Acquired Heart Diseases

I. Rheumatic

Acute Rheumatic Fever

It often involves the heart and joints, leaves a permanent damage and results in consequences leading to a surgery.

Symptoms:

  • Fever
  • Wandering joint pain, swelling, redness and heat
  • Carditis, damaged cardiac valves, cardiac insufficiency and associated asthenia, quick tiredness, tachycardia
  • Involuntary movements (e.g. tics) of arms, legs and face
  • Non-itchy circular redness looking like a map on the skin

Although other effects are minor and temporary, the effect on cardiac valves is most dangerous and can leave permanent damages for a lifetime.

Etiology (Cause)

Rheumatic fever can be caused by bacteria called Beta hemolytic streptococcus that grow on the throat and leads to angina. However, that does not mean that anyone who has this bacteria growing on the throat must catch rheumatic fever. 1-2% of patients suffered from angina catch rheumatic fever. Some constitutional factors that vary from person to person play role in development of this disease. Some persons are only carriers and do not get sick, and can have streptococcus on the throat. Such patients are called “contact” and do not require a treatment if no symptoms of inflammation are present.

Rheumatic fever is not infectious. However, bacteria called streptococcus that causes rheumatic fever and inflammation of throat is infectious.

Although no positive evidence on genetic transition (heredity), it can be more common in some families.

Diagnosis

It commonly appears in children between the ages of 5 and 15. It is quite rare other than these ages. Since the possibility of recurrence is higher in children who have previously had rheumatism, they can catch it when they are older than 15 years old. Therefore, a person who has previously suffered from rheumatism needs to be protected by repository penicillin.

Growing streptococcus on the throat does not mean a rheumatic state. Only a certain portion of patients (around 3-5%) whose angina was ill-treated and who are prone to this disease (yaklaşık%3-5) are likely to suffer from rheumatism.

Another issue, which is commonly but wrongly known by public, is ASO value. The higher ASO value indicates only that a streptococcal inflammation has occurred within the last couple of months. It is favorable if any rheumatic signs are present. Higher ASO value alone does not mean anything without other signs. But unfortunately many patients are diagnosed with rheumatism without even considering other signs and valuable laboratory findings just because the ASO value is high, and receive unnecessary treatment.

Should we suspect from Rheumatic Fever whenever our children have a leg pain?

Leg pain is very common in children particularly who are active or involved in sports. Especially lower-leg pain or pain in knees is non-noxious and subsided by resting and massaging. If a night-pain accompanied by fever is present and not subsided in the morning, if the child has difficulty in moving and if any of the signs, e.g. swelling joint, redness and heat, are present, they should suggest a rheumatic state.

Does Rheumatic Fever necessarily cause rheumatic valve?

No, rheumatic fever can only involve the joints as well as the heart. Even if it only involves the joints first, the heart can also be affected if it recurs. Therefore, preventing recurrence of the disease can also prevent involvement of the heart, and it is much easier and effortless than trying to treat.

What is Cardiac Rheumatism?

It is dysfunction of valves and heart depending on the damage to heart and valves caused by rheumatism. For example, persons who have a history of rheumatism might have initially insufficiency in other words blood leaking related to poor closure of valves then signs of thickness and narrowing of valves might appear after long years. Moreover, some patients might present symptoms of cardiac rheumatism in middle ages depending on the mild rheumatism in childhood. This is called silent cardiac rheumatism.

Can Rheumatic Fever be contracted more than once?

Yes, it is highly likely for this disease to recur. The chance of catching it again for a person who has had it for once is highly higher than those who have never caught it. Therefore the patients must receive repository penicillin every 3 weeks for protection against recurrence. Each rheumatic recurrence gradually causes more damage to valves of the heart and even replacement of valves by an operation may be required. This is both expensive and serious operation. Even the best artificial valve never works as perfectly as your own valve. After replacement of the valve, you must always use drugs to protect the valve against microbial growth and to prevent clot formation. Thus, the best thing to do is early diagnosis, good treatment and prevention of recurrence.

How can I protect myself from recurrence of Rheumatic Fever?

Repository penicillin must be applied regularly on the basis of 3 weeks.
If complaints about angina such as fever, throat pain, and swallowing difficulty are present, the child must be immediately taken to a pediatrician to receive treatment for inflammation.
If any signs of recurrence of rheumatism are present, immediately consult your doctor.
The periodic checks must not be skipped. The purpose of checks is to early spot any changes and to take precautions.

Within several weeks following fever and pain in throat, the symptoms such as painful and swelling joints, redness, headache, nausea, vomiting, fatigue, quick tiredness, apnea, and tachycardia might appear. Even if one or several of these symptoms appear, the possibility of recurrence of rheumatism must be investigated.

Follow-up

Those who have cardiac rheumatism should be checked every 6-12 months depending on the severity of valvular problem, those who have only joint rheumatism should be checked every 1-2 years.

