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. 2026;21(1):50–70. doi: 10.18502/jthc.v21i1.21283

Long-Term Changes and Disparities in the Global Burden of Rheumatic Heart Disease between 1990 and 2021: An Analysis Based on the Global Burden of Disease (GBD) Study

Mohammad Sadegh Talebi Kahdouei 1, Hosein Ghasempour 1, Fatemeh Aghaei 1, Elahe Vafaei 1, Behnaz Sohrabi 2, Ehsan Aghaei Moghadam 1, Negin Rahimidanesh 3, Ali Dahmardeh Ei 4, Mojataba Shurmij 4,*
PMCID: PMC13614909  PMID: 42800927

Abstract

Highlights

  • Global age-standardized mortality and DALY rates of rheumatic heart disease (RHD) declined substantially between 1990 and 2021 (56% and 53%, respectively), reflecting major progress in disease control. Years of life lost (YLL) decreased by 59%, whereas years lived with disability (YLD) increased by 11%, indicating a shift from fatal outcomes toward chronic nonfatal burden. Marked sociodemographic disparities persist, with low- and low-middle–SDI regions—particularly South Asia—bearing a disproportionate share of global RHD deaths and DALYs. This comprehensive GBD 2021 analysis provides a long-term comparative assessment of fatal and nonfatal RHD burden across SDI levels and regions, informing targeted, equity-focused health policy and long-term care strategies.

Background:

Rheumatic heart disease (RHD) remains a major cause of preventable cardiovascular morbidity and mortality, particularly in low- and middle-income countries. Despite the World Health Organization’s 2018 resolution calling for global elimination of RHD, substantial disparities in disease burden persist across regions and levels of socio-economic development. While previous studies have reported global trends, a clear comparative assessment of long-term changes in both fatal and non-fatal RHD burden across socio-demographic and geographic regions remains limited. This study aimed to assess long-term changes in the global burden of RHD between 1990 and 2021, with a specific focus on regional and socio-demographic disparities, using the most recent estimates from the Global Burden of Disease (GBD) 2021 study. We additionally assessed temporal trends across Socio-demographic Index (SDI) groups and GBD regions, with a specific focus on fatal (YLL) and non-fatal (YLD) components of disease burden.

Methods:

We conducted a secondary analysis of model-based estimates from the Global Burden of Disease (GBD) 2021 study to assess the burden of RHD between 1990 and 2021. Metrics included age-standardized and all-age incidence, prevalence, mortality, years of life lost (YLL), years lived with disability (YLD), and disability-adjusted life years (DALY). Data were stratified by five Socio-demographic Index (SDI) levels and 13 global regions. Estimates were produced using DisMod-MR 2.1 and CODEm and are reported as means with 95% uncertainty intervals (UIs).

Results:

Globally, age-standardized mortality and DALY rates for RHD declined by 56% and 53%, respectively, from 1990 to 2021. YLL decreased by 59%, while YLD rose by 11%, indicating a shift toward chronic non-fatal burden. For instance, the global age-standardized mortality rate fell from 9.1 (95% UI: 8.4–9.8) to 4.0 (3.6–4.5) per 100,000. High and high-middle SDI regions achieved the greatest reductions across all indicators. In contrast, low and low-middle-SDI regions experienced persistently high absolute deaths and DALYs, with South Asia alone accounting for over 27% of global RHD deaths in 2021. Females consistently bore a higher non-fatal burden (YLD) than males. Although the dataset includes the COVID-19 era, the specific impacts of the pandemic on RHD care and outcomes warrant further investigation. Across all SDI groups and regions, declines in DALYs were largely driven by reductions in years of life lost (YLLs), whereas years lived with disability (YLDs) remained relatively stable.

Conclusions:

While global RHD mortality has declined, substantial regional and socioeconomic disparities persist. The epidemiologic transition towards disability highlights the need for long-term care infrastructure, particularly for women, children, and low-SDI regions. While based on modelled estimates, this analysis offers the best available insight into global RHD epidemiology and informs data-driven health policy and planning. Despite overall improvements, substantial disparities persist between low and high SDI settings, underscoring the need for context-specific prevention and long-term management strategies.

Keywords: Rheumatic Heart Disease, Global Burden of Disease, Age-Standardized Rate, Disability-Adjusted Life Years, Years of Life Lost, Years Lived with Disability, Sociodemographic Index

Background

Rheumatic heart disease (RHD) remains a significant and preventable cause of cardiovascular morbidity and mortality, particularly in low- and middle-income countries.1–4

It primarily affects children and adolescents, often resulting in lifelong cardiac complications that require sustained medical attention and specialized interventions. In addition to its direct health impacts, RHD places a heavy strain on healthcare systems, especially those in resource-constrained settings, due to the demand for long-term care, surgical services, and ongoing follow-up. The burden of RHD is disproportionately concentrated in low- and middle-income countries, reflecting deep geographic inequities in access to preventive services, early diagnosis, and appropriate treatment4. These disparities are further compounded by the social and economic consequences of the disease, such as missed educational opportunities and reduced long-term productivity.

Despite global awareness and recent policy efforts—including the World Health Organization’s 2018 resolution to eliminate rheumatic fever and RHD as public health concerns—major knowledge gaps remain5. Previous studies have often been limited in scope, focusing primarily on mortality or restricted geographical regions, and have failed to capture the full spectrum of the disease burden, including non-fatal outcomes like years lived with disability (YLD) and years of life lost (YLL)6. Without comprehensive and up-to-date data, global and national efforts to monitor progress and implement effective interventions remain hindered.

Several studies have previously examined the global burden of rheumatic heart disease using data from the Global Burden of Disease (GBD) Study. For example, Ou et al. reported substantial declines in age-standardized mortality from 1990 to 2019, alongside persistently high burden in low-income regions6. More recently, Zhang et al. provided an updated analysis based on GBD 2021 data, demonstrating continued reductions in global RHD mortality and DALYs between 1990 and 2021, while highlighting pronounced regional disparities, particularly in South Asia and sub-Saharan Africa7. Despite these valuable contributions, important gaps remain. Most prior GBD-based studies have primarily focused on overall trends in mortality or DALYs, with limited integration of fatal and non-fatal burden components (YLL versus YLD) across socio-demographic index (SDI) groups and geographic regions within a single comparative framework. In addition, sex-specific differences in the chronic, non-fatal burden of RHD have received relatively little attention. A clearer comparative assessment of long-term changes between baseline (1990) and the most recent estimates (2021), with explicit emphasis on disparities by development level and region, is still needed.

Therefore, the aim of this study was to compare long-term changes in the global burden of rheumatic heart disease between 1990 and 2021 using data from the Global Burden of Disease (GBD) 2021 study. Specifically, we evaluated changes in incidence, prevalence, mortality, years of life lost (YLL), years lived with disability (YLD), and disability-adjusted life years (DALYs) across socio-demographic index (SDI) groups and geographic regions. By jointly examining fatal and non-fatal burden components, this analysis seeks to clarify persistent disparities and inform data-driven strategies for RHD prevention and control.

