Epidemiology

Rheumatic heart disease (RHD) is by far the most common cause of tricuspid stenosis (TS) in the US and throughout the world. In developed countries, the relative incidence of acute rheumatic fever (ARF) significantly decreased over the 20th century; however, the burden of ARF and RHD remains high in developing countries.[6]​​ ​The Global Burden of Disease study estimated 38.0 to 40.8 million cases of RHD globally in 2017, with the highest prevalence, disability, and mortality in Oceania, South Asia, and sub-Saharan Africa. The prevalence ranged from 3.4 cases per 100,000 population in non-endemic countries to >1000 cases per 100,000 in endemic countries, with endemic defined as greater than 0.15 deaths per 100,000 population among children aged 5-9 years.[7]​ Socio-economic and environmental factors such as shortage of resources, lack of healthcare providers and low level of awareness all contribute to the continued high incidence of ARF and RHD in developing countries. In developed countries such as the US, it is estimated that up to 50% of those with a history of ARF and carditis will go on to have chronic RHD.[8][9]​​

Isolated tricuspid valve involvement is extremely rare and clinical TS is usually discovered concomitantly with mitral and often aortic valve disease.[3][10][11]​ The overall incidence of TS in patients with RHD has been estimated at 9% to 15%, with clinically significant and more severe forms occurring in only 5%.[3][12][13][14][15]​ This results in an overall estimated prevalence of rheumatic TS in the US of 0.003 per 1000 population.

The incidence of ARF is equal between males and females; however, females tend to develop significant RHD more commonly.[16][17]​​ The only race in the US that has been shown to develop ARF and RHD at relatively higher rates are those of Polynesian descent living in Hawaii.

Carcinoid heart disease occurs in 40% to 50% of patients with carcinoid syndrome and typically leads to tricuspid and pulmonary valve dysfunction.​[18][19]

The incidence of congenital TS is rare, occurring in <0.1% of the population.

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