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Background: Rheumatic heart disease (RHD) is a major challenge to global health, primarily in low- and middle-income countries. Even though RHD is rare in high-income countries, it still poses a health challenge, yet there is a lack of data on its impact within the highest-income regions. Objectives: The purpose of this study was to compare the epidemiology of RHD in the United States of America (USA) and the European Union (EU). Methods: Data on RHD burden were collected using the Global Disease Burden Study 2021 using the Global Health Data Exchange query tool. Age-standardized rates of incidence (ASIR), prevalence (ASPR), death (ASDR), disability-adjusted life years (ASDALY), years lived with disability (ASYLD), and years of life lost (ASYLL) were collected. Estimated annual percentage change (EAPC) was calculated. Results: In USA in 2021, the ASPR was 123.4/100,000 with a decreasing annual trend of 0.32% since 1993. Between 2021 and 1993, the ASIR decreased from 10.6 to 10.0/100,000 cases. In the EU in 2021, the ASPR was 49.7/100,000 cases, with an annual decreasing trend of 1.6% between 2021 and 1993. Between 2021 and 1993, the ASIR decreased from 7.43 to 4.6/100,000 cases. The changing prevalence and incidence in the US was primarily driven by Florida, Nevada, and Tennessee. There has been a reversal in the burden of RHD since 2007 in the USA. In both regions, there was a significant gender disparity with female predominance. The EU reported higher age-standardized disability-adjusted life years and age-standardized years of life losts than the USA. Conclusions: The varied trends underscore the complexity of RHD epidemiology and the need for region-specific strategies to address this persistent health challenge.