What is acute rheumatic fever?

Acute rheumatic fever (ARF) refers to an autoimmune response to infection of the upper respiratory tract (and possibly of the skin (McDonald, et al 2004)) by group A streptococcus (GAS) bacteria. Not all people who have a streptococcal infection develop ARF but, in those affected, it usually develops within 2–3 weeks after the infection (Webb, 2015). ARF can affect the heart, joints, brain and subcutaneous tissues (Parnaby & Carapetis 2010) and can be extremely painful. While there is no lasting damage caused to the brain, joints or skin, ARF may cause lasting damage to the heart. Hospitalisation is required so all necessary investigations are undertaken. 

The risk of ARF recurrence is relatively high after an initial ARF episode and repeated episodes increase the likelihood of long-term heart valve damage, known as ‘rheumatic heart disease’ (Carapetis et al 2016). As each episode of ARF can worsen the damage to the heart, the priority in disease management is to prevent ARF recurrences using long-acting penicillin treatment, which is known as secondary prophylaxis.

What is rheumatic heart disease?

Rheumatic heart disease (RHD) is caused by damage to heart valves during as a result of ARF. An affected heart valve may become scarred and stiffer, obstructing blood flow (stenosis), or it may fail to close properly, causing blood to flow backwards in the heart instead of forward around the body (regurgitation). Regurgitation due to damage to the mitral valve is the most common symptom of RHD.

Symptoms of RHD include fatigue, chest pain, swelling of legs and face and shortness of breath. These symptoms can make diagnosis difficult as they are also symptoms of other cardiac diseases. The type of valve affected and severity of damage, along with a history of ARF, are important clinical indicators for an RHD diagnosis. Many patients can remain asymptomatic despite having moderate or even severe RHD Untreated RHD can cause arrhythmias (when the heart beats too fast, too slow, or irregularly), stroke, endocarditis (infection of the inner lining of the heart) and complications of pregnancy, and may be fatal.

Management of RHD includes treating symptoms and preventing worsening of disease, which requires regular echocardiography (echo) to identify and monitor which valves are damaged and how badly. Management of an RHD diagnosis is complex and involves coordination of multiple services such as primary health care, secondary prophylaxis with penicillin, monitoring of heart medications such as anticoagulation therapy, oral healthcare services, echo, specialist medical care, and other cardiothoracic and interventional cardiology services (RHD Australia, 2012).