儿童冠状动脉疾病32例临床分析

摘要

目的 探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平.方法 回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿(不包括复杂心脏畸形合并冠状动脉异常者)的临床资料,分析临床表现、辅助检查、诊断方法和治疗.结果 32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁.经ECG、超声心动图、64排CT和心导管造影检查明确诊断.左冠状动脉异常起源于肺动脉10例(31.2%),8例以心力衰竭起病,其中长期误诊为"心内膜弹力纤维增生症"4例,"扩张性心肌病"1例,"二尖瓣重度关闭不全"2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例(40.6%),9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例(18.8%),出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例(9.4%),表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重.结论 儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键.%Objective To study the etiological spectrum and clinical features of coronary artery disease in children. Methods The clinical manifestations, laboratory examinations, diagnosis and treatment of 32 children with coronary artery disease ( not including complex cardiac malformation associated with coronary artery abnormalities ) from January 2008 to December 2011 were retrospectively reviewed. Results Thirty-two children were enrolled in this study including 20 males and 12 females aged from 1 month to 18 years. The diagnosis was established on the basis of ECG,echocardiography, 64-slice CT or angiography. Anomalous origin of left coronary artery from pulmonary artery with heart failure was found in 10 cases( 4 cases misdiagnosed as endocardial fibroelastosis, 1 case as dilated cardiomyopathy, 2 cases as severe mitral regurgitation ) and needed immediate surgical treatment for left coronary artery reimplantation; Coronary artery fistula was found in 13 cases, onset with heart murmur ( 9 cases ) and heart failure ( 4 cases ), 12 cases underwent surgery for ligation or pericardial patch of the fistula, and 1 case was given transcatheter closure. Kawasaki disease( KD ) with coronary artery aneurysm was found in 6 cases, the patients appeared as left ventricular enlargement and myocardial ischemia with long-term anticoagulation treatment.Familial hypereholesterolemia was iound in 3 cases with manifestations of coronary atherosclerotic heart disease, the patients were treated by pharmaceutic drugs for lowing lipids and improving myocardial ischemia as well as diet therapy. Conclusions Anomalies of the coronary arteries in children are rare.Congenital coronary anomalies include anomalous origin of left coronary artery from pulmonary artery and coronary artery fistula. The most common causes of acquired abnormalities of the coronary vessels in children are KD and familial hypercholestero-lemia. The clinical background of children with left ventricular enlargement and/or myocardial ischemia indicates the possibility of suffering from the diseases above. Early diagnosis and therapeutic strategy may be important for improving the prognosis.