HEART & BLOOD Cardiovascular System • Aortic aneurysm • High blood pressure (hypertension) • High blood cholesterol • Thrombophlebitis • Rheumatic fever • Varicose veins • Heart arrhythmias • Atrial fibrillation • Endocarditis • Aortic valve stenosis • Aortic valve regurgitation • Mitral valve stenosis • Mitral valve regurgitation • Pulmonary hypertension • Raynaud's disease • Long QT syndrome • Mitral valve prolapse • Pericarditis • Cardiomyopathy • Myocarditis • Arteriosclerosis / Atherosclerosis • Peripheral arterial disease (PAD) • Low blood pressure (hypotension) • Aortic dissection • Lymphedema • Pulmonary valve stenosis • Ventricular septal defect (VSD) • Tetralogy of Fallot • Coarctation of the aorta • Atrial septal defect (ASD) • Patent ductus arteriosus (PDA) • Left ventricular hypertrophy • Bundle branch block • Heart murmurs • Patent foramen ovale • Transposition of the great arteries • Hypoplastic left heart syndrome • Sudden cardiac arrest • Prehypertension • Tricuspid atresia • Ebstein's anomaly • Buerger's disease

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Rheumatic fever is a serious inflammatory condition that can affect many parts of your body — heart, joints, nervous system and skin. Although rheumatic fever can occur at any age, it most frequently occurs in children between the ages of 6 and 15 years. The disease is twice as common in females as it is in males.

Symptoms of rheumatic fever generally appear within five weeks after an untreated streptococcal (strep) throat infection. Most cases of strep throat don't lead to rheumatic fever. In fact, even in untreated cases, only a small percentage of people with strep throat develop rheumatic fever.

In many cases, rheumatic fever may affect the heart valves (rheumatic carditis) and interfere with normal blood flow through the heart. There's no cure for rheumatic fever. But it can be prevented by prompt and thorough treatment of a strep throat infection with antibiotics.

Rheumatic fever isn't as common in the United States today as it was at the start of the 20th century, before the widespread use of the antibiotic penicillin. Outbreaks do occur periodically, however. Rheumatic fever is still common in developing countries.

Common signs and symptoms of a strep infection include:

• Sore throat
• Red and swollen tonsils
• Fever
• Headache
• Muscle aches

Many times, however, the initial strep infection may not cause any symptoms.

Signs and symptoms of rheumatic fever may include a combination of painful, swollen joints, chest pain, fatigue and shortness of breath. In diagnosing rheumatic fever, doctors generally look for the presence of either two of the following major criteria or the presence of one major plus two minor criteria. In all cases, evidence of a preceding strep throat infection is key to making a diagnosis of rheumatic fever.

Major criteria

• Inflammation of your heart, sometimes indicated by weakness and shortness of breath or chest pain. Your doctor might suspect heart inflammation based on a physical exam or on results of medical tests such as an electrocardiogram or a chest X-ray.
• Painful arthritis, most often affecting your ankles, wrists, knees and elbows, and often migrating from joint to joint.
• Involuntary jerky movements of your limbs and face or more subtle movement difficulties, such as definite deterioration in handwriting. These signs usually disappear over weeks to months.
• Broad, pink or faint-red, nonitching patches on your skin (uncommon).
• Lumps under your skin (uncommon).

Minor criteria

• Joint pain without inflammation
• Fever
• Previous rheumatic fever or evidence of rheumatic heart disease
• Abnormal heartbeat on an electrocardiogram
• Blood test indicating inflammation
• New heart murmurs

The exact cause of rheumatic fever isn't clear. In a few people, it seems that when the body fights a strep throat infection, other parts of the body develop inflammation. For example, the heart valves aren't necessarily infected with the streptococcal bacteria, but they can be injured or inflamed as the body fights strep throat.

Medical research has focused on an abnormal immune system response to the antigens produced by specific types of streptococcal bacteria. One possible cause for this is the similarities between streptococcal antigens and heart valve proteins and heart muscle cells. In addition, researchers are studying whether some people have a greater genetic disposition for an abnormal immune system response to streptococcal antigens.

