Rheumatic heart disease (RHD) is the leading acquired heart condition in individuals under the age of 25.¹ RHD results from the damage of heart valves caused by rheumatic fever (RF). Globally, between 222,000 and 353,000 deaths annually are attributed to RHD.²

A sore throat might seem minor at first, but it can quickly spread from person to person and, if left undetected, may lead to serious heart complications. RHD has been eliminated with improved social and health services in developed countries, however, it remains a major issue in developing, low-income countries.³ 

Understanding this condition is crucial for anyone concerned about heart health. This article aims to educate you on what RF is, how it progresses to RHD, and the steps you can take to manage and prevent these conditions.

What is rheumatic heart disease?

RHD is a long-term condition resulting from untreated or repeated RF. RF is an autoimmune condition. Let's break down this word - autoimmune. The immune system is the body's natural defence system which protects it from harmful agents. In some cases, the reaction of the immune system can be abnormal, where the host's immune system gets confused and starts attacking the body’s own tissue. Therefore an autoimmune condition is the one where the immune system attacks oneself. This results in swelling known as inflammation that can affect many of the tissues of our body, including the heart, joints, brain, and skin. The resulting damage to the heart leads to serious health problems over time.

RF mostly affects children and adolescents in low- and middle-income countries where access to medical care is limited. People living in overcrowded and poor conditions are at greatest risk of developing the disease due to its infectious nature.⁴

Symptoms

• Chest pain/ discomfort
• Shortness of breath
• Tiredness
• Swelling of the limbs
• Rapid or irregular heartbeat

Understanding rheumatic fever

RF results from Strep throat or scarlet fever. Strep throat and scarlet fever are caused by an infection with streptococcus bacteria. It usually appears several weeks after the initial infection. The immune system is responsible for defending the body against harmful organisms such as bacteria. It mounts a defence by mobilising various cells and producing various substances that target disease-causing elements. In this case, RF is an autoimmune response to streptococcus bacteria.    

Symptoms of rheumatic fever

• General symptoms including high temperature, tiredness, and headache
• Joint pain is the most common feature, commonly in the ankles, knees, elbows and wrists⁵
• Heart inflammation
• Painless, palpable masses under the skin known as nodules
• Rash consisting of pink rings with a clear centre
• Involuntary movement of the face, hands or feet is known as chorea

How rheumatic fever affects the heart

If untreated, RF can cause severe inflammation of the heart, known as carditis. The progression from RF to RHD occurs when heart valve damage develops over the years or through multiple episodes of RF. Even in the early stages of RF, severe carditis or valve damage can lead to changes in the heart muscle and impair the heart's pumping ability. This inability of the heart to effectively pump blood throughout the body is known as heart failure.

Mitral valve disease

The heart valves are like little doors that open and close to regulate blood flow through the heart. When they are damaged, they may not open or close properly, which can lead to problems with blood flow and make the heart work harder. The mitral valve is one of four valves in the heart. It is located between the left side of the heart and plays a crucial role in ensuring that blood flows in one direction through the heart. This process ensures efficient circulation of oxygen-rich blood throughout the body.

Mitral valve disease refers to any dysfunction of the mitral valve. There are two main types:

• Mitral Stenosis - narrowing of the mitral valve, restricting blood flow

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• Mitral Regurgitation - backwards flow of blood when the valve does not close properly

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If RF happens repeatedly or severely, it can cause long-term damage to the heart valves. The mitral valve is especially affected. The long-term inflammation and scarring lead to stenosis and regurgitation.

Diagnosis and treatment options

Diagnostic procedures

All patients that have been diagnosed with RF should undergo cardiac evaluation. The common diagnostic tools used are x-rays, echocardiograms, and electrocardiogram.

• Chest x-ray

X-rays are basic imaging modes used to differentiate the causes of chest pain and shortness of breath. From the image, we can detect changes in the size of the heart and congestion of the lungs due to the irregular flow of blood.

• Echocardiogram

An echocardiogram is an ultrasound image of the heart that provides detailed images of the heart valves and chambers. It is a quick assessment method to detect abnormal changes in the function of the valves and the direction of blood flow within the heart. 

• Electrocardiogram

An electrocardiogram is a fundamental cardiac assessment tool that measures the electrical activity of the heart. It can be used to reveal irregular heart rhythms due to inflammation of the cardiac tissue.

Medical treatments

Medical treatment is focused on managing the symptoms of RHD and preventing recurrent infections by streptococcus.

• Antibiotics

Antibiotics are medications that target and kill bacteria within the body. Penicillin is most commonly used for streptococcal infections. It is extremely important that the course of antibiotics is completed in order to fully eradicate the bacteria from the body, otherwise, there is a risk the bacteria may continue to survive and cause another infection. 

• Anti-inflammatory medications

Reducing the inflammation is essential for controlling the symptoms of carditis and arthritis. Common anti-inflammatory drugs like ibuprofen are used, however, for more severe symptoms, stronger drugs can be used.

• Diuretics

Best known as “pee” drugs, diuretics reduce the amount of fluid that is circulating around the body by increasing the output of urine. This in turn helps to reduce the load on the heart and assists in more efficient functioning of the heart. 

• Heart failure medications

This group of drugs works by reducing stress within the circulating system and the rate at which the heart pumps the blood. Again, this reduces the workload on the damaged heart and allows it to function with more efficiency. Drugs in this group include beta-blockers and ACE inhibitors.

Surgical interventions

Surgical management is often the method of choice for patients suffering from long-term RHD. The interventions include valve repair or valve replacement. Valve repair is often preferred as it is less invasive and does not require the patient to take long-term anticoagulation therapy (blood thinners). Valve replacement replaces the faulty valve with either a biological or mechanical valve. Patients who require a valve replacement will also have to take long-term blood thinning medication to prevent the formation of blood clots. 

Preventative measures 

The best measures for preventing RHD are to eliminate ongoing infections by streptococcus, timely treatment of RF and prevent recurrent infections. Correct diagnosis of strep throat by detection of the bacteria from swabs or the detection of molecules from the bacteria within the blood allows for prompt treatment and reduction in the risk of developing rheumatic fever.5 This involves a course of penicillin. Once diagnosed with RF the patient is recommended to intramuscular injections of penicillin every 28 days in order to reduce the progression of the disease and prevent reinfections.4 The duration of the treatment is dependent on several factors such as age, and severity of disease. 

Living with rheumatic heart disease and mitral valve disease

Living with RHD doesn't have to be a burden. Simple lifestyle adjustments such as maintaining a healthy diet, exercising regularly, avoiding smoking, and managing stress can have a positive impact on everyday health. 

Attending regular medical check-ups will help to monitor the progression and effectiveness of the treatment. Simple techniques such as ECG and echocardiogram require little time to do and involve minimal discomfort to the patient but reveal a breadth of crucial information on the functioning of the heart. These check-ups are also opportunities to express concerns and gain further information about your condition by talking with trained professionals.

Having a strong support system, including healthcare providers, family, and community resources, can help manage the emotional and physical challenges of living with RHD and mitral valve disease.

Summary

Rheumatic heart disease is a significant health concern, especially in overcrowded, low-income countries. This condition is caused by damage to the heart valves from untreated or repeated episodes of Rheumatic fever. Recognizing RHD symptoms such as chest pain, shortness of breath, and fatigue is crucial. Effective management includes antibiotics, anti-inflammatory medications, and, in severe cases, surgical interventions. Preventive strategies, such as prompt treatment of strep throat and regular medical check-ups are vital. With the right care and lifestyle adjustments, individuals with RHD can lead healthy lives. Stay informed and proactive in your heart health to effectively manage and prevent RHD.