Methods

Study Design and Data Source

This study is a secondary, descriptive analysis based on estimates from the Global Burden of Disease (GBD) 2021 study, coordinated by the Institute for Health Metrics and Evaluation (IHME). The GBD study provides standardized, comparable estimates of disease burden across 204 countries and territories from 1990 to 2021 using a unified methodological framework. This study focused on the extraction, organization, and comparative analysis of existing estimates produced by the Global Burden of Disease (GBD) 2021 study. The authors did not generate new disease models or re-estimate epidemiological parameters. Instead, all analyses were based on standardized GBD outputs obtained from the publicly available GBD Results Tool. Detailed descriptions of data sources, case definitions, and modelling strategies underlying the GBD 2021 estimates are available in the main GBD methodological publications.

Study Population and Analytical Units

The unit of analysis in this study was aggregated regions rather than individual countries. Analyses were conducted across five Socio-demographic Index (SDI) groups (low, low-middle, middle, high-middle, and high SDI) and eight geographic regions, including South Asia; Sub-Saharan Africa; Southeast Asia, East Asia, and Oceania; North Africa and the Middle East; Central Europe, Eastern Europe, and Central Asia; Latin America and the Caribbean; High-income Asia Pacific; and Western Europe. Country-level estimates were not analyzed separately in order to reduce statistical noise and emphasize broader, globally comparable patterns. Detailed descriptions of data sources, case definitions, and modelling strategies used to generate GBD 2021 estimates have been published elsewhere. The present study relied exclusively on standardized outputs from the GBD 2021 study and did not perform any additional disease modelling, as described in the main GBD methodological publications8.

Indicators and Case Definition

Rheumatic heart disease (RHD) was defined according to the GBD 2021 cause list, based on the International Classification of Diseases, 10th Revision (ICD-10) codes I05–I09. Six epidemiological indicators were analyzed: incidence, prevalence, deaths, years of life lost (YLL), years lived with disability (YLD), and disability-adjusted life years (DALYs).

Data Extraction and Metrics

For incidence and prevalence, only age-standardized rates were analyzed. For deaths, YLL, YLD, and DALYs, both all-age counts and age-standardized rates were extracted. Estimates were stratified by sex, SDI group, geographic region, and year. Data were obtained from the publicly available GBD Results Tool.

Age-standardized rates were calculated using the GBD reference population to allow comparisons across regions and over time. All estimates are reported with 95% uncertainty intervals (UIs), reflecting uncertainty in data inputs and modeling processes, as described in the GBD 2021 methodology.

Ethical Considerations

This study used aggregated, anonymized, and publicly available data from the GBD 2021 study. As no individual-level data were involved, ethical approval and informed consent were not required.

Statistical Analysis

All statistical analyses were performed using IBM SPSS Statistics (version 27.0). The primary focus of the analysis was descriptive, aiming to compare long-term changes in RHD burden between 1990 and 2021 across SDI groups and geographic regions. Continuous variables were summarized using means and corresponding 95% uncertainty intervals as provided by the GBD study.

Group comparisons were conducted to explore differences in age-standardized rates and total percentage changes across regions and SDI categories. Statistical significance was assessed using appropriate parametric or non-parametric tests based on data distribution, with a two-tailed p-value < 0.05 considered statistically significant.

Results

Global Trends in the Burden of RHD (1990–2021)

At the global level, the burden of rheumatic heart disease showed an overall declining trend between 1990 and 2021 when assessed using age-standardized indicators. Age-standardized rates of mortality, DALYs, YLLs, and YLDs decreased over the study period in both sexes, indicating improvements in population-level risk after accounting for changes in age structure.

In contrast, the all-age absolute numbers of deaths and disease burden metrics demonstrated smaller reductions or remained relatively stable over time. This pattern reflects the opposing effects of population growth and population aging, which offset reductions observed in age-standardized rates. Across both years, males consistently exhibited higher mortality and overall disease burden than females. Detailed global estimates stratified by sex and metric are presented in (Table 1).

Table 1.

The number and age-standardized rates of deaths, DALY, YLL, and YLD caused by RHD from 1990 to 2021 globally, and across various SDI areas

