How are Acute Rheumatic Fever and Rheumatic Heart Disease diagnosed and managed?
Diagnosing Acute Rheumatic Fever
A combination of signs and symptoms which can involve the joints, heart, skin and/or nervous system, plus evidence of a recent group A streptococcal (Strep A) infection, are required to confirm the diagnosis.2
Accurate diagnosis is important because:
- not diagnosing ARF in someone who does have it is a missed opportunity for treatment, and puts the person at increased risk of recurrent ARF and RHD.
- mistakenly diagnosing ARF in someone who does not have it may result in unnecessary treatment, including regular penicillin injections.
Early and accurate diagnosis of acute rheumatic fever is vital to prevent disease progression.
Managing Acute Rheumatic Fever
There is a high risk of heart damage following ARF.3 Therefore, it is strongly recommended that all people suspected or confirmed to have ARF are admitted to hospital under the care of a medical specialist.2
Management of ARF is based on confirming the diagnosis, determining whether the heart is involved using an echocardiogram (ultrasound of the heart), excluding other potential conditions that look like ARF, treating the Strep A infection and relieving symptoms. Ongoing care in hospital includes close monitoring, antibiotics, rest, pain management, relief of other symptoms as required (e.g. medications for Sydenham chorea, if present), and heart failure management and planning for surgery if heart failure is present).2
Other management in hospital includes:
- notifying the ARF illness to the local Disease Control or Public Health Unit.
- providing education about ARF and its management to the patient and family.
- commencing secondary prophylaxis.
- liaising with the patient’s usual health service to establish ongoing care.
Longer term management focuses on preventing recurrent ARF: regular, long-term secondary prophylaxis, prompt treatment for sore throats and skin sores, and preventing the development or progression of, rheumatic heart disease.
Diagnosing Rheumatic Heart Disease
Rheumatic heart disease (RHD) is diagnosed using an echocardiogram (ultrasound) machine. In 2012 the World Heart Federation published an evidence-based guideline for diagnosing RHD on echocardiogram, including classifications for mild, moderate and severe RHD. There is also a borderline RHD classification for people aged under 20 years.4
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 more accurate than using a stethoscope alone.5,6,7
Screening for RHD in populations at high risk can identify people with RHD who do not have symptoms. Screening is also performed using echocardiogram machines.
Managing Rheumatic Heart Disease
Medical and surgical management of RHD depends on which heart valve/s have been affected and the severity of disease. Management focuses on reducing symptoms, and preventing complications associated with RHD. The principles of RHD management are:2
- culturally safe medical care and support.
- regular secondary prophylaxis (penicillin injections) to prevent recurrent ARF.
- reliable access to medical specialists for routine care.
- reliable access to echocardiography services for regular monitoring.
- reliable access to dental services for routine care.
- timely access to surgery and rehabilitation care.
- Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131(20):1806-1818.
- 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
- 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;134:222-322.
- 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.
- 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.
- Marijon, E. et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. New England Journal of Medicine. 2007;357:470-476.
- 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.
LAST UPDATED 23 JUNE 2020