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。 Objective To analyze the clinical features and diagnosis and treatment of left coronary artery originated from pulmonary artery (ALCAPA) in all age groups and to improve the understanding of this disease, reduce the misdiagnosis and improve the treatment rate. Methods From April 1999 to March 2011, 37 children with ALCAPA were admitted to our hospital. There were 21 males and 16 females with a mean age of (5.0 ± 4.8) years. Among them, there were 8 infants (≤1 years old), 10 infants (> 1 ~ 3 years old) and 19 cases of large age group (> 3 years old). There were 31 cases of mitral regurgitation, 2 cases of aneurysm, tetralogy of Fallot / ASD, severe pulmonary stenosis / partial pulmonary venous drainage and 1 case of ASD. Retrospective analysis of the clinical features and diagnosis and treatment results of all age groups. Follow-up results based on outpatient review results and telephone follow-up. Results There were 13 cases (35.1%, 13/37) of Q wave and ST-T changes in ECG in infants and young children group, including 5 infants (62.5%) and 3 infants (30.0% ), 5 cases (26.3%) in the big age group, there was no significant difference among the three groups. The chest X-ray showed the heart was significantly increased, the heart chest than the infant group is larger than the infant group and the older age group, The left ventricular diastolic diameter (LVED) was significantly increased in all 3 groups and the most obvious in infants group. The left ventricular ejection fraction (LVEF) <50% in infants group was higher than that in infancy group and older group No significant difference with the older age group. In the first diagnosis, misdiagnosis or missed diagnosis of 18 cases, accounting for 48.6% (18/37 cases). Infant group 1 case due to radionuclide scan showed little myocardial viability, waiting for a heart transplant, the other two cases of parents to give up surgery. Twenty-four children underwent coronary artery grafting in 23 cases, pulmonary artery tunneling in 8 cases, coronary artery bypass grafting in 2 cases, simple ligation of the left coronary artery in 1 case. No surgery early death. There were 26 cases (76.5%, 26/34 cases) with short-term follow-up data. The follow-up ranged from 6 to 130 (13.6 ± 18.4) months, with 1 case of late death. LVED in children with surgery than before surgery to reduce, there are still mild mitral regurgitation in 8 cases. There were follow-up data in children, 8 cases of preoperative LVEF <50%, 3 to 6 months after LVEF returned to normal. Conclusion The clinical manifestations of children with ALCAPA are heavier than those of children and elders. Pediatric ALCAPA due to the lack of characteristic performance, all ages are easily misdiagnosed. For children of all ages showed heart enlargement, cardiac insufficiency, mitral regurgitation and other children, pay attention to children except ALCAPA. Can be combined with its ECG performance, repeated ultrasound ECG exploration coronary artery, if necessary, plus multi-slice spiral CT or angiography to confirm the diagnosis, diagnosis and surgery should be as soon as possible.