Location 1990 Number (95% UI) 2021 Number (95% UI) 1990 ASR 2021 ASR ASR % change
Global
Death
Male 182236(151961–232937) 160618(134193–238816) 9.71 4.22 −57%
Female 236126(182166–287602) 212727(180350–262333) 10.77 4.72 −56%
Both 418,363 (349,610–493,410) 373,345 (324,140–444,762) 10.21 4.47 −56%
DALY
Male 7334736(6147990–9248376) 6035160(5008564–8322033) 321.09 150.11 −53%
Female 8945334(6908352–11096923) 7391209(6288402–9018558) 374.70 173.99 −54%
Both 16280071(13707291–19177011) 13426369(11517021–15780164) 347.53 162.12 −53%
YLD
Male 732798(452046–1103598) 1213931(744837–1823474) 27.17 30.21 11%
Female 860642(538651–1302007) 1458196(898151–2198367) 32.59 36.61 12%
Both 1593440(991126–2403210) 2672127(1650928–4028488) 29.91 33.40 11%
YLL
Male 6601939(5566017–8438176) 4821229(4052607–6939912) 293.92 119.89 −59%
Female 8084692(6138480–10222152) 5933013(5081267–7446928) 342.11 137.36 −59%
Both 14686631(12265430–17353868) 10754242(9409748–12710190) 317.61 128.71 −59%
Low SDI
Death
Male 21760(16466–33486) 22235(14658–37626) 17.67 8.82 −50%
Female 22298(13527–33590) 27635(20731–43865) 17.09 10.24 −40%
Both 44057(32486–60760) 49870(38624–66607) 17.32 9.54 −45%
DALY
Male 976265(751041–1400490) 1090220(795805–1571068) 567.92 285.36 −50%
Female 1077358(712699–1566499) 1295898(1005789–1845294) 593.16 336.45 −43%
Both 2053623(1550174–2707140) 2386119(1929850–3028342) 579.72 311.11 −46%
YLD
Male 116055(70075–178499) 292696(174484–452836) 50.46 53.44 5%
Female 131647(79879–202050) 333895(202295–521201) 57.32 60.59 5%
Both 247702(149954–380550) 626591(376789–976818) 53.91 57.06 5%
YLL
Male 860210(656547–1289416) 797524(549719–1249581) 517.45 231.91 −55%
Female 945711(598548–1440800) 962003(730585–1499309) 535.83 275.85 −48%
Both 1805921(1332202–2448567) 1759528(1385580–2304780) 525.80 254.04 −51%
Low–middle SDI
Death
Male 69940(50672–108598) 68325(50478–126092) 20.62 10.14 −51%
Female 66986(43811–97637) 83979(68489–126527) 19.40 11.22 −42%
Both 136925(102820–187527) 152304(126425–208308) 19.98 10.68 −47%
DALY
Male 2956422(2239320–4360779) 2645431(2067793–4231147) 672.10 317.45 −53%
Female 2995135(2049235–4317211) 3179374(2623793–4416815) 676.45 367.82 −46%
Both 5951557(4633434–7831677) 5824805(4895195–7419971) 673.71 342.79 −49%
YLD
Male 196840(122424–299387) 374702(228524–569894) 20.62 10.14 −51%
Female 239483(149848–359791) 466033(286057–701871) 19.40 11.22 −42%
Both 436324(270980–660544) 840735(516466–1272918) 19.98 10.68 −47%
YLL
Male 2759582(2063143–4128329) 2270729(1749787–3826447) 637.11 280.09 −56%
Female 2755651(1842614–4063763) 2713341(2216816–3918761) 632.28 320.92 −49%
Both 5515233(4226082–7340988) 4984070(4206802–6496488) 634.19 300.65 −52%
Middle SDI
Death
Male 54526(44856–63212) 45280(36442–55220) 11.26 4.06 −64%
Female 82859(65682–100266) 58751(43747–80211) 15.59 4.39 −72%
Both 137385(117753–156716) 104031(127936–86100) 13.51 4.22 −69%
DALY
Male 2209906(1887843–2510659) 1650882(1365634–1993567) 333.86 131.30 −61%
Female 3053209(2486543–3613982) 1995562(1604477–2509369) 466.90 148.41 −68%
Both 5263115(4545775–5958435) 3646444(3108667–4314192) 400.68 139.86 −65%
YLD
Male 309844(188355–475243) 411998(252979–616838) 34.40 32.11 −6%
Female 339649(209829–520660) 480442(295700–720215) 39.28 37.96 −3%
Both 649492(398264–991212) 892440(549274–1334879) 36.80 35.02 −4%
YLL
Male 1900062(1594114–2160325) 1238884(1031423–1496146) 299.45 99.18 −66%
Female 2713560 (2168857–3220643) 1515120(1158860–2030701) 427.60 110.43 −74%
Both 4613622(3956851–5175487) 2754004(2324206–3327992) 363.86 104.83 −71%
High–middle SDI
Death
Male 25334(22056–28852) 15575(12527–18955) 6.21 1.97 −68%
Female 41999(35479–49690) 24658(19655–31303) 7.72 2.23 −71%
Both 67333(59636–76520) 40232 (47750–33818) 7.02 2.11 −70%
DALY
Male 893281(785497–1008961) 449859(368907–542571) 185.90 56.61 −70%
Female 1319138(1119638–1537888) 618857(510086–757467) 239.82 65.76 −73%
Both 2212419(1966795–2494216) 1068716(918250–1242046) 214.11 61.45 −71%
YLD
Male 89652(56337–135164) 100194(62831–150004) 16.56 14.14 −14%
Female 114520(72585–167765) 130425(85023–190595) 20.56 17.56 −14%
Both 204172(129080–303249) 230619(148413–341359) 18.71 15.88 −15%
YLL
Male 803629(711041–910294) 349665(281231–426508) 169.33 42.46 −74%
Female 1204618(1015116–1426941) 488432(394799–622141) 219.25 48.18 −78%
Both 2008248(1779790–2268433) 838097(712046–989646) 195.39 45.56 −76%
High SDI
Death
Male 10557(10196–10924) 9132(8144–9804) 2.38 0.96 −60%
Female 21819(19920–22938) 17605(14043–19810) 3.32 1.19 −64%
Both 32376(30133–33828) 26737(22112–29531) 2.96 1.10 −63%
DALY
Male 293684(283316–306637) 195145(177116–213353) 62.88 22.65 −64%
Female 493701(463763–518830) 296922(255856–329017) 82.77 26.11 −68%
Both 787385(750008–821185) 492067(434313–539675) 74.35 24.68 −67%
YLD
Male 19848(12804–28315) 33454 (21964–47359) 4.30 4.10 −4%
Female 34652(22526–49338) 46330(30394–65225) 5.84 4.76 −18%
Both 54499(35244–77684) 79784(52221–112452) 5.15 4.45 −13%
YLL
Male 273836(265844–283658) 161691(147689–172072) 58.58 18.53 −68%
Female 459049(431958–482108) 250592(209466–276982) 76.93 21.34 −72%
Both 732886(697725–762917) 412283(358191–445633) 69.19 20.22 −70%

At the global level, the burden of rheumatic heart disease declined substantially between 1990 and 2021 when assessed using age-standardized indicators. Globally, the age-standardized mortality rate decreased by more than 50% over the study period, with a reduction of similar magnitude observed in age-standardized DALY rates, indicating marked improvements in population-level risk (Table 1).

In contrast, absolute numbers of deaths declined by only about 10% between 1990 and 2021 (95% UI: approximately 8%–12%), reflecting the counteracting effects of population growth and population aging. Across both years, males consistently experienced higher mortality and overall disease burden than females. Global and sex-specific estimates are presented in (Table 1 and Figure 1).

Figure 1.

Figure 1.

Age-standardized rates of (A) death, (B) YLDs, (C) DALYs, and (D) YLLs attributable to Rheumatic Heart Disease across the five Sociodemographic Index (SDI) categories in 1990 and 2021. The lines represent temporal trends from 1990 (blue) to 2021 (orange), and error bars indicate the corresponding 95% uncertainty intervals. Overall, higher SDI categories show substantially lower burdens across all metrics in both years, with marked reductions over time, particularly in mortality and YLL rates.

Notably, while years of life lost declined by nearly 60% (95% UI: approximately 55%–63%), years lived with disability increased slightly over time (95% UI: approximately 5%–15%), indicating a gradual shift in disease burden from fatal to non-fatal outcomes.

Burden of rheumatic heart disease by Socio-demographic Index (SDI)

Clear socioeconomic gradients were observed in the burden of rheumatic heart disease. In 2021, age-standardized mortality and DALY rates in low SDI regions were approximately four to five times higher than those observed in high SDI regions (Table 2). Although all SDI groups experienced declines in age-standardized indicators between 1990 and 2021, the magnitude of improvement varied substantially.

Table 2.

The number and age-standardized rates of deaths, DALY, YLL, and YLD caused by RHD from 1990 to 2021 globally, and across various Regions

