左冠状动脉起源于肺动脉的临床分析

Anomalous origin of the left coronary artery from the pulmonary artery: report on 10 cases

摘要:

目的：左冠状动脉起源于肺动脉（anomalous origin of the left coronary artery from the pulmomary artery, ALCAPA)是临床上一种少见的先天性冠状动脉异常性疾病，目前国内报道的资料不多。该研究对其临床特点与治疗进行探讨。方法：对10例诊断为左冠状动脉起源于肺动脉患儿的临床表现、心电图特征、心脏超声、心导管资料、手术治疗及转归进行分析。结果：患儿都有不同程度的多汗、烦躁、气促、拒食等心绞痛及心功能不全的表现；心电图、彩色超声心动图及心导管检查有较特征性的改变；4例患儿行肺动脉内隧道术，4例行直接左冠状动脉移植术，2例行左冠状动脉重建术。术后8例症状改善，2例死亡。结论：掌握ALCAPA的病变特点和相关的诊断线索，辅以彩色超声心动图和心导管检查，可提高ALCAPA的检出率，并早期手术治疗。［中国当代儿科杂志，2007，9（1）：25-27］

Abstract:

OBJECTIVE: The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery abnormality. This study aimed to investigate the clinical characteristics, diagnosis and treatment of the disorder. METHODS: The medical data, including common clinical presentations, the findings of the digital subtraction angiogram (DSA), echocardiograms and the electrocardiograph (ECG), surgical records and the outcome, of 10 children with ALCAPA between June 2001 and February 2005 were retrospectively reviewed. RESULTS: Diaphoresis, fantod, breathlessness and difficult feeding were commonly presented in the patients with ALCAPA. ECG, echocardiography and DSA examinations showed characteristic changes in these patients. The Takeuchi procedure was performed in 4 patients, direct aortic reimplantation in 4 cases and aortic reconstruction in 2 cases. After operation the symptoms of 8 patients were improved but 2 died. CONCLUSIONS: ALCAPA patients have characteristic clinical manifestations and lab findings. This is helpful in making an early diagnosis of ALCAPA. Early surgical treatment can improve the patients' outcome.［Chin J Contemp Pediatr, 2007, 9 (1):25-27］