Rheumatic heart disease is the injury of heart valves due to rheumatic fever. Rheumatic fever is an autoimmune response to untreated streptococcal infection that may affect the heart, joints, brain, and subcutaneous tissue. The causing organism of rheumatic fever may be group A streptococcus (GAS) or streptococcus pyrogens.
The most common age of rheumatic fever is from 5-15 years old, but it may rarely occur at any age. Rheumatic heart disease causes the death of 300000 people every year. We can see this disease more in developing countries due to the lack of antibiotics and proper health care. Your child is at risk of this disease in an unhealthy environment or with repeated streptococcal throat infections.
Rheumatic fever occurs due to the similarity between the streptococcus antigen and the antigens of the heart, joints, and other connective tissues. So, the antibodies that your body produces against streptococcus attack the heart and other tissues.
Rheumatic fever causes exudative or proliferative lesions. Exudative lesions affect the serous membranes like the pericardium and pleura and heal without fibrosis. Proliferative lesions affect the heart and skin and heal by fibrosis. Mitral valve fibrosis occurs in 90% of cases, but fibrosis can also involve the aortic valve. The fibrosis may lead to stenosis or regurge these valves.
The symptoms of rheumatic fever take from 2-3 weeks to develop. Your child may feel fever, fatigue, painful joints, painless nodule, and (or) chorea. Rheumatic fever damages the heart valves. So, your child may complain of chest pain, shortness of breath, fatigue, and irregular heartbeat. In severe cases, your child may develop heart failure, arrhythmia, and atrial fibrillation, which may be fatal.
The doctor can diagnose your child depending on some factors. He will ask about the symptoms that make him suspect the disease. At first, your doctor diagnoses rheumatic fever through two criteria: major and minor criteria. Major criteria are carditis, arthritis, subcutaneous nodule, chorea, and erythema marginatum. Minor criteria include fever, elevated ESR, arthralgia, and elevated C-reactive protein. Then, your doctor will ask about some tests to check heart health. These tests like echocardiogram, and electrocardiogram (ECG).
There is no medical treatment for rheumatic heart disease because the injury of heart valves becomes permanent. So, the only cure is surgical intervention according to the severity of the damage. Your doctor will repair the heart valve if he can. But, he may need to replace them if the damage is very severe. Your doctor may use medications only to relieve the symptoms and decrease the risk of blood clots formation.
You can avoid rheumatic heart disease only through the prevention of rheumatic fever. You or your child should take proper antibiotics when you feel any symptoms of streptococcal infection. Finally, you should know that rheumatic heart disease is dangerous in pregnant women due to a lack of blood supply.
Now, let’s discuss this disease in more detail and answer the following questions:
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- What causes this disease, and who is at risk?
- What is the pathophysiology of it?
- How many people do have rheumatic heart disease?
- What are its symptoms, signs, and complications?
- How will your doctor diagnose and confirm this disease?
- How can your doctor manage this disease, and what are the treatment options?
- Can you prevent rheumatic heart disease, and how can you do this?
- Rheumatic heart disease in pregnant women
- What is the prognosis?
Causes of rheumatic heart disease
Rheumatic fever is the leading cause of rheumatic heart disease. Recurrent streptococcal infections lead to permanent damage of the heart valves. So, we should know the cause of rheumatic fever that leads directly to rheumatic heart disease. There are many theories, the most recent and acceptable is antigenic similarity.
The antigens of streptococcus are similar to that of the heart tissues. So, the antibodies that your body produces against these bacteria may also attack the heart tissues. The other theory states that the toxins of streptococcus may injure the heart tissue and make it abnormal, so your body attacks it. In all cases, the fibrotic lesions narrow the heart valves, and your heart can’t supply your body with enough blood.
Risk factors of rheumatic heart disease
1-Age:
The most common age of rheumatic fever is from 5 to 15 years old. So, if your child passed this age without having rheumatic fever, he (she) would be at a lower risk of rheumatic heart disease.
2-Sex:
Although rheumatic fever is equal in males and females, rheumatic heart disease occurs in females more than males. The ratio is 2:1 between females and males -who have this disease-.
3- Environment:
Rheumatic heart disease is widespread in developing countries and low socioeconomic people due to a lack of hygiene and health care.
4-Health care:
If your child didn’t have proper antibiotics for streptococcal infection, he (she) would be at a higher risk.
Pathophysiology of rheumatic heart disease
After your child has the GAS infection of the pharynx, his (her) body releases neutrophils and macrophages to phagocyte the organism. The body recognizes the antigen of GAS and presents it to T cells. B and T cells release antibodies (IgM and IgG) against these antigens.
This immune reaction may trigger an autoimmune response against the body tissues. The antibodies bind to the antigen of body tissues, which leads to inflammation of the heart, brain, joint, and subcutaneous tissue. The antibody may bind to basal ganglia, causing chorea. Also, it binds to keratin (of the skin), causing subcutaneous nodules.