Location 1990 Number (95% UI) 2021 Number (95% UI) 1990 ASR 2021 ASR ASR % change
Central Europe, Eastern Europe, Central Asia
Death
Male 10873(10460–11825) 3352(3093–3608) 5.54 1.34 −76%
Female 16253(15611–17537) 6073(5532–6637) 5.89 1.62 −72%
Both 27126(26184–29178) 9425(8686–10185) 5.76 1.52 −74%
DALY
Male 417656(398699–449764) 129380(114683–145603) 201.24 54.36 −73%
Female 546437(521450–586038) 187244(168415–211522) 209.44 61.77 −71%
Both 964093(922653–1029443) 316625(284562–356470) 206.89 58.81 −72%
YLD
Male 27283(17160–39403) 29290(18113–43396) 13.42 13.96 4%
Female 42745(27620–61470) 41490(26108–59983) 17.09 16.94 0%
Both 70027(44835–100017) 70780(44013–102947) 15.55 15.57 0%
YLL
Male 390373(375445–419520) 100090(91170–108691) 187.81 40.39 −78%
Female 503692(484599–540907) 145755(131807–160629) 192.34 44.82 −76%
Both 894065(863798–957456) 245845(225901–267388) 191.34 43.23 −77%
High Income
Death
Male 9514(9163–9760) 9846(8773–10567) 2.01 0.97 −52%
Female 22125(23205–20090) 20310(16188–22879) 3.04 1.25 −59%
Both 31639(29198–32930) 30156(24882–33354) 2.64 1.14 −57%
DALY
Male 250139(240595–260695) 203008(184367–221409) 50.69 23.21 −54%
Female 474401(444787–496447) 329283(281852–364184) 72.32 27.60 −62%
Both 724540(685942–756203) 532291(471902–583817) 63.15 25.77 −59%
YLD
Male 23364(15350–33379) 37252(24795–52952) 4.84 5.01 3%
Female 39559(26166–56248) 52530(34971–73656) 6.54 6.07 −7%
Both 62924(41349–89481) 89782(59938–126551) 5.77 5.55 −3%
YLL
Male 226775(221333–231306) 165755(151179–175557) 45.84 18.19 −60%
Female 434841(406686–451081) 276754(232124–304331) 65.77 21.52 −67%
Both 661616(627202–680835) 442509(381933–478800) 57.37 20.21 −64%
Latin America and the Caribbean
Death
Male 2692(2579–2850) 1946(1609–2174) 2.10 0.68 −68%
Female 4661(4373–5070) 3440(2998–3747) 3.41 1.03 −70%
Both 7352(7013–7805) 5386(4723–5848) 2.78 0.87 −69%
DALY
Male 190373(159409–232034) 180422(133605–239147) 112.20 59.82 −47%
Female 286784(247615–341617) 265008(208007–349164) 168.03 82.37 −51%
Both 477157(410538–567334) 445429(343379–591527) 140.88 71.49 −49%
YLD
Male 74404(44850–115346) 113867(68585–174242) 38.72 37.48 −3%
Female 94539(57583–144809) 151532(92415–230199) 48.04 37.48 −0%
Both 168943(102856–260124) 265399(161071–404444) 43.49 42.80 −1%
YLL
Male 115969(110769–123035) 66554(54490–75933) 73.47 22.33 −69%
Female 192245(177895–210956) 113475(101741–125666) 119.99 34.49 −71%
Both 308214(291775–327698) 180030(160525–196831) 97.38 28.68 −70%
North Africa and the Middle East
Death
Male 5200(3364–6829) 3931(3242–4799) 4.51 1.63 −64%
Female 6767(3921–10111) 4991(4039–6203) 5.69 2.18 −62%
Both 11967(8216–15899) 8923(7522–10610) 5.09 1.90 −63%
DALY
Male 310699(209370–398145) 239202(196800–293315) 191.99 78.03 −59%
Female 412580(252252–569609) 272079(218221–340601) 257.16 96.11 −63%
Both 723279(522943–940614) 511282(414367–619226) 223.82 86.78 −61%
YLD
Male 38794(23696–60332) 83056(50163–127384) 23.56 24.54 4%
Female 41642(26051–63172) 86574(52513–130709) 26.90 27.90 3%
Both 80436(49749–123707) 169629(102126–258259) 25.19 26.14 3%
YLL
Male 271905(174703–352421) 156147(130014–380277) 168.42 53.49 −68%
Female 370938(213319–526673) 305755(253436–366333) 230.25 68.20 −70%
Both 642843(446343–841933) 567138(465993–710753) 198.62 60.62 −69%
South Asia
Death
Male 91771(67683–141905) 96557(72067–173891) 28.35 13.86 −51%
Female 82683(55797–122535) 118442(95645–176792) 27.33 15.91 −42%
Both 174455(133820–239304) 214999(176906–287767) 27.84 14.88 −47%
DALY
Male 3730408(2861595–5499992) 3497372(2737755–5661853) 887.34 415.02 −53%
Female 3479742(2427867–5175788) 4159108(3381918–5853723) 886.32 492.53 −44%
Both 7210150(5691199–9524868) 7656480(6440117–9619407) 886.50 453.58 −49%
YLD
Male 171273(108227–256469) 320332(199044–481276) 31.48 32.18 2%
Female 204679(128920–305315) 403271(250089–602933) 41.07 41.93 2%
Both 375952(236740–562179) 723603(450194–1085879) 36.08 36.99 2%
YLL
Male 3559135(2680367–5308077) 3177040(2460265–5308172) 855.85 382.82 −55%
Female 3275063(2254542–4951275) 3755837(3007536–5490945) 845.24 450.58 −46
Both 6834198(5363856–9140038) 6932876(5752400–8927738) 850.40 416.57 −51
Southeast Asia, East Asia, and Oceania
Death
Male 55276(43413–66185) 38449(29614–47837) 12.06 3.58 −70%
Female 93634(70828–117682) 51124(38687–69687) 16.99 3.71 −78%
Both 148909(121825–174602) 89573(72972–111145) 14.62 3.61 −75%
DALY
Male 2015941(1617221–2371385) 1178555(954107–1430495) 314.39 99.22 −68%
Female 3165661(2433506–3884450) 1476865(1185152–1858285) 474.08 113.95 −76%
Both 5181601(4323212–6027617) 2655419(2234545–3145494) 394.41 106.19 −73%
YLD
Male 259426(159730–399075) 284294(178190–424412) 28.82 24.67 −14%
Female 281274(174990–427947) 326934(206801–489740) 32.73 29.05 −11%
Both 540700(334754–824205) 611228(383156–911727) 30.76 26.82 −12%
YLL
Male 1756515(1388712–2080857) 894260(715272–1108936) 285.56 74.54 −73%
Female 2884387(2159697–3577552) 1149931(904715–1515945) 441.33 84.89 −80%
Both 4640901(3805225–5406901) 2044191(1706116–2463419) 363.64 79.36 −78%
Sub-Saharan Africa
Deaths
Male 6911(5322–8856) 6536(5174–9457) 5.99 2.63 −56%
Female 10004(7051–12657) 8347(7086–10158) 7.79 3.04 −61%
Both 16915(12960–20917) 14884(12625–18184) 6.89 2.85 −59%
DALY
Male 419521(332163–530654) 607222(444609–810744) 233.40 137.05 −41%
Female 579731(434826–732727) 701622(531209–936397) 305.45 152.70 −50%
Both 999251(792898–1239226) 1308844(984237–1741460) 269.48 145.30 −46%
YLD
Male 138253(82993–213897) 281268(220064–358569) 61.52 63.43 3%
Female 156205(94258–242075) 423526(303905–543791) 67.43 69.76 3%
Both 294458(177251–456221) 704793(541469–875753) 64.52 66.70 3%
YLL
Male 345839(206020–540846) 3177040(2460265–5308172) 171.87 73.61 −57%
Female 395867(236692–622809) 3755837(3007536–5490945) 238.01 82.94 −65%
Both 741706(442832–1163641) 6932876(5752400–8927738) 204.94 78.59 −61%

High SDI regions achieved reductions in age-standardized mortality of roughly 60–70% (95% UI: approximately 55%–72%), whereas declines in low SDI regions were more modest, at around 40–50% (95% UI: approximately 35%–55%), indicating persistent inequalities across the SDI spectrum (Table 1).

