Endomyocardial Fibrosis: an Update After 70 Years. 2019

Ana Olga Mocumbi, and J Russell Stothard, and Paulo Correia-de-Sá, and Magdi Yacoub
Division of Non-Communicable Diseases, Universidade Eduardo Mondlane, Faculdade de Medicina, Maputo, Mozambique. amocumbi@gmail.com.

This review aims at highlighting the need to better understand the pathogenesis and natural history of endomyocardial fibrosis when set against its changing endemicity and disease burden, improvements in diagnosis, and new options for clinical management. Progress in imaging diagnostic techniques and availability of new targets for drug and surgical treatment of heart failure are contributing to earlier diagnosis and may lead to improvement in patient survival. Endomyocardial fibrosis was first described in Uganda by Davies more than 70 years ago (1948). Despite its poor prognosis, the etiology of this neglected tropical restrictive cardiomyopathy still remains enigmatic nowadays. Our review reflects on the journey of scientific discovery and construction of the current guiding concepts on this mysterious and fascinating condition, bringing to light the contemporary knowledge acquired over these years. Here we describe novel tools for diagnosis, give an overview of the improvement in clinical management, and finally, suggest research themes that can help improve patient outcomes focusing (whenever possible) on novel players coming into action.

UI MeSH Term Description Entries
D011203 Poverty A situation in which the level of living of an individual, family, or group is below the standard of the community. It is often related to a specific income level. Federal Poverty Level,Federal Poverty Threshold,Indigency,Low-Income Population,Absolute Poverty,Extreme Poverty,Indigents,Low Income Population,Federal Poverty Levels,Indigent,Level, Federal Poverty,Low Income Populations,Low-Income Populations,Population, Low Income,Population, Low-Income,Poverty Level, Federal,Poverty Threshold, Federal,Poverty, Absolute,Poverty, Extreme
D002313 Cardiomyopathy, Restrictive A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis. Familial Restrictive Cardiomyopathy,Cardiomyopathies, Restrictive,Restrictive Cardiomyopathies,Restrictive Cardiomyopathy
D003906 Developing Countries Countries in the process of change with economic growth, that is, an increase in production, per capita consumption, and income. The process of economic growth involves better utilization of natural and human resources, which results in a change in the social, political, and economic structures. LMICs,Less-Developed Countries,Low Income Countries,Low and Middle Income Countries,Lower-Middle-Income Country,Middle Income Countries,Third-World Countries,Under-Developed Countries,Developing Nations,Least Developed Countries,Less-Developed Nations,Third-World Nations,Under-Developed Nations,Countries, Middle Income,Countries, Third-World,Country, Least Developed,Country, Less-Developed,Country, Low Income,Country, Lower-Middle-Income,Country, Middle Income,Country, Third-World,Country, Under-Developed,Developed Country, Least,Developing Country,Developing Nation,Least Developed Country,Less Developed Countries,Less Developed Nations,Less-Developed Country,Less-Developed Nation,Low Income Country,Lower Middle Income Country,Lower-Middle-Income Countries,Middle Income Country,Nation, Less-Developed,Nation, Third-World,Nation, Under-Developed,Third World Countries,Third World Nations,Third-World Country,Third-World Nation,Under Developed Countries,Under Developed Nations,Under-Developed Country,Under-Developed Nation
D004719 Endomyocardial Fibrosis A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE). Endomyocardial Fibroses,Fibroses, Endomyocardial,Fibrosis, Endomyocardial
D006333 Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION. Cardiac Failure,Heart Decompensation,Congestive Heart Failure,Heart Failure, Congestive,Heart Failure, Left-Sided,Heart Failure, Right-Sided,Left-Sided Heart Failure,Myocardial Failure,Right-Sided Heart Failure,Decompensation, Heart,Heart Failure, Left Sided,Heart Failure, Right Sided,Left Sided Heart Failure,Right Sided Heart Failure
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017281 Cost of Illness The personal cost of disease which may be economic, social, or psychological. The cost of illness may be reflected in absenteeism, productivity, response to treatment, peace of mind, or QUALITY OF LIFE. It differs from HEALTH CARE COSTS, the societal cost of providing services related to the delivery of health care. Burden Of Disease,Burden of Illness,Disease Burden,Disease Costs,Cost of Disease,Cost of Sickness,Costs of Disease,Disease Cost,Economic Burden of Disease,Sickness Cost,Burden Of Diseases,Burden, Disease,Cost, Disease,Disease Burdens,Illness Burden,Illness Burdens,Illness Cost,Illness Costs,Sickness Costs
D058069 Neglected Diseases Diseases that are underfunded and have low name recognition but are major burdens in less developed countries. Neglected diseases include many tropical infectious diseases, e.g., HELMINTHIASIS; LEPROSY; LYMPHATIC FILARIASIS; ONCHOCERCIASIS; SCHISTOSOMIASIS; and TRACHOMA. Neglected Tropical Diseases,Disease, Neglected,Disease, Neglected Tropical,Neglected Disease,Neglected Tropical Disease,Tropical Disease, Neglected
D018450 Disease Progression The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis. Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease

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