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Clinical manifestations and diagnosis of rheumatic heart disease

Liesl Zühlke, ChB DCH FCPaeds Cert Card MPH FESC FACC PhD
Ferande Peters, MBBCH FCP(SA) FESC FACC FRCP (London)
Section Editor
Patricia A Pellikka, MD, FACC, FAHA, FASE
Deputy Editor
Susan B Yeon, MD, JD, FACC


Rheumatic heart disease (RHD) remains a major cause of cardiovascular disease in developing nations, although the prevalence of RHD has declined sharply in industrialized countries during the last century [1].

This topic will review the clinical manifestations and diagnosis of RHD. The management of RHD and the epidemiology, pathogenesis, diagnosis, treatment, and prevention of acute rheumatic fever are discussed separately. (See "Acute rheumatic fever: Epidemiology and pathogenesis" and "Acute rheumatic fever: Clinical manifestations and diagnosis" and "Acute rheumatic fever: Treatment and prevention".)


RHD is by far the most important form of acquired heart disease in children and young adults living in developing countries (which are inhabited by 80 percent of the world’s population); RHD accounts for approximately 15 percent of all patients with heart failure (HF) in endemic countries [2,3].

A study of RHD cases estimated that in 2015, there were globally 33.4 million cases of RHD, 10.5 million disability-adjusted life-years due to RHD, and 319,400 deaths due to RHD [4]. The global mortality burden of RHD decreased by nearly 50 percent from 1990 to 2015, but the prevalence varied widely among countries and was highest in Oceania, central sub-Saharan Africa, and South Asia. Estimated age-standardized prevalence of RHD in 2015 was 3.4 cases per 100,000 population in nonendemic countries and 444 cases per 100,000 population in endemic countries. Twenty countries with an endemic pattern of RHD had an age-standardized prevalence exceeding 1 percent [4].

RHD is a disease affecting predominantly those living in poverty with inadequate access to health care and unchecked exposure to group A streptococcus [5]. The impact of socioeconomic status is illustrated by a study from Kinshasa where the prevalence based on clinical examination was 22.2 per 1000 among children who lived in slums but only 4 per 1000 among children attending the city schools [6]. A later report outlined the increased risk of RHD in association with overcrowding and unemployment as well as overcrowding and distance from the nearest health center [7]. The importance of socioeconomic factors is further underscored by the virtual disappearance of RHD in industrialized countries since the mid-20th century, which started well before the introduction of penicillin. By contrast, RHD is still endemic in Africa, Asia, South America, and developing communities of Australasia [4,8-12].

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Literature review current through: Dec 2017. | This topic last updated: Dec 13, 2017.
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