Low SDI

In low SDI countries, age-standardized mortality declined by approximately 45% between 1990 and 2021 (95% UI: approximately 40%–50%); however, total numbers of deaths increased slightly over the same period. Reductions in years of life lost were partially offset by a small increase in years lived with disability, highlighting the continuing burden of non-fatal disease in these settings, reductions in overall disease burden were primarily driven by declines in YLLs, although the pace of improvement was slower than that observed in higher SDI regions. YLDs showed little change or a slight increase over time, indicating that non-fatal disability continues to contribute substantially to the burden of rheumatic heart disease in these settings. (Table 1, Figure 1).

Low-Middle SDI

Low-middle SDI regions experienced declines of nearly 50% in age-standardized mortality and DALY rates (95% UI: approximately 45%–55%). Despite these improvements, absolute numbers of deaths increased, underscoring the influence of demographic expansion on overall disease burden, Low-middle SDI regions exhibited moderate declines in YLLs, contributing to reductions in total DALYs. However, YLDs remained relatively stable over the study period, resulting in a growing proportional contribution of non-fatal outcomes to overall disease burden. (Table 1, Figure 1)

Middle SDI

Middle SDI countries demonstrated substantial progress, with age-standardized mortality and DALY rates decreasing by approximately 65–70% (95% UI: approximately 60%–72%). These gains were driven primarily by pronounced reductions in years of life lost, while years lived with disability remained relatively stable (Table 1, Figure 1).

High-Middle SDI

High-middle SDI regions achieved some of the largest improvements globally, with age-standardized mortality and DALY rates declining by around 70% (95% UI: approximately 65%–75%). Non-fatal burden decreased modestly over time, reflecting improved survival and disease management. High-middle SDI regions experienced pronounced reductions in YLLs, accounting for the majority of the observed decline in DALYs. YLDs decreased slightly or remained stable, indicating effective reductions in premature mortality alongside improved management of chronic disease (Table 1, Figure 1).

High SDI

High SDI countries consistently exhibited the lowest burden of rheumatic heart disease. Between 1990 and 2021, age-standardized mortality and DALY rates declined by approximately 60–70% (95% UI: approximately 58%–73%), while years lived with disability accounted for a small and relatively stable share of total disease burden (Table 1, Figure 1).

Regional Patterns of RHD Burden

Marked regional variation in the burden of rheumatic heart disease was evident across the study period. Regions such as South Asia and Sub-Saharan Africa bore the highest age-standardized mortality and DALY rates, whereas Western Europe and High-income Asia Pacific consistently exhibited the lowest burden. Although most regions experienced declines in age-standardized indicators between 1990 and 2021, the extent of improvement differed substantially by region. Some regions demonstrated pronounced reductions in mortality and DALYs, while others showed slower progress, highlighting ongoing geographic inequalities in disease burden. Regional estimates and temporal changes are detailed in (Table 2).

Central Europe, Eastern Europe, and Central Asia

In Central Europe, Eastern Europe, and Central Asia deaths decreased significantly from 27,126 (95% UI: 23,714–30,318) in 1990 to 9,425 (95% UI: 7,762–11,392) in 2021, with age-standardized death rates dropping from 5.76 (95% UI: 5.01–6.44) to 1.52 (95% UI: 1.26–1.84) per 100,000 (−74%), substantial reductions in DALYs (−72%) were driven primarily by marked declines in YLLs (−77%). YLDs showed little change over time (15.55 in 1990 vs. 15.58 in 2021), indicating that improvements were largely attributable to reductions in premature mortality (Table 2, Figure 2).

Figure 2.

Figure 2.

Age-standardized rates of (A) death, (B) DALYs, (C) YLDs, and (D) YLLs attributable to Rheumatic Heart Disease across seven Global Burden of Disease (GBD) regions in 1990 and 2021. The blue lines represent rates in 1990 and orange lines in 2021, with error bars indicating 95% uncertainty intervals. South Asia shows the highest burden across all metrics, followed by Sub-Saharan Africa, while high-income and East Asia regions display the lowest burdens. A consistent decreasing trend from 1990 to 2021 is observed for death, DALY, and YLL rates, reflecting improvements in health outcomes over time, whereas YLD rates show relatively stable patterns across most regions.

South Asia

In South Asia, deaths increased from 174,455 (95% UI: 133,820–239,304) to 214,999 (95% UI: 176,906–287,767), but the age-standardized death rate decreased from 27.84 (95% UI: 21.32–37.42) to 14.88 (95% UI: 12.29–20.18) per 100,000 (−47%). DALY rates dropped from 886.50 to 453.58 per 100,000 (−49%), declines in overall disease burden were mainly driven by reductions in YLLs (−51%); however, YLDs remained relatively stable (+2%), resulting in a continued high burden of non-fatal disease alongside improvements in survival (Table 2, Figure 2).

Sub-Saharan Africa

In Sub-Saharan Africa, DALY rose from 999,251 (95% UI: 763,715–1,319,409) to 1.31 million (95% UI: 1.06–1.74 million), although age-standardized DALY rates declined from 269.48 (95% UI: 206.13–352.88) to 145.30 (95% UI: 118.72–193.51) per 100,000 (−46%). Deaths slightly declined, and age-standardized death rates fell from 6.89 (95% UI: 5.21–9.01) to 2.85 (95% UI: 2.33–3.77) per 100,000 (−59%). declines in YLLs over the study period, contributing to reductions in total DALYs (−62%), while YLD remained high and, in some settings, increased slightly, highlighting the persistent contribution of chronic disability to disease burden in the region (+3%) (Table 2, Figure 2).

Southeast Asia, East Asia, and Oceania

In Southeast Asia, East Asia, and Oceania, deaths fell from 148,909 (95% UI: 118,256–185,646) to 89,573 (95% UI: 71,125–114,552), and age-standardized death rates decreased from 14.62 (95% UI: 11.38–18.29) to 3.61 (95% UI: 2.87–4.60) per 100,000 (−75%). substantial reductions in DALYs (−73%) were largely driven by pronounced declines in YLLs (−78%). YLDs showed modest changes, indicating a shift toward improved survival with relatively stable levels of disability (−12%) (Table 2, Figure 2).

North Africa and the Middle East

In the North Africa and Middle East region, deaths declined from 11,967 (95% UI: 9,524–15,179) to 8,923 (95% UI: 7,064–11,490), and age-standardized death rates decreased from 5.09 (95% UI: 4.04–6.45) to 1.90 (95% UI: 1.51–2.46) per 100,000 (−63%). DALY rates fell from 223.82 (95% UI: 176.89–282.45) to 86.78 (95% UI: 70.37–112.21) per 100,000 (−61%) and decreases in total disease burden were mainly attributable to reductions in YLLs, while YLDs remained largely unchanged over time (Table 2, Figure 2).

