核磁共振成像亦证实为主肺动脉间型ALCA-R,并伴有不同程度的心肌延迟强化。2例行冠状动脉去顶手术,2例予保守对症治疗限制活动。随访12~43个月,4例患儿随访期间无心血管事件反复出现,预后良好。结论:对于运动性晕厥伴急性心肌梗死甚至猝死,心电图出现左主干闭塞表现的青少年,应注意冠状动脉的先天发育异常,尤其是ALCA-R应引起儿科医生的重视;一旦确诊应避免剧烈活动,及时手术治疗。

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abstracts:

Objective:To analysis the clinical characteristics and to summarize therapy experience of pediatric patients with cardiac syncope caused by anomalous origin of the left coronary artery from the right sinus (ALCA-R).Methods:We retrospectively analyzed the clinical data including clinical manifestations, myocardial injury biomarkers, radiological features, treatments and prognoses of pediatric patients with ALCA-R who were admitted to Beijing Children′s Hospital from November 2015 to June 2018.Results:Four female patients were included in this analysis, age of onset was 7 to 14 years. All the patients presented with exercise-induced syncope and acute myocardial infarction. During the course, three patients presented with acute left heart failure, and one patient had history of sudden cardiac arrest. Laboratory data showed significant elevation of both the creatine kinase and troponin levels in four patients. All electrocardiogram (ECG) showed left main coronary artery occlusion, echocardiography suggested the possible anomalous origin of the left coronary artery in one child. Coronary CT angiography (CTA) revealed there was no coronary ostium in the left coronary sinus, and the left coronary artery had an anomalous origin from the right sinus. The left main coronary artery passed between the ascending artery and the root of the main pulmonary artery, which was compressed by these two large vessels. Two patients underwent cardiac magnetic resonance examination, which detected late gadolinium enhancement in ALCA-R with an interarterial course. Unroofing of the left coronary ostium (cut-back procedure) was performed in two patients, and the other two patients who were not operated were recommended to restrict their physical activities. During a regular follow-up period of 12-43 months, all the children survived without recurrent cardiovascular event.Conclusion:If an adolescent presents with exercise-induced syncope, acute myocardial infarction and even sudden death, and ECG shows left main coronary artery occlusion characteristics, we should consider the possibility of developmental abnormality of coronary artery, particularly the ALCA-R. Once diagnosed as ALCA-R, patients should be recommended to avoid strenuous activities,early recognition and surgical treatment are imperative for these patients.

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期刊: 《中华心血管病杂志》2020年48卷9期 772-776页 MEDLINEISTICPKUCSCDCABP
栏目名称: 临床研究
DOI: 10.3760/cma.j.cn112148-20191015-00631
发布时间: 2020-09-28
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