Rheumatic Heart Disease

Rheumatic heart disease (RHD) is a chronic condition caused by permanent damage to the heart valves following rheumatic fever, a complication of untreated or poorly treated group A streptococcal (GAS) infection, often manifesting as strep throat. While rheumatic fever primarily affects children, RHD is the long-term consequence, and it may take years or even decades to develop into a serious health problem. RHD is a leading cause of cardiovascular morbidity and mortality, particularly in low- and middle-income countries, where access to healthcare and antibiotics is limited.

This blog will provide an in-depth understanding of rheumatic heart disease, exploring its causes, symptoms, risk factors, diagnostic methods, treatment options, and strategies for prevention. Additionally, the global impact of the disease and public health interventions to reduce its prevalence will be discussed.

What Is Rheumatic Heart Disease?

Rheumatic heart disease is a condition where the heart valves are permanently damaged due to inflammation caused by rheumatic fever. The damage to the valves, particularly the mitral and aortic valves, can lead to stenosis (narrowing of the valve), regurgitation (leakage of blood backward through the valve), or both. Over time, these valve problems can impair the heart’s ability to function properly, leading to heart failure, arrhythmias, stroke, and other cardiovascular complications.

Rheumatic Heart Disease causes

Rheumatic heart disease develops as a result of rheumatic fever, which is triggered by an immune response to a group A streptococcal infection. When strep throat is left untreated, the immune system produces antibodies to fight the bacteria. In some cases, these antibodies mistakenly attack the body’s own tissues, including the heart, joints, skin, and brain.

The heart valves, particularly the mitral and aortic valves, are most susceptible to this autoimmune attack. The repeated episodes of inflammation that occur during rheumatic fever can cause scarring and thickening of the valves, leading to the characteristic narrowing or leaking associated with RHD.

Rheumatic Heart Disease pathophysiology

The pathophysiology of rheumatic heart disease is closely linked to the immune response triggered by rheumatic fever. When the body mounts an immune response to GAS bacteria, antibodies attack the heart valves due to molecular mimicry, where bacterial proteins resemble those found in human tissues.

The key pathological changes in rheumatic heart disease include:

1. Valve Thickening and Scarring: Repeated episodes of inflammation cause the valve leaflets to thicken and fuse together, leading to narrowing (stenosis) or leaking (regurgitation).
2. Calcification: Over time, calcium deposits can form on the damaged valves, further impairing their function.
3. Chordae Tendineae Damage: The tendinous cords that support the valves can become thickened, shortened, or ruptured, leading to valvular insufficiency.
4. Heart Muscle and Endocardial Involvement: In severe cases, the inflammation may extend to the heart muscle and the lining of the heart chambers, worsening the overall cardiac function.

Rheumatic Heart Disease symptoms

The symptoms of rheumatic heart disease often take years to develop after the initial episode of rheumatic fever. Many individuals may not experience noticeable symptoms until the disease has progressed to a more advanced stage. The severity of symptoms depends on the extent of the damage to the heart valves and the specific valve(s) affected.

Common symptoms of RHD include:

1. Shortness of Breath: This is often the most noticeable symptom, particularly during physical activity or when lying down. It occurs because the damaged heart valves make it difficult for the heart to pump blood efficiently, leading to fluid buildup in the lungs.
2. Chest Pain: Some individuals may experience chest discomfort or pain, especially during exertion. This occurs when the heart struggles to pump blood through narrowed or leaky valves.
3. Fatigue: As the heart becomes less efficient at pumping blood, individuals with RHD may feel more fatigued, even with minimal physical activity.
4. Swelling (Edema): Fluid retention, particularly in the legs, ankles, and feet, can occur due to poor circulation and heart failure.
5. Palpitations: Irregular heartbeats or a racing heart may occur, often as a result of arrhythmias, which are common in individuals with damaged heart valves.
6. Fainting or Dizziness: In severe cases, reduced blood flow to the brain may cause dizziness or fainting spells.

Complications of Rheumatic Heart Disease

Rheumatic heart disease can lead to a range of serious complications, particularly if left untreated. These complications often result from the impaired function of the damaged heart valves.

1. Heart Failure: The heart may become progressively weaker as it struggles to pump blood through narrowed or leaking valves. This can lead to congestive heart failure, a condition where the heart cannot meet the body’s demand for blood and oxygen.
2. Arrhythmias: Individuals with RHD are at increased risk of developing abnormal heart rhythms, such as atrial fibrillation. These arrhythmias can increase the risk of stroke and other complications.
3. Stroke: The formation of blood clots in the heart due to abnormal blood flow through the damaged valves can lead to stroke. If a clot travels to the brain, it can block blood flow, resulting in a stroke.
4. Infective Endocarditis: Damaged heart valves are more susceptible to infection by bacteria or other pathogens, leading to infective endocarditis. This is a serious and potentially life-threatening infection of the heart valves.
5. Pulmonary Hypertension: In cases where the mitral valve is severely damaged, blood flow from the lungs to the heart can be impaired, leading to increased pressure in the pulmonary arteries (pulmonary hypertension). This can cause additional strain on the heart and lungs.

