Chiu Hsin-Hui, Wang Jou-Kou, Chen Chun-An, Chiu Sheunn-Nan, Lin Ming-Tai, Lue Hung-Chi, Chang Chung-I, Chiu Ing-Su, Wu Mei-Hwan
Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
Eur J Pediatr. 2008 Nov;167(11):1277-82. doi: 10.1007/s00431-008-0667-4. Epub 2008 Mar 4.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.
左冠状动脉起源于肺动脉(ALCAPA)是一种罕见的先天性心脏缺陷,通常在婴儿期表现为扩张型心肌病。1984年至2005年,在我们研究所的9950例先天性心脏病患者中,有13例(5例男性和8例女性,占0.13%)被确诊为ALCAPA。矫正手术的中位年龄为9个月(范围:2个月至5岁)。11例患者接受了左冠状动脉(LCA)直接移植到主动脉的手术,2例接受了肺外分流术。总体生存率为92%。只有1例患者在LCA移植术后5个月因急性心肌缺血死亡。婴儿期接受手术的8例(62%)患者的左心室射血分数(LVEF)明显低于5个月后接受手术的患者(中位值:35%对75%)。11例患者(85%)的左心室功能恢复正常。在8例未同时进行二尖瓣环成形术的患者中,6例患者的二尖瓣反流(MR)改善至轻度或微量程度,2例患者的二尖瓣反流仍维持在术前水平。11例患者出现病理性Q波,除2例患者外,所有患者的病理性Q波最终均消退。MR、LVEF和心电图(ECG)变化的恢复中位间隔时间分别为16天、6个月和24个月。总之,ALCAPA在亚洲国家如台湾地区也是一种罕见疾病。无论修复时的年龄如何,双冠状动脉修复术后MR、左心室(LV)功能甚至病理性Q波均可预期随后恢复。