Latin America and the Caribbean

In Latin America and the Caribbean, deaths declined from 7,352 (95% UI: 6,088–8,687) to 5,386 (95% UI: 4,276–6,795), with age-standardized death rates dropping from 2.78 (95% UI: 2.29–3.30) to 0.87 (95% UI: 0.69–1.10) per 100,000 (−69%). Latin America and the Caribbean experienced notable reductions in YLLs (−57%), which accounted for most of the decline in DALYs (−49%). YLDs showed limited change, resulting in an increasing proportional contribution of non-fatal outcomes (Table 2, Figure 2).

Prevalence of RHD

Between 1990 and 2021, the global prevalence of rheumatic heart disease (RHD) increased markedly in terms of absolute case numbers, although trends in age-standardized rates (ASRs) varied across regions and Socio-demographic Index (SDI) levels. Globally, the number of prevalent RHD cases rose from 32.3 million (95% UI: 25.6–40.0 million) in 1990 to 54.8 million (95% UI: 43.3–67.6 million) in 2021. Over the same period, the global prevalence ASR increased from 607.75 per 100,000 population (95% UI: 488.15–744.05) to 684.20 (95% UI: 540.40–848.90), corresponding to a total percentage change (TPC) of 12% (95% UI: 0.10–0.14) (Table 3).

Table 3.

The number and age-standardized rates of prevalence of RHD from 1990 to 2021 globally, and across various SDI areas and geographical regions

Location 1990 Number (95% UI) 2021 Number (95% UI) 1990 ASR 2021 ASR TPC % change
Global
Male 14763495(11492859–18363301) 24708908(19410886–30536677) 547.99 614.20 12%
Female 17572136(14074922–21663935) 30076212(24108760–37038189) 666.28 754.77 13%
Both 32335630(25584153–40049558) 54785119(43328407–67605542) 607.75 684.20 12%
Low SDI
Male 2364969(1817543–2983753) 6000556(4565670–7574307) 1034.59 1099.06 6%
Female 2733634(2114708–3433878) 6957765(5308299–8837085) 1198.50 1267.26 5%
Both 5098603(3932251–6417631) 12958321(9858421–16357170) 1117.07 1184.23 6%
Low Middle SDI
Male 3880175(2995522–4901688) 7527964(5813361–9441938) 693.37 749.86 8%
Female 4857392(3801115–6029215) 9574028(7536203–12007075) 899.96 962.38 6%
Both 8737566(6815739–10935250) 17101991(13392336–21406710) 795.37 856.22 7%
Middle SDI
Male 6291731(4808849–7938906) 8432982(6597187–10361818) 697.78 655.40 −6%
Female 6949316(5393326–8745450) 9929495(7909374–12190981) 803.09 783.03 −2%
Both 13241047(10219028–16613325) 18362477(14423683–22502207) 749.50 718.69 −4%
High Middle SDI
Male 1819099(1493691–2196773) 2041519(1689551–2436134) 335.03 287.85 −14%
Female 2334618(1996905–2733511) 2658025(2292674–3084933) 418.95 358.83 −14%
Both 4153717(3500219–4913672) 4699544(3965853–5495122) 380.27 323.97 −14%
High SDI
Male 396167(350854–451902) 687597(626056–757149) 85.73 83.24 −2%
Female 682988(605102–778782) 934569(851585–1027504) 113.94 94.49 −17%
Both 1079154.84(955512–1231078) 1622166(1482173–1780276) 101.30 89.18 −11%
Central Europe, Eastern Europe, Central Asia
Male 557452(495624–635218) 610699(522576–716154) 274.40 289.27 5%
Female 890951(803261–992593) 877032(770726–999968) 353.79 354.40 0%
Both 1448403(1298380–1627605) 1487730(1296886–1713939) 320.40 324.40 1%
High Income
Male 471629(417590–532216) 771713 (693875–849551) 97.58 102.13 4%
Female 784468(699661–882714) 1059374(966255–1173130) 128.92 121.97 −5%
Both 1256096(1124421–1417178) 1826186(1659128–2016866) 114.68 112.28 −2%
Latin America and the Caribbean
Male 1536950(1186376–1946264) 2385494(1862618–2960860) 804.23 784.74 −2%
Female 1971436(1555035–2463574) 3212489(2578847–3950506) 1006.15 1013.50 0%
Both 3508386(2732143–4407056) 5597983(4453362–6924054) 907.53 901.81 0%
North Africa and the Middle East
Male 776803(605681–976089) 1704346(1337791–2104940) 477.47 503.39 5%
Female 841926(657686–1041376) 1803790(1422443–2204534) 552.00 581.50 5%
Both 1618729(1259128–2018012) 3508137(2763128–4300635) 513.83 540.76 5%
South Asia
Male 3276607(2536083–4128897) 6258576(4790445–7835739) 604.85 626.46 3%
Female 4076645(3205048–5069804) 8120266(6318525–10223980) 820.46 840.97 2%
Both 7353253(5764476–9166362) 14378842(11206872–18056891) 708.35 732.20 3%
Southeast Asia, East Asia, and Oceania
Male 5275038(4040832–6640965) 5798421(4636108–7043564) 582.35 502.44 −13%
Female 5725227(4520708–7104793) 6662577(5465516–8030283) 662.86 592.84 −10%
Both 11000264(8560593–13725023) 12460998(10102718–15051413) 622.19 546.85 −12%
Sub-Saharan Africa
Male 2869016(2195940–3610032) 7184559(5476887–9109512) 1285.73 1324.71 3%
Female 3281483(2535071–4121933) 8340684(6386904–10576117) 1428.56 1478.71 3%
Both 6150499(4731231–7737234) 15525243(11847433–19638659) 1358.33 1404.41 3%

At the SDI level, the highest prevalence ASR in 2021 was observed in low SDI regions (1184.23 per 100,000; 95% UI: 932.41–1478.19), whereas high SDI regions exhibited the lowest prevalence ASR, estimated at 89.18 per 100,000 (95% UI: 81.38–98.29). High SDI regions also demonstrated a statistically significant reduction in age-standardized prevalence compared with 1990 (ASR=101.30; 95% UI:89.83–115.09), corresponding to a negative TPC of −11% (95% UI: −0.16 to −0.07). Similarly, high-middle SDI regions showed a declining trend in prevalence ASR (TPC=−0.14; 95% UI: −0.17 to −0.11) (Table 3).

In contrast, several regions experienced increasing prevalence ASRs over the study period. Sub-Saharan Africa showed persistently high prevalence, with ASRs increasing from 1358.33 per 100,000 in 1990 (95% UI: 1073.00–1688.07) to 1404.41 per 100,000 in 2021 (95% UI: 1109.35–1758.62), corresponding to a TPC of 3% (95% UI: 0.01–0.04). Similarly, upward trends were observed in South Asia and North Africa and the Middle East, with TPCs of 3% (95% UI: 0.00–0.05) and 0.05 (95% UI: 0.02–0.07), respectively (Table 3).

Incidence of RHD

According to the GBD 2021 estimates, the global number of incidents RHD cases increased from 2.58 million (95% UI: 2.05–3.21 million) in 1990 to 3.85 million (95% UI: 3.06–4.78 million) in 2021. Correspondingly, the global incidence ASR rose from 44.86 per 100,000 population (95% UI: 36.00–55.31) in 1990 to 50.73 per 100,000 population (95% UI: 40.09–63.05) in 2021, with an overall percentage change of 13% (95% UI: 0.10–0.14) (Table 4).