If you have a sore throat along with a fever that has lasted more than 24 hours — or a severe sore throat without cold symptoms and without much fever, especially if you've been close to someone with strep throat — see your doctor to determine whether you have strep throat. Although most of the time strep throat doesn't lead to rheumatic fever, you can usually prevent rheumatic fever by using antibiotics to treat strep throat. Also see your doctor if you've recently had a sore throat and high fever and you're experiencing difficulty breathing or chest pain.

If you or your child has signs or symptoms of rheumatic fever, diagnosis will likely involve a physical examination and questions by your doctor regarding these signs and symptoms. The physical exam may include:

• Checking your joints for pain and inflammation
• Examining your skin for rashes or lumps
• Listening to your heart for abnormal rhythms or murmurs

Your doctor may recommend an electrocardiogram (ECG) of your heart to check for abnormal rhythms. Electrocardiography involves attaching electrode patches to your skin to measure electrical impulses given off by your heart. The electrical impulses, which cause your heart muscle to contract, are recorded by an electrocardiograph machine. They typically are recorded in the form of waves, which are displayed on graph paper or on a monitor.

No laboratory test can confirm that you have rheumatic fever. Doctors base a diagnosis of rheumatic fever on the presence of several major and minor criteria. The most common major criteria are:

• Joint pain and swelling that migrates from joint to joint
• Inflammation of the heart

Diagnosing rheumatic fever requires not only the presence of key signs and symptoms but also evidence of a recent strep infection. Your doctor may take a blood sample to test for the presence of antibodies to streptococcal bacteria. However, it's possible that by the time you see your doctor, your throat culture test for strep will be negative. In that case, the only suggestion of strep may be you telling your doctor about a recent sore throat accompanied by fever.

During a first rheumatic fever attack, about half of people develop heart inflammation, but this doesn't always result in permanent damage. Most people with rheumatic fever recover fully after six weeks. In some cases, however, one or more of the heart's valves may be scarred. Permanent heart damage due to rheumatic fever is known as rheumatic heart disease. In many cases, heart damage isn't discovered until years later.

In rare cases of rheumatic fever, the inflammation causes so much damage to the heart muscle that it leads to congestive heart failure. In other cases, a scarred heart valve may prevent adequate blood flow or may permit backward flow of blood. If there's serious impairment to the function of your heart valves, surgery may be needed to repair or replace the damaged valve or valves.

Rheumatic fever can also affect these parts of your body:

• Joints. Often, several joints are affected with painful swelling, redness and sensation of heat.
• Brain. If acute rheumatic fever affects the brain, loss of coordination and uncontrolled movement of the limbs and face may occur. These movements are called chorea — from the Greek word choreia, which means "choral dance." They're also sometimes called Sydenham's chorea, rheumatic chorea or St. Vitus' dance. Chorea occurs in about one in 10 rheumatic fever cases. Chorea usually subsides or disappears within weeks to months.
• Skin. The disease can produce broad patches or, irregularly, round, pink to faint-red areas on your skin (erythema marginatum). Lumps or nodules may occur beneath normal-appearing skin.

Treatment of rheumatic fever involves a dual approach:

• Antibiotics to rid your body of the streptococcal infection and prevent recurrences. To eliminate any remaining strep bacteria once you have rheumatic fever, your doctor may prescribe penicillin or another antibiotic. Usually, you'll then need to take additional antibiotics for several to many years to prevent additional attacks of rheumatic fever. Without continued antibiotic treatment, recurrence is common during the first three to five years after the initial infection.
• Other medications to ease the symptoms of the disease. To reduce inflammation of your heart or joints, your doctor may recommend specific doses of aspirin or an over-the-counter or prescription nonsteroidal anti-inflammatory medication. For severe heart inflammation, a corticosteroid medication, such as prednisone, can reduce the inflammation.

Preliminary drug trials show potential for a vaccine to prevent strep. But until an effective immunization is developed, the only known way to prevent rheumatic fever is to treat strep throat infections with antibiotics.

Most of the time, a virus is the cause of a sore throat, and viruses don't lead to rheumatic fever. However, a sore throat with fever lasting more than 24 hours may indicate a strep infection. Only a small percentage of people with untreated strep throat will develop rheumatic fever. Prompt and proper treatment of strep throat with antibiotics can prevent strep throat from progressing to rheumatic fever.