Tropical Endomyocardial Fibrosis.
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ABSTRACT:
SUBMITTER: Sutter JS
PROVIDER: S-EPMC8301721 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Endomyocardial fibrosis is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. Herein, we report the case of a 14-year-old adolescent boy who experienced endomyocardial fibrosis. Upon transthoracic echocardiography, the condition was mistakenly diagnosed as Ebstein's anomaly of the tricuspid valve. Sixteen months after undergoing tricuspid annuloplasty and receiving a bidirectional Glenn shunt, the patient showed no echocardiographic evidence of valvular regurgitation. We discuss imaging and surgical techniques that enable the diagnosis and treatment of endomyocardial fibrosis.
| S-EPMC2696495 | biostudies-literature
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| S-EPMC10133996 | biostudies-literature
Project description: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.
| S-EPMC2254164 | biostudies-literature
Project description:BACKGROUND:Endomyocardial Fibrosis (EMF) is a tropical restrictive cardiomyopathy of unknown etiology with high prevalence in Sub-Saharan Africa, for which it is unclear whether the primary target of injury is the endocardial endothelium, the subendocardial fibroblast, the coronary microcirculation or the myocyte. In an attempt to explore the possibility of endocardial lesions being a result of an immune response against the myocyte we assessed the presence and frequency of circulating anti-myocardial antibodies in EMF patients. METHODOLOGY/PRINCIPAL FINDINGS:EMF classification, assessment of severity and staging was based on echocardiography. We used sodium dodecylsulfate polyacrylamide gel electrophoresis (SDS-PAGE) of myocardial proteins followed by western blotting to screen serum samples for antiheart antibodies G and M classes. The degree of serum reactivity was correlated with the severity and activity of EMF. We studied 56 EMF patients and 10 healthy controls. IgG reactivity against myocardial proteins was stronger and more frequent in patients with EMF when compared to controls (30/56; 53.6% vs. 1/10; 10%, respectively). IgM reactivity was weak in both groups, although higher in EMF patients (11/56; 19.6%) when compared to controls (n = 0). EMF patients showed greater frequency and reactivity of IgG antibodies against myocardial proteins of molecular weights 35 kD, 42 kD and 70 kD (p values <0.01, <0.01 and <0.05 respectively). CONCLUSIONS:The presence of antibodies against myocardial proteins was demonstrated in a subset of EMF patients. These immune markers seem to be related with activity and might provide an adjunct tool for diagnosis and classification of EMF, therefore improving its management by identifying patients who may benefit from immunosuppressive therapy. Further research is needed to clarify the role of autoimmunity in the pathogenesis of EMF.
| S-EPMC2857887 | biostudies-literature
Project description: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.
| S-EPMC7319822 | biostudies-literature
Project description: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.
| S-EPMC6933894 | biostudies-literature
Project description:IntroductionEndomyocardial fibrosis is a neglected tropical disease of unknown etiology and poor prognosis. It is endemic of tropical climates where it is the most common cause of restrictive cardiomyopathy in the second and fourth decades of life. A forme fruste of the disease is thought to be present in temperate climates where the diagnosis remains exceedingly rare.Case presentationWe describe a case of isolated right ventricular endomyocardial fibrosis in a 27-year-old Caucasian man from a temperate climate who presented initially with frank hemoptysis and pulmonary thromboembolic disease. We further describe the approach utilized in the diagnosis, the surgical treatment and the outcome of the disease.ConclusionsWe conclude that endomyocardial fibrosis should be included in the differential diagnosis of apical cardiomyopathies in patients from temperate climates.
| S-EPMC4008263 | biostudies-literature
Project description:We present a case of late presentation nontropical endomyocardial fibrosis isolated to the right ventricle and tricuspid valve (TV). In response to deteriorating hemodynamics, surgical debulking and TV removal were performed before initiation of centralized venoarterial extracorporeal membrane oxygenation support. Definitive endomyocardial resection with a TV prosthesis was then successfully completed. (Level of Difficulty: Advanced.).
| S-EPMC8299774 | biostudies-literature
Project description: Not available
| S-EPMC8551396 | biostudies-literature
Project description:Endomyocardial fibrosis (EMF) is a poverty-related disease of unknown origin that afflicts predominantly young people of certain rural areas in low-income countries and can be used to expose global disparities in cardiovascular research. Despite affecting predominantly young people and causing high morbidity and mortality, efforts to understand its mechanisms and natural history have been hampered by the incapacity to detect the early stages of the disease in endemic areas. Dietary, environmental and infectious factors seem to combine in susceptible individuals to give rise to an inflammatory process that leads to endomyocardial damage and scar formation. Lack of awareness by health professionals and low access to health care determine late diagnosis, when complications such as chronic heart failure, thromboembolism and arrhythmia are already present. Open-heart surgery to detach the endocardial fibrous tissue and repair the atrioventricular valve, remains the last resource to prolong patients' survival. Community-based research is therefore needed to understand the epidemiology of EMF, detect early disease, uncover its pathogenesis and explore new therapeutic targets. Our research has shown that echocardiographic screening using standard criteria adds sensitivity and precision to the diagnosis, particularly in asymptomatic disease, providing an opportunity for longitudinal community-based research. However, researchers face major constraints in rural settings where EMF is endemic, including socioeconomic, cultural, geographical and administrative barriers. In presenting our experience we aim to describe the challenges and discuss the lessons learned while implementing community-based research in a highly endemic area in southern Mozambique, one of the poorest countries in the world. Additionally, we discuss how recent advances in medicine-such as use of point-of-care diagnostics, heart failure biomarkers and new imaging techniques-may open new possibilities for high quality research through collaborative partnerships and regional initiatives.
| S-EPMC7225430 | biostudies-literature