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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 is the source of significant morbidity and mortality rates. Rheumatic fever (Chap. 381) 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. 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), Central America, and the Middle East, rheumatic valvular disease progresses more rapidly than in more-developed nations and frequently causes serious symptoms in patients younger than 20 years of age. This accelerated natural history may be due to repeated infections with more virulent strains of rheumatogenic streptococci. Approximately 15 million to 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).

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 with age for both men and women. Important left-sided valve disease may affect as many as 12–13% of adults older than the age of 75. 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).

The incidence of infective endocarditis (Chap. 155) has increased with the aging of the population, the more widespread prevalence of vascular grafts and intracardiac devices, the emergence of more virulent multidrug-resistant microorganisms, and the growing epidemic of diabetes. The more restricted use of antibiotic prophylaxis since 2007 has thus far not been associated with an increase in incidence rates. Infective endocarditis has become a relatively more frequent cause of acute valvular regurgitation.

Bicuspid aortic valve disease affects as many as 0.5–1.4% of the general population, with an associated incidence of aortopathy involving root or ascending aortic aneurysm disease or coarctation. An increasing number of childhood survivors of congenital heart disease present later in life with valvular dysfunction. The global burden of valvular heart disease is expected to progress.

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