Left ventricle endomyocardial fibrosis: a case report

Abstract Background Endomyocardial fibrosis is a grim disease. It is the most common restrictive cardiomyopathy worldwide, but the exact etiology and pathogenesis both remain unknown. Endomyocardial fibrosis is recurrently associated with chronic eosinophilia and probable dietary, environmental, and...

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Main Authors: Raquel Reis Soares, Maria Clara Martins Avelar, Sofia Lucena Zanetti, Joao Victor Tavares Mendonça Garreto, Vinicius Dinelli Guimaraes, Elisa Soares Ferber, Mayumi de Oliveira Drumond, Matheus Ferber, Leonardo Ferber
Format: Article
Language:English
Published: BMC 2023-08-01
Series:Journal of Medical Case Reports
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Online Access:https://doi.org/10.1186/s13256-023-04056-z
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author Raquel Reis Soares
Maria Clara Martins Avelar
Sofia Lucena Zanetti
Joao Victor Tavares Mendonça Garreto
Vinicius Dinelli Guimaraes
Elisa Soares Ferber
Mayumi de Oliveira Drumond
Matheus Ferber
Leonardo Ferber
author_facet Raquel Reis Soares
Maria Clara Martins Avelar
Sofia Lucena Zanetti
Joao Victor Tavares Mendonça Garreto
Vinicius Dinelli Guimaraes
Elisa Soares Ferber
Mayumi de Oliveira Drumond
Matheus Ferber
Leonardo Ferber
author_sort Raquel Reis Soares
collection DOAJ
description Abstract Background Endomyocardial fibrosis is a grim disease. It is the most common restrictive cardiomyopathy worldwide, but the exact etiology and pathogenesis both remain unknown. Endomyocardial fibrosis is recurrently associated with chronic eosinophilia and probable dietary, environmental, and infectious factors, which contribute not only to the onset of the disease (an inflammatory process) but also to its progression and maintenance (endomyocardial damage and scar formation). The trademark of the disease is the fibrotic obliteration of the affected ventricle. The combination of such processes produces focal or diffuse endocardial thickening and fibrosis, which leads to restrictive physiology. Endomyocardial fibrosis affects the apices of the right and the left ventricle in around 50% of cases and most often extends to the posterior leaflet of the mitral valve. Sometimes it involves the papillary muscle and chordae tendineae, causing atrioventricular valve dysfunction. The fibrosis does not affect extracardiac organs. This cardiomyopathy is most recurrent in tropical areas of the world. Case presentation A 67-year-old Black male with past medical history of schistosomiasis infection in childhood presented with progressive dyspnea, lower extremity edema, and weakness for 2 years. He was diagnosed with endomyocardial fibrosis. The echocardiogram showed an increased thickness in the septum (17 mm) and free left ventricular wall (15 mm), obliteration of the left ventricular apex and inflow tract, and mitral valve regurgitation. Cardiac magnetic resonance imaging revealed apical left ventricle wall thickening with left ventricular apical obliteration associated with enlargement of the respective atrium. Delayed enhancement imaging showed endomyocardium enhancement involving left ventricular apex, mitral valve regurgitation due to annulus dilation, and a thrombus at left ventricular apex. He underwent open heart surgery with mitral valve replacement, endocardial decortication, endomyocardiectomy, and two-vessel coronary artery bypass grafting as preoperative coronary angiogram showed mild right coronary artery and proximal left anterior descending artery severe lesions. Postoperative course was uncomplicated, and he was discharged successfully from the hospital. Six months after surgery, he was New York Heart Association functional class I. Conclusion The purpose of this case report is to illustrate the aspects of endomyocardial fibrosis by reporting a case of this entity. In conclusion, progress in imaging techniques and treatment in a reference institution for cardiac diseases contribute to earlier diagnosis and survival in patients with endomyocardial fibrosis.
