Rheumatic endocarditis (postinfectious endocarditis) is an inflammation of the inner lining of the heart caused by the body’s autoimmune response to certain streptococci. Most commonly, rheumatic endocarditis affects children and adolescents, and is rare in industrialized countries today.
What is rheumatic endocarditis?
Rheumatic endocarditis is an inflammatory change of the inner lining of the heart (endocardium) caused by immunologic dysregulation of the organism (autoimmune reaction) after infection with group A beta-hemolytic streptococci and belongs to the symptomatic spectrum of rheumatic fever. In most cases, mitral and aortic valves, especially the mechanically more stressed valve margins, are affected by rheumatic endocarditis. Damage to the heart valves is a late consequence of rheumatic endocarditis. In this regard, adolescents and children, especially those between the ages of 5 and 17, primarily develop rheumatic endocarditis after a tonsillitis or pharyngitis caused by streptococci.
Causes
Rheumatic endocarditis (postinfectious endocarditis) is due to dysregulation of the body’s immune system as a result of infection with group A beta-hemolytic streptococci. Group A beta-hemolytic streptococci primarily cause inflammatory diseases of the pharynx such as tonsillitis (inflammation of the tonsils), pharyngitis (inflammation of the throat), scarlatina (scarlet fever) or otitis media (inflammation of the middle ear), and in some cases skin infections such as erysipelas (erysipelas) or pyoderma (purulent infection of the skin layers). Rheumatic endocarditis is not due to streptococcal colonization, but to an autoimmune reaction of the organism. The latter forms so-called antibodies against specific protein components of the bacteria, which, among other things, resemble the proteins on the surface of the endocardial cells. Since the antibodies also mistakenly react to endocardial structures (especially the heart valve), inflammatory changes occur, a rheumatic endocarditis, through which the heart valves can thicken, roughen as well as stiffen and ultimately become limited in their ability to function.
Symptoms, complaints, and signs
Rheumatic endocarditis is a symptom of rheumatic fever that can develop as a result of streptococcal infection. It usually takes two to three weeks for the first signs of inflammation of the inner lining of the heart to appear. There is palpitations (tachycardia) and cardiac arrhythmias (arrhythmia). The misdirected antibodies attach to the heart and trigger various reactions in the connective tissue, causing the valves of the heart to thicken and the inner lining to roughen. This changes the heart murmurs. Pain in the heart region and protruding neck veins are also possible. Because the heart no longer pumps adequately due to the inflamed inner skin, shortness of breath and decreased exercise capacity may occur. Often the valve leaflets stick together due to the inflammation and contract. As a result, they no longer close properly and lose their effect as a valve; or they no longer open wide enough, reducing blood flow from one ventricle to the other. Since rheumatic endocarditis occurs in the context of rheumatic fever, all the symptoms of this disease also present themselves. The typical symptoms are fever and a general feeling of illness. The joints are inflamed and painful, and the overlying skin is red and swollen. It usually starts in one joint and spreads to others. In addition, there are so-called rheumatoid nodules and red-spotted skin lesions.
Diagnosis and course
An initial suspicion in rheumatoid endocarditis (postinfectious endocarditis) is based on a previous infection with group A beta-hemolytic streptococci and characteristic symptoms such as abnormal heart murmurs, high fever, tachycardia (increased heart rate), general feeling of illness, and polyarthritis (joint pain) with marked pain on contact and shortness of breath. The diagnosis of rheumatic endocarditis is confirmed by an echocardiogram (ultrasound of the heart) and an ECG, which can be used to detect changes in the heart valves, cardiac insufficiency or cardiac arrhythmias.Blood analysis can detect the antibodies formed in the blood. An elevated CPR value, an increased concentration of white blood cells in the blood and an accelerated blood cell count (ESR) also indicate rheumatic endocarditis. After initiation of therapy, rheumatic endocarditis usually resolves after 6 weeks (75 percent) or 3 months (90 percent), although the course may be prolonged if there is extensive valvular involvement. If rheumatic endocarditis is left untreated, there is a 50 percent chance of recurrence with rheumatic endocarditis, which is also the most common trigger of mitral valve stenosis.
Complications
Rheumatic endocarditis can lead to malfunction of the heart valves. This increases the risk for serious cardiovascular problems and heart attacks. The scarring changes in the heart valves permanently reduce cardiac function, thereby promoting heart failure. The most serious complication of endocarditis is the spread of rheumatic fever to other regions and organs. This can lead to secondary diseases such as acute polyarthritis and chorea minor. In a severe course, multiple organ failure with a fatal outcome is possible. If the inflammation is treated early, no major complications usually occur. However, antibiotics and anti-inflammatory drugs are not free of side effects. Appropriate preparations can cause headaches, muscle and limb pain, skin irritation and gastrointestinal complaints. Allergies and symptoms of intolerance may also occur. If cortisone is administered, this can lead to an increase in blood fat, blood pressure and blood sugar. Possible late effects are osteoporosis or the so-called Cushing’s syndrome. Heart surgery is always risky and can cause complications such as bleeding, heart rhythm disturbances and heart failure. Inflammation of the heart is life-threatening and is a medical emergency accompanied by other symptoms.
