Endomyocardiofibrosis: A Systematic Review |
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| Authors: | Cristhian Emmanuel Scatularo Edith Liliana Posada Martínez Clara Saldarriaga Oswaldo Andrés Ballesteros Adrian Baranchuk Alvaro Sosa Liprandi Fernando Wyss María Ines Sosa Liprandi |
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| Affiliation: | 1. Division of Cardiology, Sanatorio de la Trinidad Palermo, Buenos Aires, Argentina;2. Echocardiography department, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México;3. Department of Cardiology and Heart Failure Clinic, Cardiovascular Clinic Santa Maria, University of Antioquia, Medellín, Colombia;4. Division of Cardiology, Kingston Health Science Center, Queen''s University, Kingston, Ontario, Canada;5. Division of Cardiology, Sanatorio Güemes, Buenos Aires, Argentina;6. Cardiovascular Services and Technology of Guatemala - Cardiosolutions, Guatemala City, Guatemala;7. Department of Cardiology and Heart Failure Unit, Sanatorio Güemes, Buenos Aires, Argentina;1. Department of Cardiology, Boali Hospital, Qazvin University of Medical Sciences, Qazvin, Iran;2. Metabolic Diseases Research Center, Research Institute for Prevention of Non-Communicable Diseases, Qazvin University of Medical Sciences, Qazvin, Iran;1. Department of Cardiology, National Heart Centre Singapore, Singapore, Singapore;2. Duke-NUS School of Medicine, National University of Singapore, Singapore, Singapore;3. Changi Aviation Medical Centre, Changi General Hospital, Singapore, Singapore;1. Department of Internal Medicine, University of Tennessee Health Science Center, Memphis, TN;2. Department of Medicine, Baptist Memorial Hospital, Memphis, TN;3. College of Medicine, University of Tennessee Health Science Center, Memphis, TN;4. Kaplan-Amonette Department of Dermatology, University of Tennessee Health Science Center, Memphis, TN,;5. Department of Medicine, Division of Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis, TN |
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| Abstract: | Endomyocardiofibrosis was described first time in Uganda as an infrequent restrictive cardiomyopathy with a poor prognosis, characterized by fibrosis of the ventricular subendocardium and severe restrictive physiology leading to difficult therapeutic management and frequently associated with hypereosinophilic syndrome. Its higher prevalence in the tropics and its relationship in some cases with hypereosinophilic endocarditis has led to the search for genetic, infectious, autoimmune and nutritional causes, but its etiology remains unclear. It is a rare cardiomyopathy, difficult to diagnose and with a nonexistent effective treatment. Imaging methods such as echocardiography and cardiac magnetic resonance are essential for the initial diagnosis, although endomyocardial biopsy establishes the definitive diagnosis. Immunosuppressive treatment is only useful in the early stages of the disease and usually ineffective if installed late when signs of heart failure are present. Surgical treatment is generally palliative. |
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