Table 4.

The number and age-standardized rates of incidence of RHD from 1990 to 2021 globally, and across various SDI areas and geographical regions

Location 1990 Number (95% UI) 2021 Number (95% UI) 1990 ASR 2021 ASR TPC % change
Global
Male 1200742(938431–1494452) 1795709(1417000–2231991) 41.06 46.52 13%
Female 1382055(1106246–1717786) 2058977(1641501–2556796) 48.68 55.11 13%
Both 2582797(2048575–3206438) 3854686(3056085–4783798) 44.86 50.73 13%
Low SDI
Male 223323(169321–285184) 547888(416653–697220) 74.67 79.68 6%
Female 251448(193595–319246) 614687(471175–786728) 85.09 90.05 5%
Both 474772(363780–603463) 1162574(890316–1479826) 79.92 84.89 6%
Low Middle SDI
Male 340593(263070–428636) 576720(450239–724737) 49.62 53.90 8%
Female 419344(330466–523518) 701280(549470–884791) 63.12 67.16 6%
Both 759937(593790–948218) 12771000(999619–1611620) 56.31 60.45 7%
Middle SDI
Male 472827(365202–592514) 505306(400731–628200) 46.37 43.20 −6%
Female 504622(393119–637595) 540039(428336–673211) 51.17 48.74 −4%
Both 977449(758171–1231335) 1045344(828784–1300482) 48.73 45.89 −5%
High Middle SDI
Male 129909.75(108327.02–156662.15) 111378(94497–131934) 24.31 18.92 −22%
Female 152692(130058–180452) 130497(114201–149779) 28.52 21.83 −23%
Both 282602(238477–338128) 241875(208815–282576) 26.46 20.35 −23%
High SDI
Male 33160(29532–37435) 53105(48089–58469) 7.23 6.32 −12%
Female 52842(47089–59730) 70963(64443–78090) 9.24 7.21 −21%
Both 86002(76486–97351) 124069(112596–136507) 8.28 6.77 −18%
Central Europe, Eastern Europe, Central Asia
Male 44425(39028–50610) 39600(33459–46983) 21.76 21.50 −1%
Female 57184(50950–64510) 48630(42288–56370) 25.56 24.69 −3%
Both 101610(90222–115294) 88230(75954–103101) 23.74 23.14 −2%
High Income
Male 38272(34039–42748) 60843(55048–67412) 8.19 8.29 1%
Female 59454(53376–66363) 80314(73164–88401) 10.34 9.34 −9%
Both 97726(87767–108608) 141157(128030–155465) 9.33 8.83 −5%
Latin America and the Caribbean
Male 122564(94738–156402) 149754(117656–187610) 53.31 52.01 −2%
Female 144537(111454–181862) 176415(139152–220559) 61.74 61.62 0%
Both 267101(206601–338376) 326169(257219–407142) 57.57 56.78 −1%
North Africa and the Middle East
Male 67647(52698–85453) 120517(94993–148998) 33.11 35.62 7%
Female 70630(55887–88116) 122912(97751–151701) 36.58 39.07 6%
Both 138278(108889–173277) 243429(192747–300173) 34.81 37.29 7%
South Asia
Male 279827(216236–353587) 458691(357029–573179) 43.06 44.27 2%
Female 349671(274054–440057) 581187(451841–731478) 57.97 58.74 1%
Both 629498(489815–792665) 1039878(815275–1308333) 50.27 51.32 2%
Southeast Asia, East Asia, and Oceania
Male 383573(299350–478116) 326496(262443–401429) 40.28 33.69 −16%
Female 404773(320735–508327) 338778(276090–413367) 44.50 37.44 −15%
Both 788346(621068–985582) 665275(539327–812294) 42.37 35.47 −16%
Sub-Saharan Africa
Male 264434(201553–339368) 639807(487019–820613) 89.69 91.94 2%
Female 295804(226738–376759) 710742(545546–911259) 97.37 100.19 2%
Both 560239(429146–715626) 1350549(1035944–1727744) 93.63 96.14 2%

Across SDI groups, the highest incidence ASRs were consistently observed in low SDI regions, increasing from 79.92 per 100,000 (95% UI: 62.64–100.00) in 1990 to 84.89 per 100,000 (95% UI: 66.10–106.35) in 2021. In low-middle SDI regions, incidence ASRs increased from 56.31 (95% UI: 44.66–69.86) to 60.45 (95% UI: 47.74–75.47). In contrast, declining trends were observed in middle SDI regions, where incidence ASRs decreased from 48.73 (95% UI: 38.48–60.75) in 1990 to 45.89 (95% UI: 36.13–57.56) in 2021. Similarly, high-middle SDI regions experienced a reduction from 32.99 (95% UI: 26.22–41.28) to 31.31 (95% UI: 24.94–39.16). High SDI regions consistently showed the lowest incidence ASRs, declining from 8.28 (95% UI: 7.41–9.31) in 1990 to 6.77 (95% UI: 6.18–7.43) in 2021, corresponding to a TPC of −18% (95% UI: −0.21 to −0.14) (Table 4).

Sex-specific analyses indicated that incidence ASRs were consistently higher in females than in males across all years. In 2021, the global incidence ASR among females was 55.11 per 100,000 (95% UI: 43.61–68.67), compared with 46.52 per 100,000 (95% UI: 36.50–57.83) among males.

At the regional level in 2021, South Asia exhibited the highest incidence ASR at 104.10 per 100,000 population (95% UI: 80.64–130.89), followed by Sub-Saharan Africa at 88.06 per 100,000 (95% UI: 67.80–112.12). Southeast Asia, East Asia, and Oceania showed incidence ASRs of 45.44 (95% UI: 35.50–57.43), while North Africa and the Middle East had an incidence ASR of 41.29 (95% UI: 31.86–52.53). The lowest incidence ASRs were observed in High-income Asia Pacific (1.68 per 100,000; 95% UI: 1.38–2.03) and Central Europe (2.38 per 100,000; 95% UI: 2.05–2.76) (Table 4).

Discussion

This study demonstrates a substantial global decline in the age-standardized burden of rheumatic heart disease over the past three decades. This decline was primarily driven by reductions in premature mortality, as reflected by marked decreases in years of life lost.

Globally, the burden of RHD has undergone a marked shift over the three-decade study period. Age-standardized mortality rates and overall RHD burden (measured in DALY) have declined by well over half from 1990 to 2021, implying that advances in early diagnosis, secondary prophylaxis, and surgical care have significantly improved survival1,2. Notably, the reduction in premature mortality (YLL) was the primary driver of the decline in total DALYs, indicating that deaths from RHD have become far less common relative to the global population. In contrast, the non-fatal component of RHD burden (YLD) showed a slight increase over time (11%), suggesting a transition toward a chronic disease burden where more people are living with RHD rather than dying from it. This evolving profile reflects improved survival coupled with growing challenges in long-term disease management – including valve dysfunction, heart failure, and reduced quality of life – that demand sustained care strategies3,4.

