Abstract

Rheumatic heart disease (RHD) constitutes a leading cause of premature death and incapacity in Africa, where it is encountered in younger people, and shows a much faster and more malignant course than that seen in Europe or North America. While it is well established that RHD is a consequence of recurrent, untreated group A β-haemolytic streptococcal infections (GAS), the pathogenesis is incompletely understood, and the variation in natural history and phenotypes are not fully explained. In Africa patients are rarely diagnosed with acute rheumatic fever (ARF). They usually present in the late stages of RHD, with the severe and virulent forms occurring at early ages, therefore leading to high morbidity and mortality in young patients.

Highlights

  • Rheumatic heart disease (RHD) constitutes a leading cause of premature death and incapacity in Africa, where it is encountered in younger people, and shows a much faster and more malignant course than that seen in Europe or North America

  • The global burden of disease caused by rheumatic fever (RF) and rheumatic heart disease (RHD) currently falls disproportionately on children living in the developing world and in marginalised communities where poverty is widespread

  • This is of particular relevance considering that the correct management of group A β-haemolytic streptococcal infections (GAS) pharyngitis and secondary prophylaxis of RF with penicillin prevent the occurrence of RHD

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Summary

Epidemiology of ARF and RHD

The global burden of disease caused by rheumatic fever (RF) and rheumatic heart disease (RHD) currently falls disproportionately on children living in the developing world and in marginalised communities where poverty is widespread. Screening with portable echocardiography has uncovered a large burden of latent RHD among asymptomatic children in endemic regions of Africa,[9,10] the significance of which remains unclear.[11] In marked contrast, there are almost no data on ARF, probably related to low access to healthcare, inadequate resources for diagnosis of throat and skin streptococcal infection, lack of awareness of the importance of correct treatment of bacterial pharyngitis, and overall, to the absence of national prevention and control programmes These usually allow notification of the disease and the institution of long-term secondary prophylaxis to those at risk of developing RHD. Despite being a major cause of premature death and disability, the pathogenesis is still incompletely understood, the natural history is not fully explained, phenotypes have been only partially described, and some aspects of management remain debatable

Pathogenesis of ARF and RHD
Gaps in knowledge and management
The role of genetic studies
Europe Latvia
Key messages
Findings
Conclusion
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