BACKGROUND Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Diagnosis is based on patient history, heart failure symptoms, and echocardiographic signs of restrictive ventricular filling, atrioventricular valve regurgitation and frequently apical thrombus. Following is a case report of an Austrian patient with EMF who eventually had to undergo a heart transplant. This case report strives to promote awareness for this in non-tropical areas uncommon but nevertheless detrimental disease. CASE PRESENTATION A 40-year-old woman was presented at our emergency department with chest pain and fever up to 38.1° Celsius. Plasma troponin-T levels and inflammatory markers were slightly elevated, but the echocardiogram was without pathological findings. The patient was hospitalized on the suspicion of acute myocarditis and discharged soon after improvement. Eight months later, she was presented again with chest pain and symptoms of heart failure. The echocardiogram showed normal systolic left ventricular (LV) function with LV wall thickening and severe restrictive mitral regurgitation as well as aortic and tricuspid regurgitation. Coronary angiogram was normal but right heart catheterization showed pulmonary hypertension due to left heart disease. Further diagnostic workup with cardiac magnetic resonance imaging revealed subendocardial late enhancement and apical thrombus formation in the left ventricle compatible with the diagnosis of EMF. A comprehensive diagnostic workup showed no evidence of infection, systemic immunologic or hematological disease, in particular hypereosinophilic syndrome. After a multidisciplinary consideration of several therapeutic options, the patient was listed for heart transplantation. On the waiting list, she deteriorated rapidly due to progressive heart failure and finally underwent a heart transplantation. Histological examination confirmed the diagnosis of EMF. Six years after her heart transplantation, the patient was presented in an excellent clinical condition. CONCLUSIONS Even in non-tropical regions, the diagnosis of EMF should always be considered in restrictive cardiomyopathy. Knowledge of the distinct phenotype of EMF facilitates diagnosis, but comprehensive workup and therapeutic management remain challenging and require a multidisciplinary approach.

中文翻译：

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非热带地区一名40岁女性患有心内膜纤维化的病例报告：从最初就诊到紧急心脏移植。

背景技术心肌内膜纤维化（EMF）代表全世界限制性心肌病的最常见原因。尽管在热带地区患病率很高，但偶尔在从未到过这些地区的患者中也会发生这种情况。尽管研究已经提出了各种可能的EMF诱因，但病因学和发病机理仍是未知之数。诊断基于患者的病史，心力衰竭症状以及心室受限的超声心动图征象，房室瓣关闭不全和经常的心尖血栓。以下是一名奥地利EMF患者的病例报告，该患者最终不得不进行心脏移植。该病例报告致力于在非热带地区但不常见但有害的疾病中提高人们对此的认识。病例介绍一名40岁的妇女在我们的急诊室就诊，出现胸痛和发烧，最高摄氏38.1度。血浆肌钙蛋白-T水平和炎性标志物略有升高，但超声心动图无病理发现。该患者因怀疑患有急性心肌炎而入院，病情好转后即出院。八个月后，她再次出现胸痛和心力衰竭症状。超声心动图显示收缩期左心室（LV）功能正常，左室壁增厚，严重的二尖瓣反流性限制以及主动脉和三尖瓣反流。冠状动脉造影正常，但右心导管检查显示由于左心疾病导致肺动脉高压。心脏磁共振成像的进一步诊断检查显示，心内膜下的晚期增强和左心室的心尖血栓形成与EMF的诊断兼容。全面的诊断检查未发现感染，全身免疫或血液系统疾病，特别是嗜酸性粒细胞增多综合征的证据。在对多种治疗选择进行多学科研究后，该患者被列为进行心脏移植。在候补名单上，她由于进行性心力衰竭而迅速恶化，最后接受了心脏移植。组织学检查证实了EMF的诊断。心脏移植六年后，该患者的临床状况良好。结论即使在非热带地区，在限制性心肌病中应始终考虑EMF的诊断。对EMF独特表型的了解有助于诊断，但是全面的检查和治疗管理仍具有挑战性，需要多学科的方法。