左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。

Clinical Characteristics, Diagnosis and Treatment of Children with Anomalous Origin of Left Coronary Artery from Pulmonary Artery in Each Age Group

Objective To investigate the clinical features,diagnosis and treatment of children with anomalous origin of left coronary artery from pulmonary artery(ALCAPA) in each age group and improve the knowledge and decrease the misdiagnosis of this disorder. Methods Between Apr.1999 and Mar.2011,37 patients21 male and 16 female,aged(5.0±4.8) years] were examined and treated for ALCAPA in Fuwai Hospital.They were divided into 3 groups:included 8 infants(≤1 year),10 toddlers(>1-3 years)and 19 older children(>3 years).Of 37 patients with ALCAPA,31 cases were sick with regurgitation(MR),2 cases with left ventricular aneurysm,1 case with Tetralogy of fallot(TOF)/atrial septal defect(ASD),1 case with severe pulmonary stenosis(PS)/partial anomalous pulmonary venous connection(PAPVC)and 1 case with ASD at the same time.The initial and final diagnosis and surgery result were also reviewed.Follow-ups of some patients were conducted in the out-patient and by recent telephone call and the records were established. Results Infants and toddlers had disorders at an earlier age than the older children.Thirteen of 37 patients(35.1%,13/37 cases),including 5 infants(62.5%),3 toddlers(30.0%) and 5 older children(26.3%) had Q and ST-T segment changes in ECG,but there was no statistical difference among the 3 groups.Chest radiograph in all patients showed marked cardiomegaly,cardiothoracic ratio was biggest in infants,bigger in toddlers compared with the old children.Meanwhile left ventricular end-diastolic dimension(LVED) measured by echocardiography was greatly increased in infants compared with the other 2 groups.There were more infants with LVEF<50% than the other 2 groups and there was no statistical differe-nce between the toddlers and the older children.Eighteen of 37 patients(48.6%,18/37 cases) were misdiagnosed at their initial visit to the pediatricians.Thirty-four patients underwent operation and 3 others did not.Among them,1 infant had profoundly depressed ventricular function and very little viable myocardium and had to wait for heart transplantation.Twenty-three of 34 patients had direct aortic reimplantation,8 cases had Takeuchi procedure,1 case had ligation of the main trunks of the left anterior descending coronary artery(LAD) and 2 cases had coronary artery bypass grafting(CABG).There were no operative deaths.Followed-up for 6 to 130 months average(13.6±18.4) months] were conducted in 26 patients(76.5%,26/34 cases),but 1 patient with ligation of the main trunks of LAD had a sudden death after 8 months of surgery.All patients had gotten smaller LVED after surgery and 8 patients with LVEF<50% recovered from low LVEF after 3 months to 6 months of surgery.Only 8 patients had mild MR. Conclusions The clinical features of the patients with ALCAPA are more serious in infants than in toddlers and older children.Without typical symptoms and signs,ALCAPA could be misdiagnosed in any age.The young infant and children who are brought to cardiologist with dilated left ventricular cardiomyopathy and MR must undergo exhaustive exclusion of ALCAPA.Very often there is classic ECG evidence of left ventricular ischemia and infarction.Echocardiography should be done repeatedly for accurate diagnosis.If necessary,MDCT and cardiac catheterization should be performed to confirm the diagnosis.Surgery should be performed earlier based on clear diagnosis.