儿童孤立性冠状动脉瘘的治疗及中期随访分析

目的对儿童孤立性冠状动脉瘘的治疗过程及中期随访结果进行总结分析。方法回顾性分析广州市妇女儿童医疗中心2009年7月至2017年7月诊断为孤立性冠状动脉瘘的17例患儿临床资料,其中男11例,女6例,年龄40日龄至12岁(中位值23个月),体重3.8~29 kg(中位体重11.3 kg)。2例存在气促表现,2例存在生长发育落后表现,2例存在反复呼吸道感染表现,1例主诉心悸,余10例无明显临床症状。其中3例心脏检查未发现明显杂音,14例发现心脏杂音。瘘口发生于右冠状动脉8例,左冠状动脉9例。瘘入右心房5例,右心室10例,左心室2例;17例中12例合并巨大冠状动脉瘤。结果1例行经皮冠状动脉瘘封堵术,1例在非体外循环下行瘘管结扎术,其余15例在体外循环下行冠状动脉瘘矫治术;平均住院时间(11.1±4.1)d。其中2例术后3 d内出现射血分数下降(最低者降至38%),术后1个月复查时射血分数均升至50%以上。7例术后出现一过性T波改变,出院时均已恢复正常;2例术后存在1~2 mm残余瘘的患儿在近期复查中残余瘘消失。围术期无一例死亡。随访10个月至9年,所有患儿无自觉症状和阳性体征,心电图均无心肌缺血表现,复查超声心动图均提示心脏收缩功能正常,冠状动脉直径均较术前缩小。结论大多数儿童孤立性冠状动脉瘘无明显症状,但可合并巨大冠状动脉瘤,应尽早手术,中远期预后良好;但因存在冠状动脉扩张,仍需长期随访。

Therapies and mid-term follow-up of children with isolated coronary artery fistula

Objective To explore the therapies and mid-term follow-ups of children with isolated coronary artery fistula(CAF).Methods From July 2009 to July 2017,the clinical data were retrospectively analyzed for 17 children of isolated CAF.There were 11 boys and 6 girls with a median age of 23 months(14 days to 12 years)and a median body weight of 11.3(3.8-29)kg.There were tachypnea(n=2),growth retardation(n=2)and recurrent respiratory infection(n=2).One child developed palpitation while another 10 cases remained asymptomatic.Heart murmurs existed in 14 cases.Fistulas originated from right coronary artery(n=8)and left coronary artery(n=9).Fistulas drained into right atrium(n=5),right ventricle(n=10)and left ventricle(n=2).Twelve patients were complicated by giant coronary artery aneurysms.Results The procedures included percutaneous CAF closure(n=1),fistula ligation without cardiopulmonary bypass(CPB)(n=1)and fistula correction with beating heart CPB(n=15).The mean hospitalization stay was(11.1±4.1)days.Two patients had a lower ejection fraction with a bottom of 38%within 3 days post-operation and returned to over 50%at 1 month.Transient T wave change occurred in 7 patients.A residual shunt with a size of 1 to 2 millimeters through fistula vanished recently in 2 cases.No perioperative mortality occurred.During a follow-up period of(10-108)months,all cases survived asymptomatically without any positive physical sign.Electrocardiogram showed no signs of myocardial ischemia while transthoracic echocardiography indicated normal cardiac function.The diameter of coronary arteries was smaller than preoperative counterparts.Conclusion Children with isolated CAF are mostly asymptomatic.However,its complication of giant coronary artery aneurysm requires early interventions for decent mid-term outcomes.However,long-term or lifelong follow-up is still essential due to dilated coronary artery.