Rheumatic Heart Disease

What is rheumatic heart disease?

In the early 1900s, before the advent of widespread antibiotics to treat strep throat, rheumatic heart disease was the most important cause of heart valve diseases and the leading cause of death for 5-20 year-olds in the U.S.  While rheumatic heart disease is now rare in developed countries, its incidence rate has not decreased in developing countries.  A patient with rheumatic heart disease suffers from potentially life-threatening problems due to valves and other heart tissues that have been damaged as a complication of rheumatic fever, an inflammatory condition that can occur 1-5 weeks after an untreated streptococcal (strep throat) infection. 

Rheumatic heart disease includes both an initial pancarditis (inflammation of the outer, middle, and inner layers of the heart muscle and lining) that resolves within several weeks and permanent, chronic heart valve damage that can last a lifetime.  Rheumatic fever can affect a person at any age, but it is most common among children 5-15 years old.  Rheumatic fever can cause inflammation problems in connective tissue throughout the body including the heart, brain, joints, and skin.  In 80% of patients, rheumatic fever subsides within 12 weeks, but the effects of rheumatic heart disease can last the rest of a patient’s life.  Actually, symptoms involving the heart valves may not surface until 20 or 30 years later.  Rheumatic fever and rheumatic heart disease are rare, even among patients who have had untreated strep throat infections and rheumatic fever does not always damage the heart to cause rheumatic heart disease.

What causes rheumatic fever and rheumatic heart disease?

Scientists do not know exactly how a streptococcus infection in the throat causes rheumatic fever in some patients, but they suspect that the condition involves a disordered autoimmune response to the infection.  When rheumatic fever occurs, it always follows a bacterial infection in the throat by group A beta-hemolytic streptococcal pharyngitis, though sometimes the infection can be present without causing any of the symptoms of strep throat.  These bacteria have antigens (parts on the surface of an organism’s cells that the body uses to identify and fight foreign germs) that are similar to parts on the surface of some cells in the heart.  Because of this similarity, some scientists think that the body can get confused and start hurting itself, causing rheumatic fever and heart disease by what researchers call antibody cross-reactivity.

What are the symptoms, dangers, and complications of a strep throat infection, rheumatic fever, and rheumatic heart disease?

A streptococcal infection in the throat does not always cause symptoms, and it is possible for a person’s symptoms to be so mild that he gets rheumatic fever without knowing that he had strep throat a few weeks earlier.  The symptoms of strep throat can include a sore, scratchy throat; fever; difficulty swallowing; headache; rash; stomach pain and vomiting in young children; red, swollen tonsils; and tender, swollen lymph glands in the neck.  Most sore throats are caused by viral infections and need no treatment to quickly go away on their own.  Some, however, are the result of strep throat bacterial infections.  It is important to identify strep throat because not only does it respond well to treatment (unlike cold viruses), but the infection, if left untreated, can lead to rheumatic heart disease and other serious complications.

When rheumatic fever damages heart valves to cause rheumatic heart disease it can cause one or more valves to open and/or close incompletely.  The mitral and aortic valves on the left side of the heart are more susceptible to damage by this disorder than the two valves on the right side of the heart, the tricuspid and pulmonary valves.  The most serious and most advanced possible consequence of altered valve function is congestive heart failure, a condition in which the heart becomes unable to pump hard enough to supply the body with enough blood to maintain normal function because the diseased valve or valves have severely decreased its efficiency.

Rheumatic fever can produce a wide variety of symptoms.  Fever, rash, headache, weight loss, nose bleeding, fatigue, excessive sweating, stomach pain, chest pain, new heart murmurs, shortness of breath, and vomiting are some of the general symptoms.  More specific symptoms include joint swollenness and pain that moves from joint to joint among different large joints such as the ankles, wrists, knees, and elbows; small lumps under the skin; personality changes such as increased irritability and decreased attention span; and involuntary movement or decreased control of muscles.  Spasmodic involuntary muscle movements are due to rheumatic inflammation of the central nervous system (the brain and spinal cord).

Symptoms of rheumatic heart disease include murmurs, a heart rate that is rapid and out of proportion to any fever that is present and other symptoms of heart valve diseases and congestive heart failure such as shortness of breath, decreased endurance, swelling, chest pain, and fatigue.

How do doctors diagnose strep throat, rheumatic fever, and rheumatic heart disease?

Physicians usually look for evidence of strep throat before diagnosing rheumatic fever or rheumatic heart disease.  The physical symptoms of strep throat are not specific enough to allow doctors to diagnose the illness without laboratory tests.  Several tests can help a doctor see if group A streptococcal bacteria are present in a patient’s throat.  A rapid antigen test is a throat swab test that only takes about 15 minutes to develop but has an error rate of 20%.  This means that a negative rapid strep test cannot rule out strep throat as a possible cause of a patient’s symptoms.  Therefore, patients who have a negative result must have their throat swabs cultured to see if the bacteria are present in their throats.  A throat culture usually takes 24-48 hours to develop and can definitively show doctors whether or not a patient has strep throat.

