Hashimoto T, Yano K, Matsumoto Y, Hashiba K
Third Department of Internal Medicine, Nagasaki University School of Medicine, Japan.
Jpn Heart J. 1988 Jan;29(1):121-6. doi: 10.1536/ihj.29.121.
A case of a 24-year-old man with the contracted form of primary endocardial fibroelastosis diagnosed by left ventricular endomyocardial biopsy showing a markedly thickened endocardium with fibroelastic proliferation is reported. He had no evident symptoms of congestive heart failure except for shortness of breath on moderate exertion. Echocardiogram showed thickened and dense echoes from the left side of the septum and from the posterior left ventricular endocardium. Hemodynamic and angiographic studies revealed marked elevation of right and left ventricular end-diastolic pressures with dip and plateau pressure contours, moderate pulmonary hypertension, left atrial enlargement and mild mitral regurgitation. Further elevation of right and left ventricular diastolic pressures and pulmonary artery pressure was observed at the second evaluation after 5 years. Our patient suggests that primary endocardial fibroelastosis should be included in the differential diagnosis of adult patients with obscure types of cardiac disease.
报告了一例24岁男性原发性心内膜弹力纤维增生症收缩型病例，经左心室心内膜活检诊断，显示心内膜明显增厚伴纤维弹性增生。除中度运动时气短外，他无明显充血性心力衰竭症状。超声心动图显示室间隔左侧和左心室后壁心内膜增厚且回声致密。血流动力学和血管造影研究显示，左右心室舒张末期压力显著升高，呈下陷-高原压力曲线，中度肺动脉高压，左心房扩大和轻度二尖瓣反流。5年后第二次评估时，观察到左右心室舒张压和肺动脉压进一步升高。我们的患者提示，原发性心内膜弹力纤维增生症应纳入成年隐匿性心脏病患者的鉴别诊断。