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spelling doaj.art-6ce065fe37334413af279ed57845fdb52023-11-20T09:31:46ZengBMCJournal of Medical Case Reports1752-19472023-08-011711610.1186/s13256-023-04056-zLeft ventricle endomyocardial fibrosis: a case reportRaquel Reis Soares0Maria Clara Martins Avelar1Sofia Lucena Zanetti2Joao Victor Tavares Mendonça Garreto3Vinicius Dinelli Guimaraes4Elisa Soares Ferber5Mayumi de Oliveira Drumond6Matheus Ferber7Leonardo Ferber8Biocor Rede D’Or InstituteFaculdade Ciências Médicas de Minas GeraisFaculdade Ciências Médicas de Minas GeraisBiocor Rede D’Or InstituteBiocor Rede D’Or InstituteUniversidade José do Rosário Vellano – UNIFENASUniversidade Federal dos Vales do Jequitinhonha e MucuriBiocor Rede D’Or InstituteBiocor Rede D’Or InstituteAbstract Background Endomyocardial fibrosis is a grim disease. It is the most common restrictive cardiomyopathy worldwide, but the exact etiology and pathogenesis both remain unknown. Endomyocardial fibrosis is recurrently associated with chronic eosinophilia and probable dietary, environmental, and infectious factors, which contribute not only to the onset of the disease (an inflammatory process) but also to its progression and maintenance (endomyocardial damage and scar formation). The trademark of the disease is the fibrotic obliteration of the affected ventricle. The combination of such processes produces focal or diffuse endocardial thickening and fibrosis, which leads to restrictive physiology. Endomyocardial fibrosis affects the apices of the right and the left ventricle in around 50% of cases and most often extends to the posterior leaflet of the mitral valve. Sometimes it involves the papillary muscle and chordae tendineae, causing atrioventricular valve dysfunction. The fibrosis does not affect extracardiac organs. This cardiomyopathy is most recurrent in tropical areas of the world. Case presentation A 67-year-old Black male with past medical history of schistosomiasis infection in childhood presented with progressive dyspnea, lower extremity edema, and weakness for 2 years. He was diagnosed with endomyocardial fibrosis. The echocardiogram showed an increased thickness in the septum (17 mm) and free left ventricular wall (15 mm), obliteration of the left ventricular apex and inflow tract, and mitral valve regurgitation. Cardiac magnetic resonance imaging revealed apical left ventricle wall thickening with left ventricular apical obliteration associated with enlargement of the respective atrium. Delayed enhancement imaging showed endomyocardium enhancement involving left ventricular apex, mitral valve regurgitation due to annulus dilation, and a thrombus at left ventricular apex. He underwent open heart surgery with mitral valve replacement, endocardial decortication, endomyocardiectomy, and two-vessel coronary artery bypass grafting as preoperative coronary angiogram showed mild right coronary artery and proximal left anterior descending artery severe lesions. Postoperative course was uncomplicated, and he was discharged successfully from the hospital. Six months after surgery, he was New York Heart Association functional class I. Conclusion The purpose of this case report is to illustrate the aspects of endomyocardial fibrosis by reporting a case of this entity. In conclusion, progress in imaging techniques and treatment in a reference institution for cardiac diseases contribute to earlier diagnosis and survival in patients with endomyocardial fibrosis.https://doi.org/10.1186/s13256-023-04056-zRestrictive cardiomyopathyEndomyocardial fibrosisHeart failureCardiac surgeryHypereosinophiliaCardiac magnetic resonance
spellingShingle Raquel Reis Soares
Maria Clara Martins Avelar
Sofia Lucena Zanetti
Joao Victor Tavares Mendonça Garreto
Vinicius Dinelli Guimaraes
Elisa Soares Ferber
Mayumi de Oliveira Drumond
Matheus Ferber
Leonardo Ferber
Left ventricle endomyocardial fibrosis: a case report
Journal of Medical Case Reports
Restrictive cardiomyopathy
Endomyocardial fibrosis
Heart failure
Cardiac surgery
Hypereosinophilia
Cardiac magnetic resonance
title Left ventricle endomyocardial fibrosis: a case report
title_full Left ventricle endomyocardial fibrosis: a case report
title_fullStr Left ventricle endomyocardial fibrosis: a case report
title_full_unstemmed Left ventricle endomyocardial fibrosis: a case report
title_short Left ventricle endomyocardial fibrosis: a case report
title_sort left ventricle endomyocardial fibrosis a case report
topic Restrictive cardiomyopathy
Endomyocardial fibrosis
Heart failure
Cardiac surgery
Hypereosinophilia
Cardiac magnetic resonance
url https://doi.org/10.1186/s13256-023-04056-z
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