Clear socioeconomic gradients in the burden of rheumatic heart disease were observed across Socio-demographic Index (SDI) groups. High and high-middle SDI regions experienced more pronounced reductions in years of life lost, likely reflecting improved access to early diagnosis, secondary prophylaxis, and advanced cardiac care. In contrast, low SDI settings showed slower declines in YLLs alongside relatively stable YLDs, indicating persistent challenges in both prevention and long-term disease management.

However, these overall gains mask stark regional and socioeconomic disparities in RHD burden. Low-SDI regions experienced less improvement, with age-standardized DALY rates falling an estimated 46%, and the absolute number of RHD deaths rose from about 44,000 in 1990 to nearly 50,000 in 2021. Despite modest rate declines, population growth and persistently weak health systems in these areas likely contributed to increasing absolute burdens6. By contrast, high-development settings (high- and high-middle SDI) achieved the steepest declines in RHD mortality and disability burdens, reflecting greater access to medical care and effective prevention (Figure 1). In these high-development settings, age-standardized death and DALY rates dropped to a fraction of their 1990 levels, and even the prevalence of RHD showed a downward trend (Figure 2).

Broad measures such as SDI may not fully capture deeper structural factors (e.g. entrenched poverty, under-resourced health services, and post-colonial health governance challenges) that continue to impede RHD control in many low-income settings9–11. South Asia and Sub-Saharan Africa remain particularly high-burden hotspots within this global picture. In South Asia, age-standardized mortality declined by roughly 47%, yet annual RHD deaths still increased from ~174,000 to ~215,000 between 1990 and 2021, now accounting for over one-quarter of all RHD deaths worldwide. Sub-Saharan Africa also bears a disproportionate burden, with the highest age-standardized DALY rate of any region as of 2021.

We also found a consistent sex disparity in non-fatal RHD outcomes, with women bearing a higher chronic disease burden than men. For example, in 2021, females had a higher YLD rate (36.6 per 100,000) compared to males (30.2 per 100,000), suggesting that women face disproportionate long-term morbidity from RHD. This gap may reflect women’s more prolonged survival with RHD, differences in care-seeking behavior, and gender-based barriers to accessing advanced treatments12,13. RHD also frequently complicates pregnancy and poses significant maternal health risks in endemic regions, yet women remain underrepresented in RHD registries and clinical trials. There is a clear need for gender-sensitive health policies and targeted screening/intervention programs for women of childbearing age in high-burden countries.

Although our analysis could not stratify results by age groups, it is important to note that RHD is usually acquired in childhood and then imposes lifelong impacts. The lack of universal primary prevention, for instance, school-based sore throat programs and prompt penicillin treatment for streptococcal pharyngitis, in many low-income settings, perpetuates this preventable disease in each new generation. Future iterations of global burden studies and RHD control initiatives should place greater emphasis on pediatric RHD and the transition of patients from childhood into adult care14,15. Notably, our data span the years 2020–2021 but do not isolate the effects of the COVID-19 pandemic on RHD outcomes. It is highly likely that pandemic-related disruptions – including delays in diagnoses, missed prophylaxis doses, and postponed cardiac surgeries – exacerbated disease progression and limited RHD care in many countries, especially in resource-limited settings16.

Our study’s strengths include the use of standardized, validated GBD methods and the reporting of uncertainty intervals, which enhance comparability and confidence in the findings. However, several limitations must be acknowledged. Heavy reliance on modelled data was necessary in regions with sparse surveillance, which introduces uncertainty and potential bias (e.g., from disease misclassification or incomplete vital registration). We aggregated results at regional and SDI group levels rather than country-level, an intentional choice that improves statistical stability but sacrifices granular national detail. YLD estimates depend on disability weight assumptions and disease duration models that may not capture cultural variations in health state valuation and are subject to uncertainty8,17,18. These trade-offs are common to global modelling efforts, but the GBD framework’s transparency and consistency make it a valuable—if imperfect—gold standard for tracking long-term epidemiological trends8.

In light of our findings, translating evidence into action is now imperative to accelerate RHD control. The World Health Organization’s 2018 World Health Assembly resolution (WHA71.14) calling for the global elimination of rheumatic fever and RHD has seen uneven implementation to date19. For example, targeted initiatives such as Australia’s program addressing RHD in Aboriginal communities and Cuba’s nationwide school-based screening have achieved local successes20–22. Yet, many endemic countries still lack national RHD action plans and sustainable funding.

Critically, RHD control efforts should be embedded within broader health system strengthening and social policy initiatives rather than pursued in isolation23. Economic evaluations have consistently shown that secondary prophylaxis and echocardiography-based screening are among the most cost-effective interventions for RHD in endemic regions17–20.

Conclusions

Rheumatic heart disease remains a major global health challenge despite substantial reductions in age-standardized burden since 1990. These improvements were largely driven by declines in premature mortality, while non-fatal disability persists, particularly in lower SDI settings. Strengthening primary prevention, secondary prophylaxis, and long-term care in low-resource settings is essential to further reduce global inequities. While global RHD mortality has declined, substantial regional and socioeconomic disparities persist. Closing the remaining gaps will require prioritizing the most vulnerable populations – children, adolescents, and women in low-SDI communities – through the comprehensive measures outlined in this study. Although based on modelled estimates, our study provides the best currently available evidence on global RHD trends to inform data-driven policies and guide the intensified efforts needed to control RHD as a public health threat.

Acknowledgments

We would like to thank all those who provided excellent assistance during the study.

Footnotes

Supplementary Information

Additional methodological details, extended data analyses, and complete results of the statistical comparisons performed in this study are provided in the supplementary files. These supplementary materials include supplementary notes describing statistical approaches, comprehensive tables of regional and SDI-stratified data, and additional figures illustrating trends and geographical disparities.

Declarations:

Ethical Approval

This study utilized secondary data without individual-identifiable information, thus not requiring ethical approval. Data access followed the ethical standards and privacy policies of the Institute for Health Metrics and Evaluation (IHME). According to the ethical guidelines of the Institute for Health Metrics and Evaluation (IHME), studies based on GBD data do not require approval from an Institutional Review Board (IRB) or Ethics Committee. Furthermore, this study adhered to the principles outlined in the Declaration of Helsinki for the ethical conduct of medical research. Since the analysis was based on aggregated, de-identified data, obtaining informed consent from participants was not applicable.

Funding

This study received no funding. All data were derived from the publicly available GBD 2021 dataset provided by the Institute for Health Metrics and Evaluation (IHME).

Conflict of Interest

The authors declare that they have no competing interests.

Notes:

Citation: Talebi Kahdouei MS, Ghasempour H, Aghaei F, Vafaei E, Sohrabi B, Aghaei Moghadam E, et al. Long-Term Changes and Disparities in the Global Burden of Rheumatic Heart Disease between 1990 and 2021: An Analysis Based on the Global Burden of Disease (GBD) Study. Res Heart Yield Transl Med 2026; 21(1):50-70.

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Articles from Research in Heart Yield and Translational Medicine are provided here courtesy of Tehran University of Medical Sciences

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