总结先天性冠状动脉瘘合并巨大冠状动脉瘤患儿的超声特点及其在诊断及随访中的应用价值。

回顾性选取首都医科大学附属北京儿童医院2009年4月至2025年4月经超声心动图诊断的127例累及单侧冠状动脉、瘘入单心腔的先天性冠状动脉瘘患儿为研究对象，根据是否合并巨大冠状动脉瘤将患儿分为巨大冠状动脉瘤组与非巨大冠状动脉瘤组。采用单因素分析对巨大冠状动脉瘤组与非巨大冠状动脉瘤组的基线资料进行组间比较，应用多因素Logistic回归分析形成巨大冠状动脉瘤的相关因素。并对巨大冠状动脉瘤组手术患儿进行术后超声随访，动态观察手术效果。

127例患儿中巨大冠状动脉瘤组49例（49/127，38.6%），右冠状动脉瘘23例（23/49，46.9%），瘘口位于右心室28例（28/49，57.1%）、右心房17例（17/49，34.7%）、左心室3例（3/49，6.1%）、左心房1例（1/49，2.0%）；14例（14/49，28.6%）患儿合并其他心脏畸形；其中16例患儿进行冠状动脉瘘封堵术或外科手术治疗（16/49，32.7%）。非巨大冠状动脉瘤组78例（78/127，61.4%）；右冠状动脉瘘21例（21/78，26.9%），瘘口位于右心室51例（51/78，65.4%）、左心室12例（12/78，15.4%）、右心房10例（10/78，12.8%）、左心房5例（5/78，6.4%）；29例（29/78，37.2%）患儿合并其他心脏畸形；本组患儿均未进行手术治疗。49例巨大冠状动脉瘤的漏误诊率约12.2%（6/49）。Logistic回归分析结果显示，右冠状动脉瘘（OR：0.362，95%CI：0.162~0.810）、瘘口位于心房（OR：3.166，95%CI：1.320~7.594）是形成巨大冠状动脉瘤的独立危险因素。冠状动脉瘘合并巨大冠状动脉瘤超声心动图主要表现为病变冠状动脉呈巨大瘤样扩张；对侧冠状动脉未见明显扩张。16例手术患儿矫治效果良好，随访中均未发现冠状动脉狭窄等严重并发症。

右冠状动脉瘘、冠状动脉心房瘘可能是形成巨大冠状动脉瘤的独立危险因素。冠状动脉瘘合并巨大冠状动脉瘤具有典型的超声心动图表现，掌握其超声表现可减少漏误诊的发生。儿童手术干预短期获益虽不显著，但与成人相比，可能降低严重并发症的发生风险。

To summarize the echocardiographic characteristics of congenital coronary artery fistula (CAF) complicated with giant coronary artery aneurysm in children and assess its utility in diagnosis and follow-up.

A retrospective study was conducted on 127 children with congenital CAF involving a single coronary artery and draining into a single cardiac chamber, who were diagnosed by echocardiography at Beijing Children's Hospital Affiliated to Capital Medical University from April 2009 to April 2025. According to the presence of giant coronary artery aneurysm or not, they were divided into a giant coronary artery aneurysm group and a non-giant coronary artery aneurysm group. Univariate analysis was used to compare baseline data between the two groups, and multivariate Logistic regression analysis was performed to identify factors associated with the development of giant coronary artery aneurysm. Postoperative echocardiographic follow-up was performed for children in the giant coronary artery aneurysm group who underwent surgery to dynamically observe the surgical outcomes.

Among the 127 children included, 49 (49/127, 38.6%) were in the giant coronary artery aneurysm group, including 23 cases (23/49, 46.9%) of right CAF. The drainage sites were the right ventricle in 28 cases (28/49, 57.1%), right atrium in 17 (17/49, 34.7%), left ventricle in 3 (3/49, 6.1%), and left atrium in 1 (1/49, 2.0%). Fourteen children (14/49, 28.6%) had other cardiac malformations. Sixteen children (16/49, 32.7%) underwent transcatheter closure or surgical treatment. The non-giant coronary artery aneurysm group consisted of 78 children (78/127, 61.4%), including 21 cases (21/78, 26.9%) of right CAF. The drainage sites were the right ventricle in 51 cases (51/78, 65.4%), left ventricle in 12 (12/78, 15.4%), right atrium in 10 (10/78, 12.8%), and left atrium in 5 (5/78, 6.4%). Twenty-nine children (29/78, 37.2%) had other cardiac malformations. None of the children in this group underwent surgical treatment. The rate of missed diagnosis or misdiagnosis in the 49 children with giant coronary artery aneurysm was approximately 12.2% (6/49). Logistic regression analysis showed that right CAF (odds ratio [OR]=0.362, 95% confidence interval [CI]: 0.162-0.810) and fistula draining into the atrium (OR=3.166, 95%CI: 1.320-7.594) were independent risk factors for the formation of giant coronary artery aneurysm. Echocardiographic findings of CAF complicated with giant coronary artery aneurysm mainly included giant aneurysmal dilation of the affected coronary artery, with no significant dilation of the contralateral coronary artery. All 16 surgically treated children showed good corrective outcomes, and no severe complications such as coronary artery stenosis were found during follow-up.

Right CAF and CAF draining into the atrium may be independent risk factors for the formation of giant coronary artery aneurysm. CAF complicated with giant coronary artery aneurysm has typical echocardiographic manifestations, and familiarity with these imaging features can reduce missed and misdiagnosis. Although the short-term benefits of surgical intervention in children are not very significant, compared with adults, it may reduce the risk of serious complications.