Endomyocardial fibrosis: why is there striking ascites with little or no peripheral oedema?

Freers, Juergen; Mayanja‐Kizza, Harriet; Rutakingirwa, M; Gerwing, E

doi:10.1016/s0140-6736(96)90383-9

Cited by 28 publications

(19 citation statements)

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“…It has been speculated that, in genetically predisposed people, poor diet per se may lead to a dysfunction in the regulatory and functional mechanisms of eosinophils, which, as in eosinophilic leukemia, may cause necrosis, thrombosis, and finally fibrosis. 67 This is supported by the fact that long-standing dietary imbalance with low-protein intake is generally seen along with EMF. 12 One of the more popular diet-related hypotheses has proposed an important role for cassava, which is almost universally consumed in certain areas of Africa and other developing areas.…”

Section: Environmental Factors Dietary Factors and Toxinsmentioning

confidence: 96%

“…67,68 The disease afflicts individuals in rural settings, which are also endemic for malaria, but again, geographic distributions of EMF and malaria have not matched in a coherent fashion to allow causal links to be drawn. 30,[69][70][71][72][73][74][75][76][77][78][79] Furthermore, malaria has never been described as being associated with myocardial or endocardial damage.…”

Section: 63mentioning

confidence: 99%

“…[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%

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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: Environmental Factors Dietary Factors and Toxinsmentioning

confidence: 96%

Section: 63mentioning

confidence: 99%

Section: Chronic Formmentioning

confidence: 99%

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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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“…Patients with huge ascites are usually hard to clinically control and only slightly responsive to diuretics 4 . Some authors have suggested the existence of an inflammatory component in the formation of ascites 5 . Approximately 60% of the patients have eosinophilia 6 in the peripheral blood, which may be related to parasitic infestation.…”

Section: Endomyocardial Fibrosis Is a Common Disease In Tropical Counmentioning

confidence: 99%

Transplante cardíaco em portadora de endomiocardiofibrose

Freitas¹,

Castro²,

Chizzola³

et al. 2005

Arq. Bras. Cardiol.

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a therapeutic option, although little used, with only one report of heterotopic transplantation reported in the literature. Case ReportThe patient is a 50-year-old white female, housewife, born in the town of São Sebastião, in the state of Alagoas, who was referred to InCor in 1996, complaining of enlargement of the abdominal volume associated with lower limb edema for 3 years. The patient reported dyspnea associated with huge ascites, which improved with relief punctures performed every 20-30 days, even though she was using diuretics. The patient underwent investigation for hepatopathy in the gastroenterology unit of the Hospital das Clínicas. Once the viral, alcoholic and schistosomal etiologies were excluded, the patient was referred to InCor with the diagnostic hypothesis of chronic hepatopathy secondary to right heart failure. On admission, the physical examination showed significant jugular venous distension, irregular cardiac rhythm with a blood pressure of 140/80 mmHg, and heart rate of 80 bpm. Her pulmonary auscultation was normal. The patient had voluminous ascites with significant lower limb edema. The electrocardiogram showed atrial fibrillation and right bundle-branch block. The laboratory tests were normal.The echocardiogram showed a dilated and hypokinetic right ventricle, with a diastolic diameter of 3.8 cm, and left ventricular diastolic diameter and ejection fraction of 4.5 cm and 73%, respectively. The right atrium was enlarged. The pericardium was thickened with a mild effusion. The atrioventricular valvular filling was of the protodiastolic type with the E wave greater than the A wave. The clinical diagnostic hypothesis of severe restrictive cardiomyopathy with right predominance and significant tricuspid insufficiency was established.The patient underwent cardiac catheterization, which showed coronary circulation without obstructive lesions, a normal left ventricle, and loss of the right ventricular intracavitary geography and amputation of the right ventricular trabecular region, suggesting the diagnosis of right ventricular endomyocardial fibrosis.As the patient remained symptomatic with refractory ascites, requiring relief punctures every 15 days, surgical treatment was chosen, with resection of the right ventricular endomyocardial fibrosis and DeVega tricuspid valve annuloplasty.The anatomicopathological examination of the surgical specimen confirmed the clinical and surgical impression, revealing "dense fibrosis of the endocardium, with a band containing neoformed vessels and calcified areas, an aspect compatible with endomyocardial fibrosis".

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Endomyocardial fibrosis: why is there striking ascites with little or no peripheral oedema?

Cited by 28 publications

References 1 publication

Tropical Endomyocardial Fibrosis

Tropical Endomyocardial Fibrosis

Ascites in Tropical Endomyocardial Fibrosis

Transplante cardíaco em portadora de endomiocardiofibrose

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