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单中心10年间冠状动脉起源异常患儿临床分析

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目的 分析不同类型冠状动脉(冠脉)起源异常的临床特点。方法 病例系列研究。根据2013年1月至2023年1月上海交通大学医学院附属上海儿童医学中心收治的确诊冠脉起源异常患儿的各项临床资料,对不同类型冠脉起源异常的疾病情况进行归纳总结。结果 共收治177例冠脉起源异常患儿,其中,左冠脉异常起源于肺动脉(ALCAPA)122例,男54例,女68例,中位年龄1。2岁;右冠脉异常起源于肺动脉(ARCAPA)6例,男3例,女3例,中位年龄4。3岁;左冠脉异常起源于右冠窦(ALCA)9例,男6例,女3例,中位年龄9。5岁;右冠脉异常起源于左冠窦(ARCA)40例,男24例,女16例,中位年龄7。7岁。确诊ALCAPA的患儿多在1岁以内起病,以慢性心力衰竭为主要表现,小年龄患儿常伴有严重的二尖瓣反流;共111例患儿进行手术,11例患儿术后死亡。6例ARCAPA患儿无明显临床症状,确诊后根据双冠脉循环原则行手术治疗。9例ALCA患儿均以运动后晕厥,胸痛或腹痛起病,有8例患儿进行外科手术治疗,其中1例患儿进行心脏移植。40例ARCA患儿中,23例出现临床表现,主要以运动后胸闷,晕厥,胸痛为主诉,术前16例患儿平板运动试验阳性,共13例患儿进行外科手术治疗。结论 不同类型的冠脉起源异常严重程度各有不同,左冠脉起源异常临床表现一般较重,多有心功能不全或猝死风险,一旦确诊,应及时外科治疗;右冠脉起源异常相对较轻,少数可有严重后果,一般遵循个体化或者双冠脉循环原则进行治疗。
Clinical analysis of children with anomalous origin of coronary artery over ten years in a single center
Objective To analyze the clinical characteristics of different types of anomalous origin of the coronary artery.Methods A case-series study was conducted.Based on the clinical data of children diagnosed with anomalous origin of the coronary artery at Shanghai Children's Medical Center,Shanghai Jiao Tong University School of Medicine from January 2013 to January 2023,the diseases of different types of anomalous origin of the coronary artery were summarized.Results A total of 177 children with anomalous origin of the coronary artery were treated.Among them,122 children developed the anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA),including 54 males and 68 females,with a median age of 1.2 years;6 children developed the anomalous origin of the right coronary artery from the pulmonary artery(ARCAPA),including 3 males and 3 females,with a median age of 4.3 years;9 children developed the anomalous left coronary artery from the right coronary sinus(ALCA),including 6 males and 3 females,with a median age of 9.5 years;40 children developed the anomalous right coronary artery from the left coronary sinus(ARCA),including 24 males and 16 females,with a median age of 7.7 years.Most children diagnosed with ALCAPA had onset within 1 year of age,with chronic heart failure as the main manifestation,and young children were often accompanied by severe mitral regurgitation.A total of 111 children underwent surgery,and 11 children died.Six children with ARCAPA had no obvious clinical symptoms and were treated by operation according to the principle of double coronary circulation after diagnosis.Nine children with ALCA started with syncope,chest pain or abdominal pain after exercise.Eight of the children underwent surgical treatment,including 1 who received a heart transplant.Of the 40 children with ARC A,23 children had clinical manifestations,with chest tightness,syncope,and chest pain after exercise as chief complaints;16 children were tested positive for treadmill exercise before surgery;and a total of 13 children received surgical treatment.Conclusions Different types of anomalous origin of the coronary artery vary in severity.The clinical manifestations of the anomalous origin of the left coronary artery are generally serious,and most of such patients have the risk of cardiac insufficiency or sudden death.Once diagnosed,surgical treatment should be performed timely.The clinical manifestations of the anomalous origin of the right coronary artery are relatively mild,and only a few may have serious consequences,which are usually treated according to the principle of individualization or double coronary circulation.

ChildCongenital heart malformationsAnomalous origin of coronary artery

林洁靓、张锰、李奋、傅立军、高伟、刘廷亮、郭颖、钟玉敏、沈捷

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上海交通大学医学院附属上海儿童医学中心心内科,上海 200127

上海交通大学医学院附属上海儿童医学中心放射科,上海 200127

儿童 先天性心脏畸形 冠状动脉起源异常

2024

中华实用儿科临床杂志
中华医学会

中华实用儿科临床杂志

CSTPCD北大核心
影响因子:1.5
ISSN:2095-428X
年,卷(期):2024.39(10)