The antibodies bind to the heart tissue, causing inflammation of the myocardium and permanent injury of heart valves. The T cells infiltrate the valves, which damages the endothelium and causes remodeling of the valvular structure. Destruction of the valves causes collagen release and deposition, which accelerates their injury.
How many people have rheumatic heart disease?
About 33 million people have rheumatic heart disease right now, and most of them are children. More than 9 million have disabilities from rheumatic heart disease.
Unfortunately, more than 300000 people die from this disease every year.
Symptoms of rheumatic heart disease
A) Rheumatic fever:
At first, you should know the symptoms of rheumatic fever because it is the principal cause of rheumatic heart disease. You may any of the following:
1- Carditis
means the inflammation of the layers of the heart wall. Pericarditis causes pericardial effusion and stitching pain, while myocarditis causes tachycardia and heart failure. Endocarditis causes stenosis or regurge of the valves.
2-Arthritis (joint inflammation)
It affects multiple large joints. You may have hotness, redness, swelling, and tenderness at the affected joints.
3-Subcutaneous nodule
It is a painless and firm nodule, which presents over bony prominences.
4-Chorea
means irregular and involuntary movements that start to appear after streptococcal infection.
5-Erythema marginatum
A skin rash affects the trunk and limbs.
B) Symptoms of rheumatic heart disease
The fibrosis of the valves may cause stenosis or regurge according to the degree of fibrosis.
1-Mitral stenosis:
The mitral valve becomes narrow through calcification, fibrosis, or fusion of its cusps. The stenosis prevents the blood flow from the left atrium to the left ventricle, which increases the pressure in the left atrium, leading to pulmonary congestion. Also, mitral stenosis decreases cardiac output, which is the amount of blood that your heart pumps every minute. If you have mitral stenosis, you may have:
- Pulmonary congestion:
- It is the accumulation of fluids in the lung, which may lead to dyspnea (shortness of breath), exertional cough, chest infection, hemoptysis (coughing blood), pleural effusion (fluids in the pleura), and pulmonary edema.
- Low cardiac output:
- Low blood supply to your body may make you feel with headaches, syncope (temporary loss of consciousness), cardiac ischemia, oliguria (urine output less than 400ml per day), fatigue, cyanosis (bluish skin), hypotension, and weak pulse.
2- Mitral regurge:
It means incomplete closure of the mitral valve, so the blood return from the left ventricle to the left atrium. Mitral regurge increases the pressure in the left atrium during systole (the time of ventricular contraction), which leads to pulmonary congestion then pulmonary hypertension. While during diastole, the blood flow through the mitral valve increases and leads to left ventricle enlargement then left side heart failure. If you have mitral regurge, you may have:
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- Pulmonary congestion
- Pulmonary hypertension: is high pulmonary arterial blood pressure that may lead to right-side heart failure.
- Acute pulmonary edema: is a sudden accumulation of fluids in the lung. You may have shortness of breath, sweating, cyanosis, and cough.
3-Aortic stenosis:
The aortic valve narrows through fibrosis, calcification, or cardiomyopathy. Aortic stenosis obstructs the left ventricle outflow, which increases the pressure in the left ventricle, leading to left ventricular hypertrophy. Aortic stenosis may be asymptomatic for many years, and the symptoms appear suddenly. Also, obstruction of the left ventricular outflow decreases the cardiac output. If you have aortic stenosis, you may have:
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- Low cardiac output
- syncope (temporary unconscious)
- Angina: it is chest pain due to decrease blood supply to your heart. You may complain of shortness of breath, sweating, and dizziness.
4- Aortic regurge:
It means incomplete closure of the aortic valve, so the blood return from the aorta to the left ventricle. Aortic regurge increases the volume overload on the left ventricle, which leads to left ventricular enlargement; left-side heart failure may occur.
Aortic regurge decreases the blood supply to the heart, which may cause angina. Also, large blood volume in the left ventricle causes high systolic blood pressure. You can feel your heartbeat during left ventricular contraction.
5-Tricuspid stenosis:
The tricuspid valve narrows through fibrosis. Tricuspid stenosis prevents blood flow from the right atrium to the right ventricle. Tricuspid stenosis usually occurs with mitral or aortic stenosis, not alone. Tricuspid stenosis increases the pressure in the right atrium, which leads to the right atrium enlargement, and systemic congestion may occur.
Tricuspid stenosis decreases RV filling, which reduces cardiac output. Right atrium enlargement may lead to tricuspid regurgitation. If you have tricuspid stenosis, you may have:
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- Low cardiac output
- Systemic congestion: it is the accumulation of fluids outside the lung. You may have sweating, congested neck vein, enlarged liver, edema, and pleural effusion.
Complications of rheumatic heart disease
In severe cases, you may develop serious complications, as follows:
1- Heart failure:
Your heart can’t pump enough blood to meet your body’s demands. The affected valve determines the side of heart failure. The Mitral valve affects the right side, while the aortic valve affects the left. Right-side heart failure causes systemic congestion, while the left side causes pulmonary congestion.
