scholarly journals Endomyocardial fibrosis: A restrictive cardiomyopathy in developing countries

Author(s):  
Juan F. Vasquez-Rodriguez ◽  
Ramón Medina-Mur ◽  
Luis E. Giraldo ◽  
Marietta L. Juan-Guardela ◽  
Julián Gelves ◽  
...  
2019 ◽  
Vol 12 (4) ◽  
pp. e227642 ◽  
Author(s):  
Pramod Kumar ◽  
Sheragaru Hanumanthappa Chandrashekhara ◽  
Sanjeev Kumar ◽  
Amarinder Singh Malhi

Loeffler endocarditis is an uncommon restrictive cardiomyopathy associated with eosinophilia and endomyocardial fibrosis causing diastolic restriction, predominantly involving the right ventricle. Cardiac MRI plays a crucial role in early detection of disease. Early disease usually responds well to corticosteroids. We describe a case of Loeffler endocarditis with isolated left ventricular involvement on MRI in a young male having hypereosinophilia.


Burden of cardiovascular disease in less-developed countries 654Infectious disease and the heart 656Diphtheria 657HIV and the cardiovascular system 658Chagas’ disease and the heart 660Cardiovascular syphilis 662Beri-beri (thiamine deficiency) 664Tetanus 666Keshan disease 667Restrictive cardiomyopathy 667Endomyocardial fibrosis ...


2020 ◽  
Vol 4 (3) ◽  
pp. 1-5
Author(s):  
Guillaume Théry ◽  
Laurent Faroux ◽  
Philippe Deleuze ◽  
Damien Metz

Abstract Background Endomyocardial fibrosis (EMF) is a rare cause of restrictive cardiomyopathy, mainly found in tropical/subtropical country. Endomyocardial fibrosis causes severe congestive symptoms and may lead to end-stage heart failure. Case summary A French Caucasian 44-year-old man without noticeable medical history and who had never travelled outside of France was hospitalized for a first episode of acute heart failure revealing an atypical appearance of the left ventricle. Cardiac magnetic resonance (CMR) identified EMF, but investigations did not identify any aetiology (no eosinophilia). Despite optimal management of chronic heart failure, functional class declined rapidly resulting in several hospitalizations for heart failure. The patient finally underwent an elective heart transplantation with good results at 6-month follow-up. Discussion Endomyocardial fibrosis exact physiopathology remains unclear, although association with eosinophilia has been reported. Diagnosis is challenging and is based on multi-modal imagery with a central role of CMR. There is no consensus on optimal management, medical therapy having poor outcomes and rate of peri-operative complications being high. Heart transplantation should be considered for eligible patients.


2011 ◽  
Vol 44 (5) ◽  
pp. 644-645 ◽  
Author(s):  
Renata de Carvalho Bicalho Carneiro ◽  
Alexandre Lemos da Silveira Santos ◽  
Luisa Campos Caldeira Brant ◽  
Fábio Tôrres Rabelo ◽  
Carla Maia Ligeiro ◽  
...  

Endomyocardial fibrosis (EMF) is a neglected tropical disease that affects millions of people worldwide. EMF is the most common cause of restrictive cardiomyopathy, caused by deposition of fibrous tissue on endocardial surfaces. EMF is a major cause of death in areas where it is endemic, but the pathogenesis of the disease is poorly understood. Schistosomiasis mansoni is a parasitic disease endemic in Brazil, where EMF has also been described. The association between EMF and schistosomiasis has been suggested in various publications, seeking a possible correlation between endocardial and periportal fibroses. This report describes a case of EMF associated with schistosomiasis.


2019 ◽  
Vol 71 (1) ◽  
Author(s):  
Mahmoud Abdelnabi ◽  
Abdallah Almaghraby ◽  
Yehia Saleh ◽  
Sherif Abd Elsamad ◽  
And Sara Elfawal

Abstract Background Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy that is diagnosed mainly in children and young adults and is geographically found in Africa, Latin America, and Asia. It is a condition with high morbidity and mortality, unknown etiology, and no definitive treatment. Although its main clinical presentation is congestive heart failure with or without related supraventricular arrhythmia like atrial fibrillation, it very rarely presents with ventricular arrhythmias and tachycardias (VA, VT). Case presentation We report a case of right ventricular (RV) EMF presented with recurrent attacks of hemodynamically unstable VT that required direct current (DC) cardioversion. The diagnosis was suspected by transthoracic echocardiography (TTE) and established by cardiac magnetic resonance (CMR). The patient underwent implantable cardioverter–defibrillator (ICD) implantation for secondary prevention of VT, and he was discharged safely on antiarrhythmic drugs with regular follow-up visits. Conclusion EMF presenting with VT are quite rare and to the best of our knowledge, our case is the fourth case in the literature to report VT as a clinical presentation of EMF.


2017 ◽  
Vol 10 ◽  
pp. 117954761772364 ◽  
Author(s):  
Amit Alam ◽  
Shankar Thampi ◽  
Shahryar G Saba ◽  
Rita Jermyn

Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis. The severe progression of the disease required surgical intervention with endocardial stripping to treat the right-sided diastolic heart failure.


Author(s):  
Ajay Kumar Sharma ◽  
Tarun Kumar ◽  
S. Ramakrishnan

Background: Endomyocardial fibrosis has varied presentatation and difficult to diagnose. Aim to elucidate the role of Cardiac Magnetic Resonance (CMR) imaging in the evaluation of Endomyocardial Fibrosis (EMF) and to devise diagnostic criteria for the disease.Methods: Retrospective analysis of cases of restrictive cardiomyopathy referred for Magnetic resonance imaging over a period of 5 years. All patients underwent 1.5 T CMR imaging (Magnetom Avanto, Siemens, Germany) with standard cardiomyopathy protocol. Criteria for diagnosis of RCM included normal sized ventricles, normal/reduced systolic function, uni-/bi-atrial enlargement, normal pericardium and absent septal bounce. Cases diagnosed as EMF on CMR were included in this study. Statistical analysis performed using SPSS software.Results: EMF was diagnosed in 20 patients (31%) [12 males; age 39±18 years]. Ten patients had Right Centricular (RV) EMF, 3 had Left Ventricular (LV) EMF, while 7 had bi-ventricular EMF. Oedema indicating ongoing inflammation was seen in 4 (20%) cases. Apical thrombus was seen in 8(40%) cases and was present in 35% cases of RV and 20% cases of LV involvement. Subendocardial delayed enhancement was always present in the involved ventricle. The RV apex was obliterated in 100% of patients with RV EMF, while LV apex was similarly obliterated in 66% cases with LV disease. Mild-moderate pericardial effusion was observed in 8 patients. On the basis of CMR findings, the disease was classified as early necrotic phase in 1, thrombotic necrotic in 4 and late fibrotic phase in 13 and of different stages in ventricles in 2 cases.Conclusions: EMF was the commonest cause of RCM in this series. Major diagnostic criteria of EMF on CMR include subendocardial delayed enhancement and apical obliteration. Oedema and thrombus are variable findings, depending on disease severity.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Gernot Wagner ◽  
Markus Haumer ◽  
Gerhard Poelzl ◽  
Dominik Wiedemann ◽  
Andreas Kliegel ◽  
...  

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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