心肌致密化不全合并弥漫性冠状动脉右室瘘1例

发布时间：2019-02-22 10:53

【摘要】：目的探讨心肌致密化不全合并弥漫性冠状动脉瘘的临床特点及诊断方法。方法报告1例心肌致密化不全合并弥漫性冠状动脉右室瘘患儿,回顾其临床症状、辅助检查结果和治疗方案。在国外数据库(Pubmed、OVID和Elsevier)和国内数据库(万方和维普)中检索心肌致密化不全合并冠状动脉瘘和心肌病合并冠状动脉瘘病例的文献,检索时间为建库至2017年3月30日。总结此类病例的临床表现、诊断和治疗。结果本文患儿为6月龄女婴,因"胃纳差伴盗汗2周"于2013年10月12日在上海交通大学医学院附属上海儿童医学中心心内科就诊。二维超声心动图显示,左心室增大伴左室致密化不全、左右冠状动脉扩张、右心室内心肌窦状间隙持续状态、二尖瓣中度返流、心室收缩功能低下等。心导管造影及冠状动脉造影检查显示,右冠状动脉及左前降支弥漫性微小右心室瘘、二尖瓣返流、左心室增大。予地高辛、速尿、安体舒通后患儿症状稍好转。国外数据库中检索到2例心肌致密化不全合并冠状动脉左室瘘的成年病例,以进行性呼吸困难和心前区疼痛为主要临床表现,心肌缺血表现更明显,诊断主要依靠二维超声心动图及心导管造影和冠状动脉造影检查,缺乏有效治疗手段;有部分心肌病合并心肌致密化不全患者接受选择性冠状动脉瘘介入封堵术的尝试。结论心肌致密化不全合并冠状动脉瘘是一类罕见的先天性心脏发育异常疾病,婴儿临床症状主要为纳差、盗汗等心功能不全的表现,确诊主要依靠二维超声心动图以及心导管造影、冠脉造影检查,目前尚缺乏有效治疗手段,以强心、利尿、扩血管改善心功能治疗为主。
[Abstract]:Objective to investigate the clinical features and diagnostic methods of myocardial compaction insufficiency complicated with diffuse coronary artery fistula. Methods A case of myocardial compaction insufficiency complicated with diffuse coronary artery right ventricular fistula (RVF) was reported. The clinical symptoms, adjuvant examination and treatment were reviewed. The literatures of myocardial compaction insufficiency complicated with coronary artery fistula and cardiomyopathy complicated with coronary artery fistula were searched in foreign databases (Pubmed,OVID and Elsevier) and domestic databases (Wanfang and Wiper). The retrieval time was until March 30, 2017. To summarize the clinical manifestations, diagnosis and treatment of these cases. Results the child was a 6-month-old female infant who was admitted to the Department of Cardiology of Shanghai Children's Medical Center affiliated to Shanghai Jiaotong University Medical College on October 12, 2013 because of "dysphagia with night sweat for 2 weeks". Two-dimensional echocardiography showed that left ventricular enlargement accompanied by left ventricular compaction insufficiency left and right coronary artery dilatation the continuous state of right ventricular sinus space mitral valve moderate regurgitation ventricular systolic function and so on. Cardiac catheterization and coronary angiography showed diffuse right ventricular fistula, mitral regurgitation and left ventricular enlargement in right coronary artery and left anterior descending branch. After giving digoxin, furosemide, acetonolactone, children's symptoms improved slightly. Two adult patients with myocardial compaction insufficiency associated with coronary artery left ventricular fistula were found in foreign databases. The main clinical manifestations were progressive dyspnea and pain in the precordial area, especially in myocardial ischemia. The diagnosis mainly depends on two dimensional echocardiography, cardiac catheterization and coronary arteriography, lack of effective treatment. Some patients with cardiomyopathy complicated with myocardial compaction insufficiency underwent selective coronary artery fistula closure. Conclusion Myocardial compaction insufficiency combined with coronary artery fistula is a kind of rare congenital cardiac dysplasia. The main clinical symptoms of infants are anorexia, night sweating and other cardiac dysfunction. The diagnosis mainly depends on two dimensional echocardiography, cardiac catheterization and coronary angiography. At present, there is still a lack of effective treatment, mainly to strengthen the heart, diuresis, vasodilatation to improve cardiac function.
【作者单位】：上海交通大学医学院附属上海儿童医学中心;
【分类号】：R726.5

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