2- Arrhythmia:
You can feel irregular heartbeats. Healthy people don’t feel their heartbeat because it’s regular all over the time. There are many subtypes of arrhythmia, but the primary types are tachycardia and bradycardia. Tachycardia means a rapid heart rate (more than 100 beats per minute), while bradycardia means a heart rate slower than 60 beats per minute. You may feel shortness of breath, fluttering, fatigue, fast or slow heartbeats, sweating, and anxiety.
3- Stroke
Is a block of blood vessels that supply the brain, which leads to the death of the brain cells. Stroke is an extreme emergency condition, so early management reduces the possibility of extensive brain cells death. You may have problems with speech, vision, and walking. Also, you may have a headache and paralysis of one side of the body.
4- Left atrium enlargement:
You may have cough and dyspnea due to lung compression by the enlarged left atrium. Also, an enlarged left atrium compresses the esophagus and left recurrent laryngeal nerve, causing dysphagia and hoarseness of voice.
5-Infective endocarditis:
It is inflammation of your heart valves due to infection. Infective endocarditis occurs in all valves, but it is rare with mitral stenosis. You may have a fever, pallor, night sweat, fatigue, weight loss, and enlarged tender spleen. Also, you may notice blood in your urine and swelling on your legs.
Diagnosis of rheumatic heart disease
Your doctor diagnoses you depending on some factors. Your doctor will ask you about the symptoms that may make him suspect the disease. Also, your doctor looks for rheumatic fever because it is the direct cause of rheumatic heart disease. You should have one major and two minor criteria to confirm your diagnosis. Your doctor will ask you to do some investigations:
1- Blood tests: usually, the patient with rheumatic heart disease has a streptococcal infection, so the blood picture show leukocytosis, elevated ESR, and elevated C-reactive protein.
2- Echocardiogram: Your doctor uses sound waves to check the heart valves. Echocardiogram show enlargement of heart chambers and damage of heart valves.
3- Electrocardiogram (ECG): Your doctor uses ECG to detect the electrical activity of your heart. ECG shows an irregular rhythm and damage to heart valves. Also, ECG can show chamber enlargement and pulmonary hypertension.
4- Chest X-Ray: your doctor uses the X-Ray to check the lung and heart. The X-Ray shows pulmonary congestion and chamber enlargement.
Treatment of rheumatic heart disease
There is no medical treatment for rheumatic heart disease because the damage of heart valves is permanent. Your doctor uses medication only to manage the symptoms.
A) Surgical treatment:
1- Stenosis: your doctor tries to reopen the valve and make it at a healthy range. Your doctor will repair or replace the valve according to the severity of the damage. The first choice for your doctor is valve repair because it has a low risk of infection and saves the strength of the valve.
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- Balloon valvuloplasty: your doctor applies a catheter with a balloon under X-ray guidance to the narrow valve, then inflates the balloon to widen the opening of the valve and cut the fibrosis. Then, your doctor deflates the balloon and removes the catheter.
- Valvotomy: your doctor makes incisions at the edges of the leaflets of the valves to relieve the fibrosis.
- Valve replacement: If your doctor can’t repair the valve, he will replace your damaged valve. Your doctor uses tissue or synthetic valve.
2- Regurge: your doctor tries to restore the healthy range of the valve through valve repair or valve replacement.
B) Medical treatment:
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- Diuretics: your doctor prescribes diuretics to remove excess fluids that result from pulmonary congestion. Diuretics are like hydrochlorothiazide and Spironolactone.
- Anticoagulants: your doctor prescribes anticoagulants to decrease the risk of pulmonary embolism. Anticoagulants are like warfarin and aspirin.
- Antibiotics: your doctor prescribes antibiotics to treat the associated infection like infective endocarditis. Antibiotics are like penicillin G and gentamycin.
How can you prevent rheumatic heart disease?
You can avoid rheumatic heart disease through the prevention of the leading cause.
Prevention of rheumatic fever:
1. Primary prevention we can prevent acute rheumatic fever by treating the upper respiratory tract infection well. We should do a tonsillectomy for chronic tonsillitis.
2. Secondary preventionaims to prevent recurrent rheumatic fever through the following antibiotics:
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- Long-acting penicillin (benzathine penicillin)
- Penicillin V (ospen)
- Erythromycin if you have an allergy to penicillin
3. Tertiary preventionfocuses on prophylaxis against infective endocarditis through antibiotics like amoxicillin, ampicillin, or cefotaxime.
Rheumatic heart disease in pregnant women
Your blood demands increase during pregnancy. On the other side, rheumatic heart disease decreases the circulating blood volume (cardiac output). So, rheumatic heart disease is a life-threatening condition for a pregnant woman. Also, the fetus may die from this.
Prognosis of rheumatic heart disease
If the patients received good management, they could live healthy life without problems. Early management reduces the risk of heart failure. Finally, you should know that rheumatic heart diseases are permanent, and you need health care for the rest of your life.