Cağli Kumral, Uygur Belma, Ozlü Fatih, Gölbaşi Zehra
Department of Cardiology, Türkiye Yüksek Ihtisas Training and Research Hospital, Ankara, Turkey.
Turk Kardiyol Dern Ars. 2009 Mar;37(2):136-40.
Endomyocardial disease is a form of restrictive cardiomyopathy, of unknown etiology, which occurs most commonly in tropical and subtropical areas. It is characterized by the formation of endomyocardial fibrosis of the apical and subvalvular regions of one or both ventricles. A 29-year-old male patient was admitted with restrictive cardiomyopathy and decompensated heart failure. Telecardiography showed cardiomegaly and right pleural effusion. Transthoracic echocardiography revealed preserved left ventricular systolic functions, biatrial dilatation, apical obliteration of both ventricles, increased endocardial echoreflectivity, and pericardial effusion. The right ventricular outflow tract was dilated. There was no endocardial thickening in this region. Doppler examination showed grade 3 mitral and tricuspid regurgitation. Ventriculograms showed apical obliteration of both ventricles, marked decrease in the size of the right ventricular cavity, significant dilatation of the right ventricular outflow tract and both atria, and severe mitral and tricuspid regurgitation. Laboratory findings showed no hypereosinophilia. Hepatic congestion, splenomegaly, and ascites were noted on abdominal ultrasonography. Following cardiac catheterization, the patient was placed on the waiting list for cardiac transplantation.
心内膜疾病是一种病因不明的限制性心肌病，最常见于热带和亚热带地区。其特征是一个或两个心室的心尖和瓣膜下区域形成心内膜纤维化。一名29岁男性患者因限制性心肌病和失代偿性心力衰竭入院。心电图显示心脏扩大和右侧胸腔积液。经胸超声心动图显示左心室收缩功能正常、双房扩大、双心室心尖闭塞、心内膜回声增强以及心包积液。右心室流出道扩张。该区域无心内膜增厚。多普勒检查显示二尖瓣和三尖瓣反流3级。心室造影显示双心室心尖闭塞、右心室腔大小明显减小、右心室流出道和双房显著扩张以及严重的二尖瓣和三尖瓣反流。实验室检查结果显示无嗜酸性粒细胞增多。腹部超声检查发现肝淤血、脾肿大和腹水。心脏导管检查后，该患者被列入心脏移植等待名单。
