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Rheumatic heart disease, often neglected by media and policy makers, is a major burden in developing countries where it causes most of the cardiovascular morbidity and mortality in young people, leading to about 250 000 deaths per year worldwide. The disease results from an abnormal autoimmune response to a group A streptococcal infection in a genetically susceptible host. Acute rheumatic fever—the precursor to rheumatic heart disease—can affect different organs and lead to irreversible valve damage and heart failure. Although penicillin is effective in the prevention of the disease, treatment of advanced stages uses up a vast amount of resources, which makes disease management especially challenging in emerging nations. Guidelines have therefore emphasised antibiotic prophylaxis against recurrent episodes of acute rheumatic fever, which seems feasible and cost effective. Early detection and targeted treatment might be possible if populations at risk for rheumatic heart disease in endemic areas are screened. In this setting, active surveillance with echocardiography-based screening might become very important.

Among patients with rheumatic heart disease and atrial fibrillation who received a vitamin K antagonist or rivaroxaban, the rate of stroke, systemic embolism, MI, or death from vascular or unknown causes was lower with a VKA, without increased bleeding.

Data from the Global Burden of Disease study indicate that there were 319,400 deaths due to rheumatic heart disease and 33.4 million cases of rheumatic heart disease in 2015. The highest death and prevalence rates were found in Oceania, South Asia, and central sub-Saharan Africa.

We describe trends in acute rheumatic fever (ARF), rheumatic heart disease (RHD), and RHD deaths among population groups in New Zealand. We analyzed initial primary ARF and RHD hospitalizations during 2000-2018 and RHD mortality rates during 2000-2016. We found elevated rates of initial ARF hospitalizations for persons of Māori (adjusted rate ratio [aRR] 11.8, 95% CI 10.0-14.0) and Pacific Islander (aRR 23.6, 95% CI 19.9-27.9) ethnicity compared with persons of European/other ethnicity. We also noted higher rates of initial RHD hospitalization for Māori (aRR 3.2, 95% CI 2.9-3.5) and Pacific Islander (aRR 4.6, 95% CI 4.2-5.1) groups and RHD deaths among these groups (Māori aRR 12.3, 95% CI 10.3-14.6, and Pacific Islanders aRR 11.2, 95% CI 9.1-13.8). Rates also were higher in socioeconomically disadvantaged neighborhoods. To curb high rates of ARF and RHD, New Zealand must address increasing social and ethnic inequalities.

In a randomized trial of secondary antibiotic prophylaxis in Ugandan children and adolescents with latent rheumatic heart disease, penicillin G benzathine given every 4 weeks for 2 years reduced the risk of disease progression. Among 458 participants in the prophylaxis group, 2 had serious adverse events that were attributable to prophylaxis, including one episode of anaphylaxis.

Rheumatic heart disease follows Group A Streptococcus (GAS) infection in Aboriginal Australians. A genome-wide study identified HLA as the strongest genetic risk factor. Risk and protective HLA_DQA1_DQB1 haplotypes bound with different affinities to core epitopes of pathogenic GAS M proteins. Abstract Background Rheumatic heart disease (RHD) after group A streptococcus (GAS) infections is heritable and prevalent in Indigenous populations. Molecular mimicry between human and GAS proteins triggers proinflammatory cardiac valve-reactive T cells. Methods Genome-wide genetic analysis was undertaken in 1263 Aboriginal Australians (398 RHD cases; 865 controls). Single-nucleotide polymorphisms were genotyped using Illumina HumanCoreExome BeadChips. Direct typing and imputation was used to fine-map the human leukocyte antigen (HLA) region. Epitope binding affinities were mapped for human cross-reactive GAS proteins, including M5 and M6. Results The strongest genetic association was intronic to HLA-DQA1 (rs9272622; P = 1.86 × 10−7). Conditional analyses showed rs9272622 and/or DQA1*AA16 account for the HLA signal. HLA-DQA1*0101_DQB1*0503 (odds ratio [OR], 1.44; 95% confidence interval [CI], 1.09–1.90; P = 9.56 × 10−3) and HLA-DQA1*0103_DQB1*0601 (OR, 1.27; 95% CI, 1.07–1.52; P = 7.15 × 10−3) were risk haplotypes; HLA_DQA1*0301-DQB1*0402 (OR 0.30, 95%CI 0.14–0.65, P = 2.36 × 10−3) was protective. Human myosin cross-reactive N-terminal and B repeat epitopes of GAS M5/M6 bind with higher affinity to DQA1/DQB1 alpha/beta dimers for the 2-risk haplotypes than the protective haplotype. Conclusions Variation at HLA_DQA1-DQB1 is the major genetic risk factor for RHD in Aboriginal Australians studied here. Cross-reactive epitopes bind with higher affinity to alpha/beta dimers formed by risk haplotypes, supporting molecular mimicry as the key mechanism of RHD pathogenesis.

