Endomyocardial Fibrosis

Mocumbi, Ana Olga

doi:10.1007/978-1-4471-4619-3_10

Cited by 2 publications

(4 citation statements)

References 54 publications

(97 reference statements)

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“…Central cyanosis in the absence of a patent foramen ovale, severe right heart failure with ascites but no pedal edema, marked parotid swelling, asthma-like episodes in the absence of blood hypereosinophilia, and the characteristic hyperpigmentation of lips and gums remain subjects of controversy. 23,24 Right EMF is clearly associated with increased clot formation and pulmonary embolism. Early disease causes acute pulmonary embolism and sudden death from ventricular thrombi, while advanced EMF is associated with chronic pulmonary thromboembolism due to stasis and clot formation inside the right atrium.…”

Section: Pathophysiologymentioning

confidence: 99%

“…The pathophysiology of some distinctive features of right EMF is not clearly understood. Central cyanosis in the absence of a patent foramen ovale, severe right heart failure with ascites but no pedal edema, marked parotid swelling, asthma‐like episodes in the absence of blood hypereosinophilia, and the characteristic hyperpigmentation of lips and gums remain subjects of controversy 23 , 24 …”

Section: Pathophysiologymentioning

confidence: 99%

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Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

Mocumbi

2014

Pulm. circ.

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Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy of unknown origin, affecting predominantly the right side of the heart. Its highest prevalence is in poor regions of sub-Saharan Africa, where it is usually found in children and adolescents. In areas where it is endemic, EMF is a major cause of heart failure and premature death. EMF of the right ventricle has unique clinical, electrocardiographic, and echocardiographic signs. Several features of advanced disease are not fully understood, the most striking being the presence of severe ascites with little or no pedal edema. Echocardiography is the main diagnostic tool and supports management of the disease in most patients, as it allows assessment of the severity and extension of endocardial fibrosis, the quality of the right atrioventricular valve, and the presence of intracardiac thrombi. Pulmonary hypertension in the setting of right EMF is related to chronic thromboembolism, through the chronic thrombi present in the severely enlarged right atrium found in advanced disease. The management of right EMF is difficult. Treatment of symptomatic patients should be surgical. However, as the disease is usually detected in late stages, with severe and advanced lesions, surgery cannot be performed without a very high risk of death and complications. New surgical approaches addressing several components of the structural cardiac abnormalities have been attempted, with promising results. EMF remains one of the most neglected diseases worldwide, and research into its pathophysiological mechanisms will probably improve outcomes and alter the natural history of the disease. This requires improvement in the health systems in areas of endemicity as well as the design of international collaborative research projects.

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Section: Pathophysiologymentioning

confidence: 99%

Section: Pathophysiologymentioning

confidence: 99%

Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

Mocumbi

2014

Pulm. circ.

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“…In 2014, Mocumbi considered that in the field pf cardiovascular medicine, EMF is perhaps the most neglected disease; and despite its high prevalence rate in Africa, Asia and South America, few human and material resources are made available in these regions for research on its mechanism—[ 129 ]…”

Section: Introductionmentioning

confidence: 99%

“…Today (2021), the exact aetiology and pathogenesis of EMF still remains unknown (See e.g., [ 123 , 125 , 129 ])…”

Section: Introductionmentioning

confidence: 99%

The position of geochemical variables as causal co-factors of diseases of unknown aetiology

Davies

2022

SN Appl. Sci.

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The term diseases of unknown aetiology (DUA) or idiopathic diseases is used to describe diseases that are of uncertain or unknown cause or origin. Among plausible geoenvironmental co-factors in causation of DUA, this article focusses on the entry of trace elements, including metals and metalloids into humans, and their involvement in humoral and cellular immune responses, representing potentially toxic agents with implications as co-factors for certain DUA. Several trace elements/metals/metalloids (micronutrients) play vital roles as co-factors for essential enzymes and antioxidant molecules, thus, conferring protection against disease. However, inborn errors of trace element/metal/metalloid metabolisms can occur to produce toxicity, such as when there are basic defects in the element transport mechanism. Ultimately, it is the amount of trace element, metal or metalloid that is taken up, its mode of accumulation in human tissues, and related geomedical attributes such as the chemical form and bioavailability that decisively determine whether the exerted effects are toxic or beneficial. Several case descriptions of DUA that are common worldwide are given to illustrate our knowledge so far of how trace element/metal/metalloid interactions in the immune system may engender its dysregulation and be implicated as causal co-factors of DUA. Article highlights The importance of a proper understanding of geochemical perturbations in human metabolisms is emphasised It is proferred that such an understanding would aid greatly in the decipherment of diseases of unknown aetiology (DUA) The thesis presented may pave the way towards better diagnosis and therapy of DUA

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Endomyocardial Fibrosis

Cited by 2 publications

References 54 publications

Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

The position of geochemical variables as causal co-factors of diseases of unknown aetiology

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