儿童先天性冠状动脉瘘的外科治疗及随访研究

The surgical outcome and follow-up of congenital coronary artery fistula in children

目的总结儿童先天性冠状动脉瘘的治疗效果及随访结果，探讨其治疗方法。方法回顾性分析2008年7月至2017年1月22例外科手术治疗的冠状动脉瘘患儿临床资料，男14例，女8例；年龄14天～12岁（中位年龄17个月），体质量3．0-31．0kg（中位值8．8kg）。结果全组临床表现为气促2例，生长发育落后3例，17例无症状。体检发现心脏杂音20例。瘘口起源于右冠状动脉11例，左冠状动脉10例，双侧冠状动脉1例。瘘人右心室13例，右心房6例，左心室2例，冠状静脉窦1例，多发瘘口2例。患儿病变冠状动脉均明显扩张，直径4．0-12．0mm，其中8例呈瘤样扩张。非体外循环下行瘘管结扎术患儿2例，体外循环心脏停搏下行冠状动脉瘘矫治术20例：3例直接缝闭瘘的人口；11例直接缝闭出口；6例同时关闭出、人口，其中2例采用心包补片连续缝合出口。7例合并其他心内畸形者同时矫治。术中未处理瘤样扩张的冠状动脉。患儿住院（12．3±3．2）天。2例术后3天内出现心功能下降，术后1个月复查恢复正常。2例术后出现1～2mm残余瘘。全组无死亡。随访3个月～8年，患儿均无明显症状，未规律服用抗凝药物，复查超声心动图提示心功能正常，术后6个月复查，冠状动脉扩张较术前减轻，冠状动脉内无血栓形成，心电图未提示心肌缺血。结论儿童先天性冠状动脉瘘分流量大可出现临床症状，外科手术治疗效果确切，同时可纠正合并的心内畸形，效果良好。

Objective Analyze the surgical and follow-up outcomes of congenital coronary artery fistula （CAF） in chil- dren and to discuss the treatment methods. Methods The clinical data were analyzed retrospectively in 22 patients who were diagnosed as congenital CAF and received surgical treatment between July 2008 and January 2017 in Guangzhou Women and Children＇ s Medical Center. There were 14 boys and 8 gifts. The median age was 17 months ranging from 14 days to 12 years old. The median weight was 8.8 kilograms ranging from 3.0 to 31.0 kilograms. Results Two patients had shortness of breath. Three had growth retardation with recurrent respiratory infection or tachypnea. The remaining 17 patients were asympto- matic. Twenty had heart murmurs. Fistulas originated from right coronary artery accounted for 11 patients, with 10 from left coronary artery, 1 from both right and left coronary arteries. Fistulas drained into coronary sinus in 1 patient, right atrium in 6 patients, right ventricular in 13 patients and left ventricular in 2 patients. Single fistula occurred in 20 patients and multiple fis- tulas in 2 patients. The coronary arteries were obviously dilated in all patients with diameter from 4.0 to 12.0 millimeters. There were 8 cases with aneurysmal dilation of coronary arteries. Two patients underwent fistula ligation without cardiopulmo- nary bypass（CPB）. The remaining 20 cases received fistula correction with beating heart CPB. Direct suture was used in 3 pa- tients for closure of fistula inlet and 11 children for outlet. Both inlet and outlet were closed in 6 patients, and in 2 of them au- tologous pericardial patch was used for outlet. Other associated intra-eardiac anomalies in 7 children were corrected during the operation. The aneurysmal coronary arteries remained original shape without any intervention during the operation. The mean hospital delay was（ 12.3 ± 3.2） days. Two patients appeared decreased heart function within 3 days after the operation, and recovered in return visit one month later. Another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure. During the perioperative period and the follow-up period（3 months to 8 years）, all 22 patients were asymptomafically alive without regular anticoagulation management. Transthoracic echocardio- graphy showed normal cardiac function. Compared with preoperative status, the diameter of dilated coronary arteries was re-duced over 6 months follow up after the operation. There were no formation of thrombus in the coronary arteries. Electrocardio- graphy showed no ST-T changes or arrhythmia or myocardial ischemia. Conclusion Clinical symptoms can be appeared in children with congenital CAF due to large shunt. Surgical correction is an effective method for the management of single CAF or CAF with coexisted intra-cardiac anomalies. The outcome was well.