Treatment

Bed Rest

The work of ill and tired heart is reduced by resting to allow quicker and smooth recovery. In case of severe carditis, the child’s need to eat and use the toilet should be met in the bed on the onset of the disease, or they should be carried to the toilet. As the child recovers, he/she can gradually go back to normal daily activities according to doctor’s recommendations. During bed rest, if any signs of apnea are present the head and back of the patient should be supported by pillows. Longer resting periods than recommended are unfavorable.

Long-Acting Penicillin

Prevents bacteria growth on the throat that causes rheumatic fever and recurrence of rheumatic fever. Although it can be sometimes applied every 4 weeks, it is better at every 3 weeks. Because the efficacy of medication is reduced in the week four, its protective effect is also reduced. Therefore, not to leave it to luck, administration of long-acting penicillin every 3 weeks will provide safer protection. The use period of repository penicillin should be up to age of 20 for patients who have only suffered from joint rheumatism or from mild cardiac rheumatism but whose valves are completely corrected. It is recommended to use repository penicillin for a lifetime for patients who have previously suffered from cardiac rheumatism and had permanent damage to valves.

CAUTION!

If any of the symptoms such as rash and skin eruption, itching, and swelling are present after administration of penicillin, immediately consult the nearest doctor. You should have repository penicillin injected at a fully equipped hospital with an emergency room against the possibility of allergic problems, e.g. anaphylaxis which is rare but serious, and must stay there for at least 30 minutes in case of development of any allergy.

Aspirin

Although aspirin is an old drug, it is still essential for many diseases and rheumatism. Nevertheless, one should exercise care with using it, aware of side effects and be careful. It could cause stomach irritation, resulting in stomachache, and acid indigestion. So, it should be taken on a full stomach. If it still causes complaints, it can be taken with milk or antacid drugs.

CAUTION!

The use of drug must be discontinued and the doctor must be informed in case of tinnitus, nausea, vomiting, headache, purple spots in places on the skin, black defecation, and bleeding at any locations (e.g. nosebleed). In case of chicken pox or coming to contact with a person with chicken pox during use of aspirin, administration of drug must be immediately discontinued and the doctor must be informed.

Cortisone:

It is the most effective drug for cardiac rheumatism. Side effects include facial swelling, weight gain, increased appetite, stretch marks, and increased hairing during the use of drug. They will disappear when the use of drug is stopped. In addition, because the cortisone causes retention of salt and water in the body, the blood pressure could raise. To prevent this, you should avoid salt as much as possible during use of cortisone (and can go back to your regular regime of salt when cortisone is completely finished). You should take it on a full stomach or event with little milk because it can cause stomachache, acid indigestion and gastric acidity. Cortisone must not be cut off all at once. You should follow your doctor’s recommendations when cutting off. Sudden cut-offs are highly harmful.

II. Infectious Myocarditis (myocardial inflammation)

Myocardial inflammation and necrosis depending on various causes. Acute and chronic Coxsackie B virus is typical examples of myocarditis (account for 50%).

Diagnosis

It can vary between asymptomatic manifestation and chronic myocarditis + dilate cardiomyopathy depending on the age, being acute or chronic, and immune response of person. It can progress very severely and rapidly in newborns whereas the diagnosis can only be made in infants and children when they develop cardiomyopathy .

In a Newborn with Coxsackie B

The symptoms include fever, tachycardia, gallop rhythm, cyanosis, gray skin (sign of shock), respiratory distress, severe cardiac insufficiency, and hepatomegaly. The sound of heart beats come from deep; signs of acidosis are present, and murmur of mitral regurgitation can be auscultated in apex.

Children / Adults with Coxsackie B

The symptoms include fever, tachycardia, chest pain, signs of influenza, gallop rhythm, dysrhythmia, signs of cardiac insufficiency, and signs of mitral and tricuspid insufficiency. The findings of Tele and ECG are same.

Diagnosis

Sedimentation is higher, cardiac enzymes (SGOT, CK MB band, LDH) and Coxsackie virus IgM are also higher. Especially Coxsackie virus IgM can remain high for 5-10 years. Cardiac dilatation, valvular insufficiency, and failure of myocardium to contract which appear on echocardiography support the diagnosis. The final diagnosis is made by biopsy.

Treatment

Bed rest, oxygen, monitorization (in case of sudden deaths), drugs to strengthen myocardium, diuretics, restriction of liquids/salt are required. Drugs to regulate the rhythm are administrated in case of arrhythmia. Corticosteroid and immunosuppressive therapy should be considered.

Prognosis

The mortality rate is 75% for newborns. 10-20% can recover spontaneously. The rate of recovery in adults is reported 10-50%. In case of development of dilate cardiomyopathy, untreated 50% die within the first 2 years.

Kawasaki Syndrome

A disease causing vasculitis od medium-sized arteries including coronary arteries accompanied by skin eruption and fever in children particularly younger than 5 years old. It is not infectious but expected to play a superantigen role in development of certain types of bacteria toxins. Autoimmunity is also considered but no definite data is available. It is good if prognosis coronary aneurism has not been developed; the disease is subsided spontaneously.

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