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      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

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          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.

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          Most cited references 24

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          Cardiovascular manifestations of hypereosinophilic syndromes.

          The hypereosinophilic syndromes (HESs) are characterized by persistent marked eosinophilia (>1500 eosinophils/mm(3)), the absence of a primary cause of eosinophilia (such as parasitic or allergic disease), and evidence of eosinophil-mediated end organ damage. Cardiovascular complications of HES are a major source of morbidity and mortality in these disorders. The most characteristic cardiovascular abnormality in HES is endomyocardial fibrosis. Patients who have an HES also may develop thrombosis, particularly in the cardiac ventricles, but also occasionally in deep veins. Because of the rarity of these disorders, specific guidelines for the management of the cardiac and thrombotic complications of HES are lacking. This article reviews the diagnosis and management of the cardiovascular manifestations of HES.
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            A population study of endomyocardial fibrosis in a rural area of Mozambique.

            Endomyocardial fibrosis is the most common restrictive cardiomyopathy worldwide. It has no specific treatment and carries a poor prognosis, since most patients present with advanced heart failure. On the basis of clinical series, regional variations in distribution have been reported within several countries in Africa, Asia, and South America, but large-scale data are lacking on the epidemiologic features and early stages of the disease. We used transthoracic echocardiography to determine the prevalence of endomyocardial fibrosis in a rural area of Mozambique. We screened a random sample of 1063 subjects of all age groups selected by clustering. Major and minor diagnostic criteria were defined, and a severity score was developed and applied. Cases were classified according to the distribution and severity of the lesions in the heart. The estimated overall prevalence of endomyocardial fibrosis was 19.8%, or 211 of 1063 subjects (95% confidence interval [CI], 17.4 to 22.2). The prevalence was highest among persons 10 to 19 years of age (28.1%, or 73 of 260 subjects [95% CI, 22.6 to 33.6]) and was higher among male than among female subjects (23.0% vs. 17.5%, P=0.03). The most common form was biventricular endomyocardial fibrosis (a prevalence of 55.5%, or 117 of 211 subjects [95% CI, 48.8 to 62.2]), followed by right-sided endomyocardial fibrosis (a prevalence of 28.0%, or 59 of 211 subjects [95% CI, 21.9 to 34.1]). Most affected subjects had mild-to-moderate structural and functional echocardiographic abnormalities. Only 48 persons with endomyocardial fibrosis (22.7%) were symptomatic. The frequency of familial occurrence was high. Endomyocardial fibrosis is common in a rural area of Mozambique. By using echocardiography, we were able to detect early, asymptomatic stages of the disease. These findings may aid in the study of the pathogenesis of the disease and in the development of new management strategies. 2008 Massachusetts Medical Society
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              Endomyocardial Fibrosis: Still a Mystery after 60 Years

              The pathologist Jack N. P. Davies identified endomyocardial fibrosis in Uganda in 1947. Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America. In Kampala, the disease accounts for 20% of heart disease patients referred for echocardiography. We conducted a systematic review of research on the epidemiology and etiology of endomyocardial fibrosis. We relied primarily on articles in the MEDLINE database with either “endomyocardial fibrosis” or “endomyocardial sclerosis” in the title. The volume of publications on endomyocardial fibrosis has declined since the 1980s. Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.
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                Author and article information

                Contributors
                gernot.wagner@donau-uni.ac.at
                markus.haumer@wienerneustadt.lknoe.at
                gerhard.poelzl@tirol-kliniken.at
                dominik.wiedemann@meduniwien.ac.at
                andreas.kliegel@stpoelten.lknoe.at
                robert.ullrich@meduniwien.ac.at
                gerald.gartlehner@donau-uni.ac.at
                andreas.zuckermann@meduniwien.ac.at
                ludwig.mueller@tirol-kliniken.at
                harald.mayr@stpoelten.lknoe.at
                deddo.moertl@stpoelten.lknoe.at
                Journal
                BMC Cardiovasc Disord
                BMC Cardiovasc Disord
                BMC Cardiovascular Disorders
                BioMed Central (London )
                1471-2261
                19 December 2019
                19 December 2019
                2019
                : 19
                Affiliations
                [1 ]GRID grid.15462.34, ISNI 0000 0001 2108 5830, Department for Evidence-based Medicine and Evaluation, , Danube University Krems, ; Dr. Karl Dorrek Strasse 30, 3500 Krems, Austria
                [2 ]Department of Internal Medicine 2, Landesklinikum Wiener Neustadt, Corvinusring 3-5, 2700 Wiener Neustadt, Austria
                [3 ]GRID grid.5361.1, ISNI 0000 0000 8853 2677, Department of Internal Medicine III, Clinical Division of Cardiology & Angiology, , Innsbruck Medical University, ; Anichstrasse 35, 6020 Innsbruck, Austria
                [4 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Department of Cardiac Surgery, , Medical University Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria
                [5 ]GRID grid.459693.4, Department of Internal Medicine 3, University Hospital St. Poelten, , Karl Landsteiner University of Health Sciences, ; Dunantplatz 1, 3100 St. Poelten, Austria
                [6 ]GRID grid.487248.5, Institute for Research of Ischaemic Cardiac Disease and Rhythmology, , Karl Landsteiner Society, ; Propst-Fuehrer-Strasse 4, 3100 St. Poelten, Austria
                [7 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Clinical Institute of Pathology, , Medical University Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria
                [8 ]GRID grid.62562.35, ISNI 0000000100301493, RTI International, ; 3040 East Cornwallis Road, PO Box 12194, Research Triangle Park, North Carolina 27709-2194 USA
                [9 ]GRID grid.5361.1, ISNI 0000 0000 8853 2677, Department of Cardiac Surgery, , Medical University Innsbruck, ; Anichstrasse 35, 6020 Innsbruck, Austria
                Article
                1243
                10.1186/s12872-019-1243-8
                6933894
                31881943
                © The Author(s). 2019

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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