Presentation
The salient features of this condition are present in the case presented here of a 14-year-old boy who died from progressive heart failure due to right ventricular EMF. [ncbi.nlm.nih.gov]
Entire Body System
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Turkish
We report a case of right ventricular endomyocardial fibrosis mimicking Ebstein anomaly in a 26-year-old male Turkish patient with Behçet's disease, who had heart failure symptoms. [ncbi.nlm.nih.gov]
Respiratoric
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Respiratory Distress
The newborn weighed 3,400 g and had progressive respiratory distress right after birth. [scielo.br]
Cardiovascular
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Heart Disease
We report this rare case of mitral valve prolapse in EMF, in a geographical area where rheumatic heart disease is endemic, to showcase how a rare manifestation of EMF can be misdiagnosed as that of rheumatic heart disease. [ncbi.nlm.nih.gov]
Rheumatic heart disease and endomyocardial fibrosis: Distinguishing the etiology of mitral regurgitation in low-resourced areas Abstract Rheumatic heart disease (RHD) and endomyocardial fibrosis (EMF) are 2 neglected cardiovascular diseases that disproportionately [journals.ac.za]
Heart Disease. 5th ed. Philadelphia, Pa:. WB Saunders and Co;1997:1433-4. Zabsonre P, Renambot J, Adoh-Adoh M, et al. [wikidoc.org]
In endemic areas of Africa endomyocardial fibrosis is as common a cause of heart failure as rheumatic heart disease, accounting for up to 20% of cases of heart failure and imposes a considerable burden to the communities and the health systems. [globalcardiologyscienceandpractice.com]
Not uncommonly EMF is detected incidentally in patients with rheumatic or other heart diseases. [18] The first case was initially managed as rheumatic heart disease before referral to the cardiology unit. [nigjcardiol.org]
Neurologic
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Neglect
KEYWORDS: Endomyocarial fibrosis; Heart failure; Neglected diseases; Restrictive cardiomyopathy [ncbi.nlm.nih.gov]
Workup
European citizens with no history of travelling to tropical countries. 3 Some of these reports have communicated severely calcified forms of this disease. 4 We would like to state that an exhaustive evaluation was performed to complete the diagnostic workup [revespcardiol.org]
Ultrasound
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Enlargement of the Spleen
Facial swelling (edema), swelling of the legs, enlargement of the spleen and liver (hepatosplenomegaly), and an accumulation of fluid in the abdominal cavity (ascites) are to be expected. [rarediseases.org]
Other Pathologies
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Myocardial Fibrosis
A 50-year-old female patient with endo- myocardial fibrosis presented with unusual calcifications of the right ventricular endocardium that embedded the tricuspid valve papillary muscles. [ncbi.nlm.nih.gov]
fibrosis are evident, Endomyocardial Fibrosis with Ventricular Thrombosis: Gross, fixed tissue, horizontal section of ventricles showing thrombus filling both.Left ventricle endocardial fibrosis and focal myocardial fibrosis. [wikidoc.org]
Finally, on cardiac MRI subendocardial late gadolinium hyperenhancement was observed in the left ventricular apex ( Figure 1 D, arrow), reflecting myocardial fibrosis. The patient's clinical context supports the diagnosis of an initial stage of EF. [revportcardiol.org]
Diagnosis: “Endo myocardial fibrosis.” The correspondent author’s postmortem report External appearances: The body was that of an emaciated young man who was very pale. The ankles pitted on pressure. [scitcentral.com]
Treatment
Outcome was favorable under treatment with prednisone and azathioprine.This case illustrates a favorable outcome of endomyocardial fibrosis during pregnancy. [ncbi.nlm.nih.gov]
Prognosis
Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients. [ncbi.nlm.nih.gov]
Etiology
Endomyocardial fibrosis (EMF) is a rare progressive restrictive cardiomyopathy of unknown etiology that mainly presents with heart failure. [ncbi.nlm.nih.gov]
The etiology, pathogenesis, echocardiographic findings, interventions and prognosis are quite distinct. [journals.ac.za]
Epidemiology
However, due to lack of resources for research in these areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. [globalcardiologyscienceandpractice.com]
Due to lack of resources for research in the disease endemic areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. [scopemed.org]
Part II: An epidemiologic, clinical, and pathologic study. Am Heart J 1968; 75: 107–124. Connor DH, Somers K, Hutt MS, et al. Endomyocardial fibrosis in Uganda (Davies’ disease). Part I: An epidemiologic, clinical, and pathologic study. [jrcd.eu]
Advances have, however, been made in establishing the epidemiology and improving clinical diagnosis and management, through modern medical therapy and improved surgical techniques. [ncbi.nlm.nih.gov]
An epidemiologic, clinical, and pathologic study. Am Heart J 74 : 687–709 3. Sliwa K et al. (2005) Epidemiology and etiology of cardiomyopathy in Africa. Circulation 112 : 3577–3583 4. [nature.com]
Pathophysiology
The pathophysiological hallmark of the disease is focal or diffuse endocardial thickening involving mainly the inflow, the apices, and the subvalvular region leading to valvular regurgitation, diastolic dysfunction and obliteration of the ventricular [ncbi.nlm.nih.gov]
Pathophysiology EMF involves both the right and left ventricle in 50% of cases. Forty percent of cases have solely the left ventricle involved, and in the remaining 10% of cases, the right ventricle is involved. [statpearls.com]
[…] including hypersensitivity myocarditis [14], parasitic infections [12,13], eosinophilic leukemia, sarcoma, carcinoma, and lymphoma [15], GM-CSF administration [16], and prolonged drug-induced eosinophilia Several infections have been implicated in the pathophysiology [slideshare.net]
However, there is need to fully understand the pathogenesis and pathophysiology of EMF in order to improve the management and outcome of the affected patients. [link.springer.com]
Prevention
CONCLUSION: Short term prednisolone use was generally safe and did not prevent reaccumulation of ascites in this study population. TRIAL REGISTRATION NUMBER: ISRCTN63999319, 28/03/2013. [ncbi.nlm.nih.gov]
While RHD is unarguably the most preventable of all cardiac diseases, resulting from untreated or undertreated group A streptococcal infections, EMF’s etiology remains unclear. [journals.ac.za]