Diagnosis of Rheumatic Heart Disease

Diagnosing rheumatic heart disease requires a combination of medical history, physical examination, and diagnostic tests. Early diagnosis is crucial to prevent the progression of the disease and manage its symptoms effectively.

1. Medical History and Physical Examination: A doctor will review the patient’s history of rheumatic fever or strep throat, as well as any symptoms such as shortness of breath, chest pain, or palpitations. During the physical examination, the doctor may listen for abnormal heart sounds, such as a heart murmur, which can indicate valve damage.
2. Echocardiogram: An echocardiogram is the most important diagnostic tool for rheumatic heart disease. This non-invasive test uses sound waves to create detailed images of the heart, allowing doctors to assess the structure and function of the heart valves. It can show valve thickening, narrowing, or leakage, as well as any impact on the heart’s ability to pump blood.
3. Electrocardiogram (ECG): An ECG measures the electrical activity of the heart and can detect arrhythmias, which are common in individuals with RHD.
4. Chest X-ray: A chest X-ray can reveal signs of heart enlargement or fluid buildup in the lungs, both of which are common in advanced stages of RHD.
5. Blood Tests: Blood tests may be performed to check for evidence of a recent streptococcal infection or inflammation. Elevated levels of certain markers, such as C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), can indicate ongoing inflammation.

Rheumatic Heart Disease treatment

The treatment of rheumatic heart disease focuses on managing symptoms, preventing complications, and addressing the underlying valve damage. The specific treatment plan will depend on the severity of the disease and the extent of valve damage.

1. Medical Management

• Antibiotic Prophylaxis: Long-term antibiotic therapy is essential for preventing recurrent episodes of rheumatic fever and further damage to the heart valves. This usually involves monthly injections of benzathine penicillin or daily oral penicillin. Antibiotic prophylaxis may continue for several years, depending on the severity of the disease and the patient’s risk of recurrence.
• Medications for Heart Failure: In individuals with heart failure, medications such as diuretics, beta-blockers, or ACE inhibitors may be prescribed to help manage symptoms and improve heart function.
• Anticoagulants: If a patient has developed atrial fibrillation or other arrhythmias, anticoagulant medications may be prescribed to reduce the risk of blood clots and stroke.
• Anti-inflammatory Medications: In cases where inflammation is ongoing, corticosteroids or nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to reduce inflammation and relieve symptoms.

2. Surgical Treatment

In more severe cases of rheumatic heart disease, surgical intervention may be necessary to repair or replace the damaged heart valves. Surgery is typically recommended when the valve damage is causing significant symptoms or complications, such as heart failure.

• Valve Repair: In some cases, surgeons may be able to repair the damaged valve, restoring its normal function. This may involve removing scar tissue, reshaping the valve, or inserting a device to support the valve’s structure.
• Valve Replacement: If the damage to the valve is too severe to be repaired, valve replacement surgery may be necessary. This involves removing the damaged valve and replacing it with a mechanical valve or a biological valve (made from animal or human tissue).

Prevention of Rheumatic Heart Disease

Preventing rheumatic heart disease involves both primary and secondary prevention strategies, with a focus on preventing the initial streptococcal infection and recurrent episodes of rheumatic fever.

1. Primary Prevention

The most effective way to prevent rheumatic heart disease is to prevent rheumatic fever by ensuring that streptococcal infections, such as strep throat, are promptly and adequately treated with antibiotics. Public health education on recognizing the signs of strep throat and seeking timely medical treatment is essential, particularly in areas where RHD is prevalent.

2. Secondary Prevention

For individuals who have had rheumatic fever, long-term antibiotic prophylaxis is crucial to prevent recurrent episodes of the disease and further damage to the heart valves. This is particularly important in children and young adults, who are at higher risk of recurrence.

The Global Impact of Rheumatic Heart Disease

Rheumatic heart disease disproportionately affects populations in low- and middle-income countries, where access to healthcare and antibiotics is limited. According to the World Health Organization (WHO), an estimated 40 million people worldwide are affected by RHD, with 233,000 deaths annually. The highest prevalence is seen in sub-Saharan Africa, South Asia, and the Pacific Islands, where poverty, overcrowding, and limited healthcare infrastructure contribute to the persistence of rheumatic fever and RHD.

Rheumatic heart disease remains a significant global health challenge, particularly in developing nations. While the disease is preventable, it continues to cause long-term disability and death, particularly among children and young adults. Early diagnosis and treatment of streptococcal infections, combined with long-term antibiotic prophylaxis for those at risk, are essential to preventing the development of RHD.

Efforts to reduce the global burden of rheumatic heart disease must focus on improving access to healthcare, increasing public awareness, and implementing effective prevention programs. By addressing the underlying social and economic factors that contribute to the disease, we can make significant progress in reducing the impact of this preventable condition on vulnerable populations worldwide.