RESUMO
Rheumatic Fever (RF)/ Rheumatic Heart Disease (RHD) is the result of autoimmune response triggered by group A Beta-haemolytic streptococcal pharyngitis leading to immune-inflammatory injury to cardiac valves. It is practically disappeared in developed countries. However, it continues to be a major cause of disease burden among children, adolescents, and young adults in low-income countries and even in high-income countries with socioeconomic inequalities. For decades, many cases of Acute Rheumatic Fever (ARF) and RHD were missed and were denied the secondary prophylaxis, as a result these patients used to end up with complications and untimely death. Advanced understanding of the echocardiography can prevent both under diagnosis and over diagnosis and thus help in management strategy. Another new advancement in recent past is the mitral valve repair, which is technically demanding, and the results are acceptable in experienced cardiac surgical units. Whenever feasible, valve repair should be preferred over valve replacement since it precludes the need for anticoagulation and future risks of prosthesis dysfunction.