A case report of a 40-year-old woman with endomyocardial fibrosis in a non-tropical area: from initial presentation to high urgent heart transplantation

BMC Cardiovascular Disorders

Robert Ullrich¹  Gernot Wagner²  Gerald Gartlehner²  Ludwig Müller³  Dominik Wiedemann⁴  Andreas Zuckermann⁴  Markus Haumer⁵  Harald Mayr⁶  Andreas Kliegel⁶  Deddo Moertl⁶  Gerhard Poelzl⁷ 

[1] Clinical Institute of Pathology, Medical University Vienna;Department for Evidence-based Medicine and Evaluation, Danube University Krems;Department of Cardiac Surgery, Medical University Innsbruck;Department of Cardiac Surgery, Medical University Vienna;Department of Internal Medicine 2, Landesklinikum Wiener Neustadt;Department of Internal Medicine 3, University Hospital St. Poelten, Karl Landsteiner University of Health Sciences;Department of Internal Medicine III, Clinical Division of Cardiology & Angiology, Innsbruck Medical University;

关键词: Restrictive cardiomyopathy;    Endomyocardial fibrosis;    Heart failure;    Eosinophilia;   

DOI  :  10.1186/s12872-019-1243-8

来源: DOAJ

【 摘 要 】

Abstract 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.

【 授权许可】

Unknown