左冠状动脉起源于肺动脉6例患儿的诊断与治疗

Diagnosis and Treatment of 6 Children with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

目的分析左冠状动脉起源于肺动脉(ALCAPA)的诊断特点,评估其手术效果。方法本院2006年3月-2007年10月共收治ALCAPA患儿6例。男4例,女2例;年龄6个月～10岁,平均43.5个月。对这6例患儿的临床表现、心电图(ECG)特征、心脏超声、64排螺旋CT及心导管检查资料、手术治疗及转归进行分析。结果ALCAPA5例患儿有不同程度的多汗、烦躁、气促、拒食等临床表现,临床检查示心功能不全。ECG特异表现:4例患儿ECG存在前侧壁异常Q波及ST段改变,1例患儿存在ST段改变,1例患儿ECG大致正常。典型彩色超声心动图表现为:4例患儿出现左室扩大伴左室收缩功能减低;2例患儿存在二尖瓣轻-中量返流,2例患儿为大量二尖瓣返流;2例患儿表现为右冠状动脉扩张。增强CT及心导管检查有特征性改变。其中5例患儿行手术治疗:2例直接移植,3例行左冠状动脉重建术;2例重度二尖瓣返流患儿同时行二尖瓣成形术。1例无症状患儿未行手术治疗。术后患儿症状改善,无死亡病例。结论了解ALCAPA的病变特点及相关辅助检查,可提高ALCAPA的检出率,尽早手术治疗,改善预后。

Objective To analyze diagnostic experience of chihtren with anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） and assess its surgical results. Methods Six children with ALCAPA had been diagnosed in our hospital from Mar. 2006 to Oct. 2007 aged from 6 months to 10 years old（ median 43.5 months）. The medical data included common clinical presentations,the findings of electrocardiogram （ECG） , the echocardiogram, ECG- gated 64 -slice computed tomography, the digital subtraction angiogram （DSA） , surgical records and the outcome. Results Diaphoresis,fantod,breathlessness and difficult feeding were commonly presented in 5 eases ALCAPA and the 5 children presented with left ventricular failure in infancy. The predominant ECG findings were Q waves and ST segment changes in the anterolateral chest leads in 4 cases,ST changes in 1 case,and normal ECG in 1 case. Cross - sectional eehocardiography showed dilated left ventricles with poor contractility in 4 cases,medium mitral regurgitation in 2 cases and severe mitral regurgitation in 2 cases, aneurysmal dilatation of the right coronary, artery.（RCA） in 2 cases. CT and DSA examinations showed characteristic changes in these children. The surgical reestablishment of a two - coronary system was performed in 5 cases, direct reimplantation in 2 cases and creation of an autologous extrapulmonary tunnel in 3 cases. Simultaneous mitral annuloplasty was performed in 2 eases. After operation,the symptoms of 5 cases were all improved and none died. Conclusions Understanding the clinical manifestations and lab findings of ALCAPA is helpful to making an early diagnosis of ALCAPA. Early surgical treatment can improve the patients＇ outcome.