Acute rheumatic fever (ARF) and rheumatic heart disease (RHD)

ARF is caused by infection with group A Streptococcus

ARF is an abnormal immune response following untreated infection with the group A Streptococcus bacterium in the throat (GAS pharyngitis or ‘strep throat’). There is emerging evidence that group A Streptococcus skin infections (impetigo) may also trigger ARF.

In susceptible people, this abnormal immune response occurs two to three weeks after an untreated group A Streptococcus infection. The immune response can lead to inflammation (swelling) in multiple organs including the joints, heart, brain, and skin, causing the symptoms of ARF.

People can’t catch ARF from someone else because it is an immune response and not an infection. However, group A Streptococcus is spread between people. A person can become infected with group A Streptococcus multiple times over their lifetime, and can experience subsequent episodes of ARF.

Heart damage from ARF leads to RHD

Damage to the heart can persist after a person recovers from ARF. This permanent damage is known as RHD. While RHD may develop after a single bout of ARF, it is typically associated with recurrent or severe episodes.

The most common part of the heart damaged is the valves, although damage to other parts of the heart can also occur. Damaged heart valves can’t open and shut properly. This may mean that the heart can’t pump blood as well as it should.

Complications of RHD include heart failure, which means the heart is unable to pump blood effectively. Other complications of RHD include infection of damaged heart valves (infective endocarditis) and stroke due to clots forming in the heart or on damaged valves.

People affected by ARF and RHD

ARF is more common in children aged 5 to 15 years and is rare in adults, while RHD can be diagnosed in children, adolescents and adults. People who have had ARF in the past are more likely to get it again in the future.

In Australia, ARF and RHD most commonly affect Aboriginal and Torres Strait Islander, Māori, and Pacific Islander people, and migrants from low- and middle-income countries. ARF and RHD can affect anyone but are less common among people who are not part of one of these groups.

Diagnosing and treating ARF

ARF can be undiagnosed and this can cause failure to prevent or recognise RHD. Failure to recognise ARF and limited access to healthcare can contribute to the under-diagnosis of ARF.

Symptoms of ARF can include:

• Fever and fatigue
• Swollen painful joints (arthritis)
• Uncontrollable body movements (chorea)
• Symptoms caused by heart damage, including shortness of breath or racing heartbeat
• Skin changes including painless lumps or rash.

There is no single test for ARF. To diagnose ARF, a doctor will take a history and examine the patient. They may order tests including:

• Throat or skin swabs to look for group A streptococcal infection
• Blood tests to look for signs of inflammation and recent group A strep infection
• Electrocardiogram (ECG) or echocardiography (echo) to check for damage to the heart

Treatment of ARF involves giving antibiotics to treat the responsible group A streptococcal infection. Symptoms can be treated with medicines for pain, fever, and inflammation.

Diagnosing and treating RHD

RHD often does not cause symptoms. When it does, the most common symptoms are shortness of breath and reduced ability to exercise.

RHD is usually diagnosed with an echocardiogram (an ultrasound of the heart which can assess the heart valves and look for heart failure). Doctors will also take a medical history and do a physical examination, and may order additional tests including:

• Chest x-ray – to check for enlargement of the heart or fluid on the lungs
• Electrocardiogram (ECG) – to look for abnormal heart rhythm (arrhythmia)

Treatment of RHD can manage symptoms and prevent complications. Management for someone diagnosed with RHD may involve:

• Regular check-ups with a cardiologist (heart specialist) to monitor the heart
• Surgery to repair or replace damaged heart valves
• Giving medication to treat heart failure, which may involve admission to hospital
• Blood-thinning medicine to prevent stroke after valve replacement, or if an abnormal heart rhythm develops
• Good antenatal care, as pregnancy can make rheumatic heart disease worse
• Regular (preventive) antibiotics to prevent further group A streptococcal infections
• Early treatment of possible group A streptococcal infections
• Vaccinations (influenza and pneumococcal) to prevent other illnesses that may put strain on the heart
• Good dental hygiene (tooth brushing and flossing) and regular dental check-ups, to stop mouth bacteria getting into the blood and causing infection of damaged heart valves
• Antibiotics are given before some dental or surgical procedures to prevent infection of the heart.

ARF and RHD can be prevented

Preventing ARF and RHD is important because these diseases can have long-term or permanent impacts, even if treated.

Risk factors for ARF and RHD include poverty, overcrowding and reduced access to medical care.

ARF can be prevented by:

• Reducing the spread of group A Streptococcus, through improving living conditions for those at risk. This may include reducing household overcrowding and providing “health hardware” (e.g., clean water) for washing hands and bodies.
• Prompt diagnosis and treatment of group A streptococcal throat and skin infections, especially among those at higher risk.

RHD can be prevented by the same strategies, and by giving regular antibiotics (secondary prophylaxis) to people who have had ARF to prevent further group A streptococcal infections.

Where to get help

• Your GP (doctor)
• Cardiologist
• Heart Foundation Tel. 13 11 12
• HeartKids VictoriaTel. 1800432785
• RHD Australia Tel. (08) 8946 8654