儿童冠状动脉异常主动脉起源69例临床分析

Clinical analysis of 69 cases of anomalous aortic origin of a coronary artery in children

目的分析不同类型及年龄阶段的冠状动脉异常主动脉起源(AAOCA)患儿的临床特点,揭示心肌缺血表现的相关因素。方法纳入首都医科大学附属北京儿童医院2014年1月1日至2022年12月31日经CT冠状动脉成像确诊为AAOCA患儿,分别按AAOCA类型、年龄段、高危解剖结构分组,比较不同异常冠状动脉类型及年龄段患儿的临床特点,分析临床表现严重程度与高危解剖结构的相关性。结果共纳入69例AAOCA患儿,男34例(49.3%),女35例(50.7%),首次发病年龄为(8.89±4.40)岁。其中左侧冠状动脉起源于右冠状窦(ALCA)10例(14.5%)、右侧冠状动脉起源于左冠状窦(ARCA)57例(82.6%)、冠状动脉异常起源于无冠窦2例(2.9%)。19例(27.5%)无自诉临床症状,35例(50.7%)表现为胸闷、胸痛、乏力等轻型症状,13例(18.8%)为晕厥,2例(2.9%)表现为阵发性哭闹。所有患儿经治疗后均临床症状好转出院,无死亡病例。ALCA、ARCA、冠状动脉异常起源于无冠窦3组在性别、临床表现、心肌损伤标志物阳性比例、心电图、经胸超声心动图、高危解剖结构比例上差异均无统计学意义。按年龄段分组,婴幼儿组9例(13.1%)、学龄前组11例(15.9%)、学龄组49例(71.0%),婴幼儿组和学龄前组患儿无症状比例高(P<0.001)。按是否合并高危解剖结构分组,高危组43例(62.3%),包括锐角起始2例、开口狭窄或裂隙样开口4例、动脉间走行37例;非高危组26例(37.7%)。两组比较发现合并高危解剖结构患儿症状重,易发生心源性晕厥(P<0.05)。多因素Logistic回归分析提示存在高危解剖结构为发生心源性晕厥的独立危险因素(OR=9.026,95%CI 1.071~76.084,P=0.043)。结论不同类型AAOCA患儿合并高危解剖结构的比例及临床特征均无明显差异;婴幼儿可因临床症状不典型或不显著而易被漏诊;该疾病的临床症状严重程度与是否合并高危解剖结构密切相关,高危解剖结构为AAOCA患儿发生心源性晕厥的独立危险因素。

Objective To analyze the various clinical presentations of anomalous aortic origin of a coronary artery(AAOCA)and determine factors related to myocardial ischemia.Methods Children diagnosed with AAOCA on CT coronary angiography at Beijing Children′s Hospital,Capital Medical University from 1 January 2014 to 31 December 2022 were classified based on AAOCA type,age and high-risk anatomy.The clinical characteristics of the different AAOCA types and age groups were compared and the correlation between the severity of manifestations and high-risk anatomy was analyzed.Results A total of 69 children with AAOCA[34 males and 35 females,aged(8.89±4.40)years]were included.Ten(14.5%)patients had anomalous origin of the left coronary artery(ALCA)from the right coronary artery sinus and 57(82.6%)patients had anomalous origin of the right coronary artery(ARCA)from the left coronary artery sinus.In two(2.9%)patients,AAOCA did not arise from a coronary sinus.Nineteen(27.5%)patients were asymptomatic,including 35(50.7%)cases presented with minor symptoms(chest tightness,chest pain,fatigue),13(18.8%)cases had severe symptoms(syncope),and two(2.9%)cases had atypical symptoms(paroxysmal crying).All children were successfully treated;no deaths were reported during follow-up.There were no significant differences in gender,clinical manifestations,positive myocardial injury markers,electrocardiogram and transthoracic echocardiography findings,and proportion of children with high-risk anatomy among the different AAOCA groups(ALCA,ARCA,and non-coronary sinus AAOCA).Divided by age,there were nine(13.1%)children in infant group,11(15.9%)in preschool group,and 49(71.0%)in school age group.More infants were asymptomatic than preschoolers(P<0.001).Forty-three(62.3%)patients had a high-risk anatomy:two patients with acute take-off angles,four patients with stenosis or slit-like orifices,and 37 patients with interarterial courses.The remaining patients(37.7%)had non-high-risk anatomies.Children with a high-risk anatomy had severe symptoms and were prone to cardiac syncope(P<0.05).Logistic multivariate analysis suggested that high-risk anatomy was an independent risk factor for cardiogenic syncope(OR=9.026,95%CI 1.071~76.084,P=0.043).Conclusion There are no significant differences in the proportion of high-risk anatomy and clinical characteristics among children with different AAOCA types.Younger children are often misdiagnosed due to atypical or insignificant clinical symptoms.The severity of clinical symptoms is related to the high-risk anatomy.High-risk anatomy is an independent risk factor for cardiogenic syncope in children with AAOCA.