發(fā)布時間：2019-02-22 10:53

【摘要】：目的探討心肌致密化不全合并彌漫性冠狀動脈瘺的臨床特點及診斷方法。方法報告1例心肌致密化不全合并彌漫性冠狀動脈右室瘺患兒,回顧其臨床癥狀、輔助檢查結(jié)果和治療方案。在國外數(shù)據(jù)庫(Pubmed、OVID和Elsevier)和國內(nèi)數(shù)據(jù)庫(萬方和維普)中檢索心肌致密化不全合并冠狀動脈瘺和心肌病合并冠狀動脈瘺病例的文獻,檢索時間為建庫至2017年3月30日�？偨Y(jié)此類病例的臨床表現(xiàn)、診斷和治療。結(jié)果本文患兒為6月齡女嬰,因"胃納差伴盜汗2周"于2013年10月12日在上海交通大學醫(yī)學院附屬上海兒童醫(yī)學中心心內(nèi)科就診。二維超聲心動圖顯示,左心室增大伴左室致密化不全、左右冠狀動脈擴張、右心室內(nèi)心肌竇狀間隙持續(xù)狀態(tài)、二尖瓣中度返流、心室收縮功能低下等。心導管造影及冠狀動脈造影檢查顯示,右冠狀動脈及左前降支彌漫性微小右心室瘺、二尖瓣返流、左心室增大。予地高辛、速尿、安體舒通后患兒癥狀稍好轉(zhuǎn)。國外數(shù)據(jù)庫中檢索到2例心肌致密化不全合并冠狀動脈左室瘺的成年病例,以進行性呼吸困難和心前區(qū)疼痛為主要臨床表現(xiàn),心肌缺血表現(xiàn)更明顯,診斷主要依靠二維超聲心動圖及心導管造影和冠狀動脈造影檢查,缺乏有效治療手段;有部分心肌病合并心肌致密化不全患者接受選擇性冠狀動脈瘺介入封堵術(shù)的嘗試。結(jié)論心肌致密化不全合并冠狀動脈瘺是一類罕見的先天性心臟發(fā)育異常疾病,嬰兒臨床癥狀主要為納差、盜汗等心功能不全的表現(xiàn),確診主要依靠二維超聲心動圖以及心導管造影、冠脈造影檢查,目前尚缺乏有效治療手段,以強心、利尿、擴血管改善心功能治療為主。
[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.
【作者單位】： 上海交通大學醫(yī)學院附屬上海兒童醫(yī)學中心;
【分類號】：R726.5

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