Rheumatic Heart Disease in the Twenty-First Century

Abstract

Rheumatic heart disease (RHD) is a chronic valvular disease resulting after severe or repetitive episodes of acute rheumatic fever (ARF), an autoimmune response to group A Streptococcus infection. RHD has been almost eliminated with improved social and health infrastructure in affluent countries while it remains a neglected disease with major cause of morbidity and mortality in many low- and middle-income countries, and resource-limited regions of high-income countries. Despite our evolving understanding of the pathogenesis of RHD, there have not been any significant advances to prevent or halt progression of disease in recent history. Long-term penicillin-based treatment and surgery remain the backbone of a RHD control program in the absence of an effective vaccine. The advent of echocardiographic screening algorithms has improved the accuracy of diagnosing RHD and has shed light on the enormous burden of disease. Encouragingly, this has led to a rekindled commitment from researchers in the most affected countries to advocate and take bold actions to end this disease of social inequality.

Keywords: Acute rheumatic fever; Rheumatic heart disease.

Fig. 1

Rheumatic mitral valve disease on echocardiogram. Still image from an echocardiogram showing rheumatic mitral valve disease from the parasternal long-axis window in mid-diastole. Valve commissural thickening, dilated left atrium, and characteristic “hockey stick” deformity of the anterior mitral valve leaflet are shown