Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
Chin Med J (Engl). 2010 Oct;123(20):2888-94.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart anomaly. We aimed to illustrate the clinical features and long-term prognosis of patients with ALCAPA.
Twenty three patients (13 males and 10 females, ages ranging from 2.5 months to 65 years) identified as ALCAPA in Beijing Anzhen Hospital from April 1984 to June 2009 were divided into two groups, based on the age of onset: group 1 (≤ 12 months, n = 16) and group 2 (> 12 months, n = 7).
Fifty six point three percent of patients in group 1 had been misdiagnosed as endocardial fibroelastosis (9/16), 18.8% as dilated cardiomyopathy (3/16) and 6.3% as myocardial infarction (1/16). Patients in group 2 were usually diagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. Electrocardiography in group 1 revealed abnormal Q waves with T wave inversion in leads I, avL, V(4)-V(6), especially in lead avL (deep and wide Q wave); but no specific manifestations in group 2. A higher percentage of patients in group 1 had cardiomegaly on chest radiograph (86.7% vs. 33.3%, P = 0.031), while pulmonary artery protrusion was more common in group 2 (26.7% vs. 83.3%, P = 0.046). Lower left ventricular ejection fraction (LVEF) was present in group 1 than in group 2 ((48.5 ± 11.5)% vs. (65.0 ± 6.1)%, P < 0.001). Apical ventricular aneurysm (62.5% vs. 0%, P = 0.007), enhanced echogenicity of papillary muscles (87.5% vs. 28.6%, P = 0.011) and endocardial thickening (93.8% vs. 14.3%, P < 0.001) were more frequent in group 1 than in group 2. The ratio of the proximal right coronary artery (RCA) diameter to the aortic root diameter exceeded 0.14 in all cases, more prominent in group 2 (0.26 ± 0.05 vs. 0.33 ± 0.03, P = 0.009). Increased coronary artery collaterals within the interventricular septum were detected in 18 patients (78.3%) by Doppler imaging. Twenty one patients underwent cardiac surgery, including left coronary artery (LCA) ligation (1/21), LCA ligation plus coronary artery bypass grafting (1/21), Takeuchi operation (7/21), and LCA reimplantation surgery (12/21). Four patients underwent concomitant mitral valve repair and one received mitral valve replacement. Aneurysm resection was performed in 3 cases. Six patients died in hospital after surgery, and the rest of the cohort had no overt symptoms during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed or disappeared, and the LVEF and left ventricle size returned to normal range with alleviation of mitral insufficiency.
The accurate diagnosis of ALCAPA can be made with serial diagnostic methods. ALCAPA can be successfully treated with several types of cardiac surgery, and surgeries of establishing two-coronary-artery circulation are the preferred operations nowadays, with good long-term prognosis.
左冠状动脉异常起源于肺动脉（ALCAPA）是一种罕见的先天性心脏病。我们旨在阐明 ALCAPA 患者的临床特征和长期预后。
2009 年 6 月前，23 例（男 13 例，女 10 例，年龄 2.5 个月至 65 岁）在首都医科大学附属北京安贞医院被诊断为 ALCAPA 的患者，根据发病年龄分为两组：组 1（≤ 12 个月，n = 16）和组 2（> 12 个月，n = 7）。
组 1 中 56.3%的患者被误诊为心内膜弹力纤维增生症（9/16）、18.8%为扩张型心肌病（3/16）和 6.3%为心肌梗死（1/16）。组 2 患者通常被诊断为冠心病、心肌炎或动脉导管未闭。组 1 的心电图显示 I、avL、V(4)-V(6)导联异常 Q 波伴 T 波倒置，尤其在 avL 导联（深而宽的 Q 波）；但组 2 无特异性表现。组 1 胸部 X 线片中心脏增大的发生率较高（86.7%比 33.3%，P = 0.031），而组 2 肺动脉突出更常见（26.7%比 83.3%，P = 0.046）。组 1 的左心室射血分数（LVEF）较组 2 低（（48.5 ± 11.5）%比（65.0 ± 6.1）%，P < 0.001）。组 1 中，心尖室壁瘤（62.5%比 0%，P = 0.007）、乳头肌回声增强（87.5%比 28.6%，P = 0.011）和心内膜增厚（93.8%比 14.3%，P < 0.001）较组 2 更常见。所有患者的右冠状动脉（RCA）近端直径与主动脉根部直径之比均超过 0.14，组 2 更为明显（0.26 ± 0.05 比 0.33 ± 0.03，P = 0.009）。多普勒成像显示 18 例（78.3%）患者室间隔内有冠状动脉侧支增加。21 例患者接受了心脏手术，包括左冠状动脉（LCA）结扎（1/21）、LCA 结扎加冠状动脉旁路移植术（1/21）、Takeuchi 手术（7/21）和 LCA 再植入术（12/21）。4 例患者同时行二尖瓣修复术，1 例患者行二尖瓣置换术。3 例患者行动脉瘤切除术。术后 6 例患者死亡，其余患者在 6 至 166 个月的随访期间无明显症状。他们的异常 Q 波逐渐消退或消失，LVEF 和左心室大小恢复正常，二尖瓣关闭不全减轻。
通过连续的诊断方法可以做出准确的 ALCAPA 诊断。ALCAPA 可以通过几种类型的心脏手术成功治疗，目前建立双冠状动脉循环的手术是首选手术，具有良好的长期预后。