Rheumatic fever (RF) and rheumatic heart disease (RHD) are neglected diseases, although RHD remains the most common cardiovascular disease among the young people. This position statement is a declaration of the World Heart Federation Working Group on RF and RHD strategic goal to reduce by 25% the number of premature deaths from RF and RHD among individuals aged <25 years by the year 2025. The Working Group affirms key strategic targets, reviews barriers to RF and RHD control, and identify the actions required to change the trajectory of control for these diseases. In the 21 st century, rheumatic fever (RF) and rheumatic heart disease (RHD) are neglected diseases of marginalized communities. Globally, RHD remains the most-common cardiovascular disease in young people aged <25 years. Although RF and RHD have been almost eradicated in areas with established economies, migration from low-income to high-income settings might be responsible for a new burden of RHD in high-income countries. The World Heart Federation (WHF) and its Working Group on RF and RHD unites global experts, combines their experience and enthusiasm, and provides a platform for RHD control. This paper is a declaration of the WHF institutional strategic goal—a 25% reduction in premature deaths from RF and RHD among individuals aged <25 years by the year 2025. The position statement affirms WHF commitments to five key strategic targets: comprehensive register-based control programmes, global access to benzathine penicillin G, identification and development of public figures as 'RHD champions', expansion of RHD training hubs, and support for vaccine development. In this paper, we also review existing barriers to RF and RHD control and identify the actions required to change the trajectory of control for these diseases. This approach provides the foundation for governments, civil society, patient advocates, clinicians, researchers, and funding agencies to develop partnerships and unify global efforts to control RF and RHD. The WHF plans to expand this position statement to an operational plan that will be founded on science, research, and quantifiable progress indicators to impact positively on the millions of people who are affected by RHD and its long-term sequelae.

Acute Rheumatic Fever and Rheumatic Heart Disease (ARF/RHD) affect over 45 million people globally. ARF/RHD are autoimmune complications following group A streptococcal infections. Current diagnosis of ARF requires thorough medical examination, echocardiography and laboratory tests that are unavailable in most primary care settings where patients with ARF typically first present. This pilot study was conducted to determine whether machine learning-based predictive models could be used to stratify host tissue protein reactive antibodies associated with ARF, that could be incorporated into a lateral flow point-of-care (POC) platform for ARF screening. We investigated serum antibody levels against four host tissue proteins (cardiac myosin, laminin, keratin, and tropomyosin) known to increase in ARF. Serum samples were obtained from: (i) a rat autoimmune valvulitis model (RAV) of RHD (30 streptococcal M protein-injected rats versus 30 controls); and (ii) human samples (25 newly diagnosed ARF patients versus 50 healthy controls). Four machine learning algorithms (logistic regression, decision tree, random forest, and AdaBoost) predicted ARF status using antibody levels detected by enzyme-linked immunosorbent assay (ELISA). Rats injected with streptococcal M protein developed cardiac pathology and demonstrated three-fold higher optical density values for all four host tissue protein reactive antibodies compared to controls. ARF patients showed significantly elevated antibody levels against all host tissue proteins tested ( p  < 0.01). Random forest achieved optimal performance for rat data (sensitivity 100%, specificity 92.5%, AUROC = 0.97), while AdaBoost excelled for human samples using binary biomarkers (sensitivity 85.0%, specificity 82.8%, AUROC = 0.87). We demonstrate the screening potential of known host tissue protein reactive antibodies and propose lateral flow assay POC technology as a possible advancement toward improved early screening for ARF in resource-constrained environments.

This systematic review and meta-analysis aimed to provide a contemporaneous estimate of the global burden of rheumatic heart disease (RHD) from echocardiographic population-based studies. We searched multiple databases between January 01, 1996 and October 17, 2017. Random-effect meta-analysis was used to pool data. We included 82 studies (1,090,792 participant) reporting data on the prevalence of RHD and 9 studies on the evolution of RHD lesions. The pooled prevalence of RHD was 26.1‰ (95%CI 19.2–33.1) and 11.3‰ (95%CI 7.2–16.2) for studies which used the World Heart Federation (WHF) and World Health Organization (WHO) criteria, respectively. The prevalence of RHD varied inversely with the level of a country’s income, was lower with the WHO criteria compared to the WHF criteria, and was lowest in South East Asia. Definite RHD progressed in 7.5% (95% CI 1.5–17.6) of the cases, while 60.7% (95% CI 42.4–77.5) of cases remained stable over the course of follow-up. The proportion of cases borderline RHD who progressed to definite RHD was 11.3% (95% CI 6.9–16.5). The prevalence of RHD across WHO regions remains high. The highest prevalence of RHD was noted among studies which used the WHF diagnostic criteria. Definite RHD tends to progress or remain stable over time.

Acute rheumatic fever (ARF) is an autoimmune disease caused by group A streptococcal infection. Recurrent episodes of ARF can lead to rheumatic heart disease (RHD), which is the leading cause of cardiovascular mortality in children worldwide, especially in low- and middle-income countries. Investigations into the etiology of ARF and RHD constitute a crucial milestone in the advancement of both preventive measures and therapeutic interventions. The purpose of this mini review is to delineate the etiology and pathophysiological mechanisms underlying ARF and RHD. Selective searches were conducted in PubMed to retrieve literature published between 1968 and 2024, employing key terms such as “acute rheumatic fever”, “rheumatic heart disease”, “group A Streptococcus ”, “streptococcal pharyngitis”, “pathogenesis”, and “pathophysiology”. The pathogenesis of infections caused by group A streptococci, and their effects on ARF and RHD, have been thoroughly examined. A central hypothesis is that autoimmune responses are triggered by molecular mimicry, but alternate pathogenic mechanisms are continuously being explored. There is an urgent need for high-quality research that can inform efforts aimed at decreasing the occurrence of ARF and halting the advancement of RHD, which requires researchers to understand its causes and to develop appropriate preventive and therapeutic programs.