Zheng Jianyong, Ding Wenhong, Xiao Yanyan, Jin Mei, Zhang Guizhen, Cheng Pei, Han Ling
Department of Pediatric Cardiology and Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, No. 2 Anzhen Road, Beijing 100029, People's Republic of China.
Pediatr Cardiol. 2011 Jan;32(1):24-31. doi: 10.1007/s00246-010-9798-2. Epub 2010 Oct 26.
This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdiagnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.
本研究旨在阐述治疗左冠状动脉起源于肺动脉(ALCAPA)患儿的经验。回顾了1993年8月至2009年6月在北京安贞医院收治的19例ALCAPA患儿的临床资料。根据资料,47.4%(9/19)的患者曾被误诊为心内膜弹力纤维增生症,15.8%(3/19)曾被误诊为扩张型心肌病。心电图显示18例患者在I、avL及V4-V6导联出现异常Q波伴T波倒置,尤其是在avL导联。16例患者中有15例近端右冠状动脉直径与主动脉根部直径之比超过0.20。超声心动图检查时经常发现心尖部室壁瘤或瘤样扩张(52.6%,10/19)、乳头肌回声增强(84.2%,16/19)以及冠状动脉侧支增多(78.9%,15/19)。共有18例患者接受了心脏手术,其中1例(5.6%)行左冠状动脉(LCA)结扎术,1例(5.6%)行LCA结扎加冠状动脉旁路移植术,7例(38.9%)行竹内手术,9例(50.0%)行LCA再植术。5例患者在医院死亡,其余患者在6至166个月的随访期内无症状。他们的异常Q波逐渐消退,随着二尖瓣反流的减轻,左心室收缩功能和大小恢复正常。ALCAPA的临床特征有助于做出准确诊断。这种畸形可通过几种手术成功治疗,预后良好。