Kawasaki disease is an acute inflammatory disease of the blood vessels that occurs in infants and young children. The inflammation affects many parts of the body, but the most serious effect is on the heart. Kawasaki disease is the leading cause of acquired heart disease among children living in the United States and other developed nations.

There is no known cause of Kawasaki disease. Symptoms include:

  • Fever
  • Changes in extremities, including redness and swelling of the hands and feet
  • Rash
  • Redness of the white part of the eye
  • Red, cracked lips and red tongue ("strawberry tongue")
  • Lymph node enlargement of the neck

Many illnesses present with some or all of the clinical signs and symptoms listed above. There is no simple diagnostic test for Kawasaki disease. Instead there are criteria based on symptoms and test results that help us make the diagnosis. If fever is present for 5 days or more with at least 4 of the above symptoms, your pediatrician may diagnose Kawasaki disease.

When these criteria are not met, an evaluation including blood work, urinalysis, and possibly an echocardiogram (ultrasound of the heart) can help your pediatrician make the correct diagnosis.

How Kawasaki Disease Affects the Heart

Diagnosis and management of Kawasaki disease requires the involvement of a pediatric cardiologist who can perform and evaluate an echocardiogram.

Kawasaki disease can produce inflammation of the coronary arteries (the arteries which supply blood to the heart muscle), resulting in dilation (enlargement) or in the formation of aneurysms.

Clots can form inside aneurysms and block the flow of blood to the heart muscle. This can damage the heart muscle, causing a heart attack.

Over time, aneurysms can resolve, although sometimes a narrowing can develop where the aneurysm was. A narrowing in the blood vessel may also limit blood flow to the heart muscle.

Treating Kawasaki Disease

A child diagnosed with Kawasaki disease will be admitted to the hospital and receive IV Immunoglobulins (IVIG) at the time of diagnosis. IVIG helps to "clear the blood" of inflammatory cells circulating in the body. High-dose aspirin will also be used to help decrease the inflammation.

If the initial echocardiogram does not show any signs of coronary artery involvement, the aspirin dose will be decreased and treatment with a lower dose of aspirin will continue for a total of 6-8 weeks. Most children respond to this treatment regimen. For most children who receive IVIG and aspirin, their fevers subside quickly and other symptoms improve over a short period of time. This treatment decreases the chance that the coronary arteries will develop aneurysms.

If your child continues to have fevers after receiving a dose of IVIG or has a large coronary artery aneurysm, an additional dose of IVIG may be administered or other medications may be prescribed.

After discharge from the hospital, your child will be closely followed by a pediatric cardiologist. If there are coronary aneurysms present, other aspirin or blood thinners might be used to treat your child. Length of time for treatment with blood thinners will depend on how the coronary artery aneurysms behave over time.

Long-term Prognosis

All children with Kawasaki disease will have at least three echocardiograms to allow the pediatric cardiologist to look at the coronary arteries. One echocardiogram will be performed at the initial diagnosis, the second will take place two weeks after diagnosis, and the final echocardiogram will take place 6 to 8 weeks following the initial diagnosis. Since coronary artery aneurysms develop within 6 to 8 weeks of the initial illness, most pediatric cardiologists will "discharge" the patient from any further follow-up.

If there are coronary abnormalities noted at any time, your child will have much closer follow-up with a pediatric cardiologist. The pediatric cardiologist will create the best individualized course of treatment and follow-up plan for your child.

Long-term risks for coronary artery disease in adulthood are a concern for patients who have had coronary artery aneurysms secondary to Kawasaki disease. It is essential to make sure that your child decreases the chances for long-term coronary artery atherosclerosis by eating a heart-healthy diet. Cholesterol screening should be done as your child gets older, especially if there is a family history of high cholesterol.

Our team of dedicated pediatric cardiologists and dieticians will also work with your child, your family, and your pediatrician to discuss and develop an individualized exercise program, healthy living practices and surveillance plan to support your child throughout his or her life.

To make an appointment with a Children's Heart Program physician, please call 410-328-4348.