Endomyocardial fibrosis, first described >75 years ago, is a cause of restrictive cardiomyopathy with an unclear aetiopathogenesis that is most commonly found in children and adolescents from tropical regions of Africa, Asia and South America. The epidemiological trends of this cardiomyopathy are difficult to ascertain. The characteristic hallmark of endomyocardial fibrosis is ventricular fibrosis that causes diastolic dysfunction and atrioventricular regurgitation. Although advances in medical treatment for heart failure and more tailored surgical techniques to treat the condition have increased survival, the outcomes in affected patients remain poor. A major focus of research is the identification of biomarkers of preclinical disease and new therapeutic targets. Collaborative multidisciplinary research and cross-learning from other fibrotic conditions should impart knowledge and help to improve the survival rates and the quality of life of patients with endomyocardial fibrosis.
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