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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Le résumé fourni par la source
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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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- 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
- Date Crossref
- 01/12/2019
- Éditeur
- Springer Science and Business Media LLC
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Universität für Weiterbildung Krems pays non établi dans la noticeUniversité ou école supérieure
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Fachhochschule Wiener Neustadt pays non établi dans la noticeUniversité ou école supérieure
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Innsbruck Medical University Department of Internal Medicine III pays non établi dans la noticeUniversité ou école supérieure
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Medical University of Vienna pays non établi dans la noticeUniversité ou école supérieure
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Karl Landsteiner University of Health Sciences pays non établi dans la noticeUniversité ou école supérieure
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Karl Landsteiner Society pays non établi dans la noticeInstitution
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RTI International pays non établi dans la noticeOrganisation à but non lucratif
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Danube University Krems Department for Evidence-based Medicine and Evaluation pays non établi dans la noticeUniversité ou école supérieure
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Department of Internal Medicine 2 pays non établi dans la noticeInstitution
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Medical University Vienna Department of Cardiac Surgery pays non établi dans la noticeUniversité ou école supérieure
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University Hospital St. Poelten Department of Internal Medicine 3 pays non établi dans la noticeUniversité ou école supérieure
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Institute for Research of Ischaemic Cardiac Disease and Rhythmology pays non établi dans la noticeStructure de recherche
Universität für Weiterbildung Krems, Fachhochschule Wiener Neustadt et Department of Internal Medicine III — Innsbruck Medical University, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.