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image Primary valvular heart disease ranks well below coronary heart disease, stroke, hypertension, obesity, and diabetes as a major threat to the public health. Nevertheless, it can cause significant morbidity and lead to premature death. Rheumatic fever (Chap. 352) is the dominant cause of valvular heart disease in developing and low-income countries. Its prevalence has been estimated to range from as low as 1 per 100,000 school-age children in Costa Rica to as high as 150 per 100,000 in China (Fig. 256-1). Rheumatic heart disease accounts for 12–65% of hospital admissions related to cardiovascular disease and 2–10% of hospital discharges in some developing countries. Prevalence and mortality rates vary among communities even within the same country as a function of overcrowding and the availability of medical resources and population-wide programs for detection and treatment of group A streptococcal pharyngitis. In economically deprived areas, tropical and subtropical climates (particularly on the Indian subcontinent and in Southeast Asia), Central America, and the Middle East, rheumatic valvular disease progresses more rapidly than in more-developed nations and frequently causes serious symptoms in patients aged <20 years. This accelerated natural history may be due to repeated infections with more virulent strains of rheumatogenic streptococci. Approximately 15–20 million people live with rheumatic heart disease worldwide, an estimated prevalence characterized by 300,000 new cases and 233,000 case fatalities per year, with the highest mortality rates reported from Southeast Asia (~7.6 per 100,000). In the United States, rheumatic heart disease accounted for 20,000 hospital admissions in 2010 and 3281 deaths in 2014.

FIGURE 256-1

The global burden of rheumatic heart disease. This world map provides a snapshot of both the change in prevalence of rheumatic heart disease cases between 1990 and 2013 (upper right legend) and the estimated number of rheumatic heart disease cases per country (lower right legend). Regions in which the disease is highly prevalent include sub-Saharan Africa, India, China, and Southeast Asia. (From JR Carapetis et al: Nat Rev Dis Primers 2:15084, 2016.)

Although there have been recent reports of isolated outbreaks of streptococcal infection in North America, valve disease in high-income countries is dominated by degenerative or inflammatory processes that lead to valve thickening, calcification, and dysfunction. The prevalence of valvular heart disease increases significantly with age for both men and women. Important left-sided valve disease may affect as many as 12–13% of adults aged >75 years (Fig. 256-2). Severe aortic stenosis (AS) is estimated to affect 3.5% of the population aged >75 years. In the United States, there were 85,000 hospital discharges with valvular heart disease in 2010, and the vast majority of these were related to surgical procedures for heart valve disease (mostly involving the aortic and mitral valves).

FIGURE 256-2

The burden of moderate or severe mitral and aortic valve disease in the ...

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