Endomyocardial fibrosis syndrome in Uganda

Freers, Juergen; Masembe, Victoria; Schmauz, R; Mayanja‐Kizza, Harriet

doi:10.1016/s0140-6736(05)72932-9

Cited by 33 publications

(27 citation statements)

References 3 publications

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“…Abundant ascites with lymphocytic exudates and peritoneal fibrosis but without pedal edema had led researchers to propose an EMF systemic syndrome with peritoneal inflammation. [113][114][115] Decreased reabsorption of proteins and liver disease with exudative enteropathy may also occur in the most severe cases. 67,[116][117][118][119][120] In addition to nonspecific signs of chronic heart failure, including digital clubbing, growth retardation, testicular atrophy, failure to develop male secondary sexual characteristics, 121,122 and cachexia, more specific features of EMF include hypertrophy of the parotid gland, periorbital edema, and proptosis.…”

Section: Chronic Formmentioning

confidence: 99%

Tropical Endomyocardial Fibrosis

et al. 2016

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Abstract-Tropical endomyocardial fibrosis (EMF) is a neglected disease of poverty that afflicts rural populations in tropical low-income countries, with some certain high-prevalence areas. Tropical EMF is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology. Since the first descriptions in Uganda in 1948, high-frequency areas for EMF have included Africa, Asia, and South America. Although there is no clear consensus on a unified hypothesis, it seems likely that dietary, environmental, and infectious factors may combine in a susceptible individual to give rise to an inflammatory process leading to endomyocardial damage and scar formation. The natural history of EMF includes an active phase with recurrent flare-ups of inflammation evolving to a chronic phase leading to restrictive heart failure. In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF. EMF carries a very poor prognosis. In addition to medical management of heart failure, early open heart surgery (endocardectomy and valve repair/replacement) appears to improve outcomes to some extent; however, surgery is technically challenging and not available in most endemic areas. Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies.

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Section: Chronic Formmentioning

confidence: 99%

Tropical Endomyocardial Fibrosis

et al. 2016

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“…1,2 Several hypotheses may be postulated: (1) Inflammatory cause of ascites related to EMF, in favor of a systemic disease, 3,4 (2) Other causes of ascites associated with EMF, in particular, related to chronic hepatopathy, 5 the hepato-cave junction through the diaphragm muscle and pericardium with partial obstruction to hepatic venous flow as noted by Desser's comment in reference to Snapper, and (4) Different consequences of right cardiac pressure on both hepatic and systemic venous systems. Indeed, Freers and colleagues' findings substantiate speculations that EMF is a cardiac and systemic disease: peritoneal inflammation with high protein content as well as fibrosis, both leading to an increased sequestration of ascitic fluid.…”

Section: Author's Replymentioning

confidence: 99%

Ascites in Tropical Endomyocardial Fibrosis

Marijon¹

2007

Clinical Cardiology

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“…Endomyocardial fibrosis (EMF) is a type of restrictive cardiomyopathy of unknown etiology, usually found in tropical countries [1]. Davies and Conners first time described it in Uganda [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

Isolated right ventricular endomyocardial fibrosis in a young male with chronic myeloid leukemia (RCD code: III-3F.2)

Sultan

Hasan

2017

Journal of Rare Cardiovascular Diseases

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Endomyocardial fibrosis (EMF) is a rare disease of unknown etiology which is more prevalent in tropical countries. EMF is associated with fibrosis of endomyocardium, involving one or both ventricles. It usually presents with heart failure and has a poor prognosis. The diagnosis is usually made on echocardiogram, however cardiac magnetic resonance (CMR) is ideal for the diagnosis of EMF. We report a rare case of right ventricular endomyocardial fibrosis in a young male with chronic myeloid leukemia but no eosinophilia. Right ventricular EMF was diagnosed on the basis of classic findings on CMR. JRCD 2017; 3 (3): 98-100

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Endomyocardial fibrosis syndrome in Uganda

Cited by 33 publications

References 3 publications

Tropical Endomyocardial Fibrosis

Tropical Endomyocardial Fibrosis

Ascites in Tropical Endomyocardial Fibrosis

Isolated right ventricular endomyocardial fibrosis in a young male with chronic myeloid leukemia (RCD code: III-3F.2)

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