Doctors usually diagnose heart-related symptoms as rheumatic heart disease after diagnosing rheumatic fever.  To diagnose rheumatic fever, doctors may use a set of guidelines called the “modified Jones criteria.”  They will be highly suspicious of a recurring attack in patients previously diagnosed with rheumatic fever.  A recent strep throat infection is not necessary to diagnose rheumatic fever in recurring rheumatic fever patients because they are much more susceptible to an attack.  In order to have a firm diagnosis of patients with no history of rheumatic fever, however, physicians usually try to confirm first that a strep throat infection was present in the recent past and that at least 2 of the major criteria or 1 major and 2 minor criteria are present in the patient they are helping.  The major diagnostic criteria include an inflamed heart; arthritis (pain and inflammation) in more than one joint; involuntary movement and decreased control of muscles; wide, red patches of skin that do not itch; and lumps under the skin.  Doctors may be able to find evidence of an inflamed heart by asking about symptoms like weakness, shortness of breath, or chest pain, or by looking at an electrocardiogram (ECG or EKG), Doppler ultrasound, or chest X-ray.  The minor diagnostic criteria include fever, joint pain without inflammation, a specific electrocardiogram abnormality, and blood tests indicating inflammation.

How can patients prevent rheumatic heart disease?

It is not always possible to prevent rheumatic heart disease, but prompt antibiotic treatment of strep throat can drastically reduce a patient’s risk for developing rheumatic fever and rheumatic heart disease.  Also, continued long-term antibiotic treatment of patients who have already experienced an episode of rheumatic fever can help prevent recurring episodes and therefore decrease the likelihood of heart damage.  Thus, the best way to prevent rheumatic heart disease is to prevent rheumatic fever by treating strep throat infections or by taking preventative antibiotics for patients susceptible to a recurring attack.  Researchers estimate that about 3% of patients who allow strep throat to go untreated develop rheumatic fever.  Patients should consult their physicians when they have a sore throat without a cold or runny nose, one that lasts longer than 2 days or is accompanied by a fever higher than 101°F; tender or swollen lymph glands in the neck; pus in the back of the throat; excessive drooling in a young child; severe difficulty breathing or swallowing; a rough, red rash; or joint pain.  Once a patient goes to his physician, he should strictly comply with his doctor’s recommendations and should continue to take all of the prescribed antibiotics even after he has started to feel better.

What are the treatment options for rheumatic heart disease?

The first step in treating patients with rheumatic heart disease is to eradicate any remaining streptococcal bacteria with antibiotics.  Treating rheumatic fever can involve aspirin and perhaps corticosteroids to help with fever, pain, and inflammation until the attack subsides.  The use of steroids, however, is controversial, with some sources claiming them to be ineffective.  Children less than 20 years old should only take aspirin if a physician advises them to do so because studies have shown a link between children with flu-like illnesses taking aspirin and developing Reye’s syndrome.  Reye’s syndrome is potentially fatal and affects the blood, brain, and liver of patients who are recovering from viral infections.  Patients who have previously had a rheumatic fever attack may need to take long term antibiotics because they can help prevent future attacks and minimize heart damage.  Patients who adhere to their antibiotic regimens may see their heart valve problems subside.  If a person’s heart has become inflamed, he may need bed rest and medications to help with congestive heart failure.  Most valve damage by rheumatic heart disease is minimal and needs no serious treatment, but doctors may need to surgically repair or replace severely damaged heart valves with artificial, donated, or animal valves.  Surgical reparation procedures include both invasive, open-surgery techniques to widen valves or separate leaflets and less-invasive, catheter-based procedures such as balloon valvuloplasty to widen the opening.

In April 2007, the American Heart Association revised its recommendations about preventative antibiotics for patients with rheumatic heart disease.  The organization decided that because they found no convincing evidence that dental, gastrointestinal, or genitourinary tract procedures are related to developing bacterial endocarditis (BE), they now only recommend preventative antibiotics before dental procedures for patients with the highest risk for developing bacterial endocarditis.  High risk patients include those with prosthetic heart valves, previous endocarditis infection, or certain congenital heart diseases, but not those with rheumatic heart disease alone.  Furthermore, the association does not recommend antibiotics before gastrointestinal or genitourinary tract procedures for any patients, if the purpose of those antibiotics is solely to prevent bacterial endocarditis.  Bacterial endocarditis is infection and inflammation of the inner lining of the heart or its valves.