Rheumatic heart disease (RHD) is a serious disease of the heart involving damage to one or more of the four small heart valves. Valve damage remains after an illness called acute rheumatic fever (ARF). During ARF the heart valve tissue and the heart lining or muscle can become inflamed, and this is called carditis. Following carditis, the inflamed tissues can be left scarred, resulting in an interruption to normal blood flow. Blood may flow backward across a leaky valve that does not close properly or may not flow forward though a tight valve that does not open properly. Surgery may be required to repair or replace the damaged valve.

Symptoms and signs of RHD

Symptoms of mild RHD may not be noticed for many years.1 When symptoms do develop, they usually depend on which heart valves are affected, and the type and severity of the damage. Many people with RHD have one or more heart murmurs which can be heard through a stethoscope.

Symptoms of RHD can include chest pain, fatigue, breathlessness with physical activity or when lying down, weakness and tiredness, and swelling of the legs. With severe RHD there is a risk of abnormal heart rhythms, stroke, endocarditis, and complications during pregnancy. These complications cause progressive disability, reduce quality of life, and can lead to premature death in young adults. Heart surgery can manage some of these problems and prolong life but does not cure RHD.

More than half of the people who have ARF progress to RHD within 10 years of their initial ARF episode, and more than one-third of these people develop severe RHD.2 Almost a quarter of people with RHD will develop complications including heart failure, abnormal heart rhythm or stroke, or require surgery within eight years of diagnosis.3 

Diagnosing RHD

RHD is diagnosed using an echocardiogram (ultrasound) machine. The World Heart Federation have developed criteria for diagnosing RHD on echocardiogram. This includes a borderline RHD classification. Borderline RHD classification for people aged under 20 years.4 Borderline RHD means that the individual has some but not complete features of RHD and in most cases requires a follow-up echocardiogram and a period of treatment with secondary prophylaxis antibiotics. The severity of RHD follows standard valvular heart disease guidelines and includes mild, moderate or severe valve disease.

Before echocardiography was widely available, RHD was commonly diagnosed using a stethoscope to identify abnormal heart sounds (murmurs). Diagnosis of RHD with echocardiography has been found to be significantly more accurate than using a stethoscope alone.5^,6^,7 

Managing RHD

Medical and surgical management of RHD depends on which heart valve/s have been affected and the severity of damage. Management focuses on reducing symptoms and preventing complications associated with RHD. Broadly, management principles include:8

• culturally safe medical care and support.
• delivery of regular, long-term antibiotic prophylaxis to prevent recurrent ARF.
• notifying the RHD diagnosis to the local Disease Control or Public Health Unit.
• access to medical specialists for routine care.
• access to echocardiography services for regular monitoring.
• access to dental services for routine care.
• access to timely surgery and rehabilitation care.

For more information, see Chapter 8 and Chapter 11 in the RHDAustralia 2020 Australian guidelines for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition).

• 1. Zühlke L, Karthikeyan G, Engel ME, et al. Clinical Outcomes in 3343 Children and Adults with Rheumatic Heart Disease From 14 Low- and Middle-Income Countries: Two-Year Follow-Up of the Global Rheumatic Heart Disease Registry (the REMEDY Study). Circulation. 2016;134(19):1456-1466. View Source
• 2. He VYF, Condon JR, Ralph AP, et al. Long-term outcomes from acute rheumatic fever and rheumatic heart disease: A data-linkage and survival analysis approach. Circulation. 2016;134:222-232. View Source
• 3. Stacey I, Hung J, Cannon J, et al. Long-term outcomes following rheumatic heart disease diagnosis in Australia. European Heart Journal Open. 2021;1(3):oeab035. View Source
• 4. Reményi B, Wilson N, Steer A, et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an evidence-based guideline. Nature Review Cardiology. 2012;9:297-309. View Source
• 5. Carapetis JR, Hardy M, Fakakovikaetau T, et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan school children. Nature Clinical Practice Cardiovascular Medicine. 2008;5:411-417. View Source
• 6. Marijon, E. et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. New England Journal of Medicine. 2007;357:470-476. View Source
• 7. Webb RH, Wilson N, Lennon DR, Wilson EM. Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic. Cardiology in the Young. 2011;21:436-443. View Source
• 8. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.