BACKGROUND: The aim of this study was to estimate the burden of rheumatic heart disease (RHD) and its trends in different countries, regions, genders and age groups globally. METHODS: Data were obtained from the Global Burden of Disease 2019 study. Age-standardized rates (ASRs) and the estimated annual percentage changes (EAPCs) in the ASRs were used to describe the burden of disease and its trends. Pearson's correlation was used to evaluate the correlation between sociodemographic index (SDI) values and the observed trends. RESULTS: In 2019, the ASRs of the incidence, prevalence, mortality and disability-adjusted life years (DALYs) of RHD were 37.39/10 (95%UI, 28.59/10 to 46.74/10), 513.68/10 (95%UI, 405.01/10 to 636.25/10), 3.85/10 (95%UI, 4.29/10 to 3.29/10) and 132.88/10 (95%UI, 115.02/10 to 150.34/10), respectively. From 1990 to 2019, the incidence and prevalence of RHD showed upward trends and the mortality and DALYs showed downward trends. Countries or regions in Africa, South America and South Asia had a greater burden of RHD. The burden of RHD was greater in women, where as men showed more obvious increasing trends in the incidence and prevalence. The incidence of RHD was highest in adolescents, and the prevalence was highest in young and middle-aged. The mortality and DALYs rate associated with RHD increased with age. The EAPCs in the ASRs were negatively correlated with the SDI value. CONCLUSION: Although the ASRs of mortality and DALYs attributable to RHD are decreasing globally, RHD remains an important public health problem that needs to be addressed urgently, especially in certain low- and middle-income countries and regions.