When should you see a doctor?
With any form of endocarditis, it is important to see a doctor quickly. Because without treatment, it can only worsen. Even at the first symptoms, therefore, a visit to the general practitioner is advisable. A general practitioner will recognize the signs of post-infectious endocarditis. He can distinguish the heart inflammation from other diseases with similar symptoms. If there is a high fever, he may already prescribe antibiotics. In addition to a general examination, the general practitioner will perform the first specific tests. If the results indicate endocarditis, then he will refer the patient to a heart specialist (cardiologist) as an urgent case. He or she will perform more detailed tests and begin targeted treatment as soon as possible. Unrecognized or untreated, the disease is often fatal, because there is an acute risk of heart attack. Stroke, pulmonary embolism or renal embolism can also result from untreated cardiac inflammation. If post-infectious endocarditis persists for too long, the heart valves can suffer permanent damage. In this case, surgery becomes necessary.
Treatment and therapy
Rheumatic endocarditis is treated primarily as part of antibiotic therapy (penicillin, but also macrolides) to kill any bacteria that may still remain in the organism. In parallel, the rheumatic symptoms are treated with pain-relieving and anti-inflammatory drugs such as acetylsalicylic acid, while at the same time sparing the body, especially the heart. If rheumatic endocarditis is severe, glucocorticoids and immunosuppressants are also used to reduce the overreaction of the immune system. If rheumatic endocarditis leads to more severe heart valve damage due to the inflammatory changes, surgical intervention (valve replacement) may be required. In addition, after rheumatic endocarditis, antibiotic therapy is continued prophylactically as part of long-term therapy (usually monthly antibiotic injections) for the following five years. After rheumatic endocarditis has subsided, a thorough cardiological examination should be performed in order to exclude possible damage to the heart valves or to be able to treat it at an early stage. To prevent further inflammation of the throat, tonsillectomy is also recommended.Endocarditis prophylaxis is suggested before surgical and dental procedures for those already affected by rheumatic endocarditis.
Prevention
Because rheumatic endocarditis is caused by immunologic dysregulation resulting from streptococcal infection, preventive measures are aimed at early and consistent therapy of inflammatory diseases triggered by streptococci, such as tonsillitis (inflammation of the tonsils), scarlatina (scarlet fever), or otitis media (inflammation of the middle ear). Endocarditis prophylaxis before surgical or dental procedures also serves to prevent streptococcal infection and thus rheumatic endocarditis.
Follow-up
Rheumatic endocarditis (postinfectious endocarditis) is a bacterial autoimmune sequelae. Follow-up with complete cure is possible in principle. Since there is a risk of valvular heart failure with this disease, prompt follow-up is very important. Taking antibiotics is indispensable. Here, attention should be paid to correct and regular intake. In particularly severe cases, additional intake of cortisone is necessary. To relieve possible pain, treatment with anti-inflammatory drugs such as acetylsalicylic acid is also advised. In order not to put additional strain on the body and especially the heart, stress and physical work should be avoided and, in severe cases, bed rest should also be observed. After the disease with rheumatic endocarditis (post-infectious endocarditis), regular follow-up examinations are important in order to observe the healing process and, if necessary, to initiate further drug therapy. Rheumatic endocarditis (post-infectious endocarditis) is healed after one to two months if the course is positive. However, the prognosis here depends very much on when the disease is detected and whether severe damage to the heart valve has occurred. In bad cases, this can lead to chronic changes in the heart valve and, in extreme cases, require surgical intervention.
Here’s what you can do yourself
Rheumatic endocarditis is amenable to self-help but requires treatment by specialists such as an internist or cardiologist. Self-help in everyday life refers on the one hand to the acute disease, and on the other hand also to the aftercare and the prevention of a possible relapse of the disease. Rest is an important factor with regard to the acute disease. Cooperation of the patient is crucial here. Physical exertion and sports should be avoided until the doctor allows them again. Inflammation of the body can often be favorably influenced by sleep in sufficient quantity and plenty of fluids. Water and herbal teas are particularly recommended here. Nicotine and alcohol should be avoided. Protection in wind and weather is also important in order not to strain the body’s weakened immune system. Self-help is still possible even after rheumatic endocarditis has been overcome. On the one hand, to build up fitness again in a targeted manner. This is best done in consultation with the family doctor or a specialized sports therapist in order to find the right dosage of exercise. Since rheumatic processes can also be specifically influenced by a healthy diet, it makes sense to change this as well. A Mediterranean diet with lots of fruit and vegetables instead of meat and sausage makes sense in this context. An adequate amount of drinking is also always important.
