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一名年轻成人严重左心室心内膜心肌纤维化表现为双心室衰竭:病例报告

Severe Left Ventricular Endomyocardial Fibrosis Presenting as Biventricular Failure in a Young Adult: A Case Report.

作者信息

Sandhu Harpreet Singh, Mahendrakar Sampathkumar Mahadevappa, Pethani Rajebali Ramzanali, Khan Azizullah Hafizullah, Loya Yunus Shafi

机构信息

FNB Critcal Care Medicine Trainee, Department of Intensive Care Medicine, Prince Aly Khan Hospital , Nesbit Road, Mazgaon, Mumbai, Maharashtra, India .

Intensivist, Department of Intensive Care, Prince Aly Khan Hospital , Nesbit Road, Mazgaon, Mumbai, Maharashtra, India .

出版信息

J Clin Diagn Res. 2016 Nov;10(11):OD05-OD06. doi: 10.7860/JCDR/2016/22367.8820. Epub 2016 Nov 1.

DOI:10.7860/JCDR/2016/22367.8820
PMID:28050425
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5198378/
Abstract

Endomyocardial Fibrosis (EMF) is a form of progressive restrictive cardiomyopathy of unclear aetiology prevalent in areas within 15 of equator including coastal areas of Kerala a few decades back. It inflicts young adults and carries a poor prognosis due to limited options for treatment. Fortunately, the incidence of cases is now declining due to improvement in health and hygiene standards. Here, we review the aetiology and pathogenesis of EMF and report a case of a young male from Mumbai (non-endemic area) presenting with progressively worsening breathlessness and signs of heart failure unresponsive to conventional medical treatment. To delineate the extent of the disease transthoracic echocardiography and cardiac Magnetic Resonance Imaging (MRI) was done which revealed infiltrative lesions in left ventricular apex with grade 2/3 mitral regurgitation. Due to progressive and severe nature of the disease the patient was managed conservatively. Through this report we would like to rekindle the interest of reader in a forgotten tropical disease which is considered rare in this geographical area but should not be missed as a cause heart failure considering its significant mortality.

摘要

心内膜心肌纤维化(EMF)是一种病因不明的进行性限制性心肌病,几十年前在赤道以南15度范围内的地区流行,包括喀拉拉邦沿海地区。它侵袭年轻人,由于治疗选择有限,预后较差。幸运的是,由于健康和卫生标准的改善,病例发病率目前正在下降。在此,我们回顾了EMF的病因和发病机制,并报告了一例来自孟买(非流行地区)的年轻男性病例,该患者出现进行性加重的呼吸困难和心力衰竭体征,对传统药物治疗无反应。为了明确疾病的范围,进行了经胸超声心动图和心脏磁共振成像(MRI)检查,结果显示左心室心尖部有浸润性病变,伴有2/3级二尖瓣反流。由于疾病的进行性和严重性,对该患者采取了保守治疗。通过本报告,我们希望重新唤起读者对一种被遗忘的热带疾病的关注,这种疾病在该地理区域被认为罕见,但考虑到其显著的死亡率,作为心力衰竭的一个病因不应被忽视。

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本文引用的文献

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Eur J Heart Fail. 2013 Feb;15(2):166-75. doi: 10.1093/eurjhf/hfs206.
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Tropical endomyocardial fibrosis in India: a vanishing disease!印度热带心内膜心肌纤维化:一种正在消失的疾病!
Indian J Med Res. 2012 Nov;136(5):729-38.
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Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium.心内膜心肌纤维化:手术切除的心室心内膜的病理及分子学发现
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