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The long-term management of children who have had Kawasaki disease depends on the degree of coronary artery involvement.
Children who never had any abnormalities of the coronary arteries have an excellent outlook. With almost 30 years of follow-up, children in whom aneurysms were never detected at any stage of the illness do not show a higher incidence of heart disease or any other disorders. Some small research studies suggest that their blood vessels may be stiffer and less reactive than those of people who never had Kawasaki disease. Until more research is completed, children should be screened for any additional risk factors that are known to affect coronary arteries. A cholesterol profile is recommended at the one-year follow-up visit. In addition, children should eat a heart-healthy diet, exercise regularly and avoid smoking.
Recent research carried out at Children's Hospital Boston examined the psychosocial and physical functioning of children who have recovered from Kawasaki disease. Those without aneurysms were similar in overall health and psychosocial status to the general population.
Children who have developed aneurysms are monitored more frequently. The severity of coronary artery abnormalities determines the frequency and type of testing. Chronic management of patients with coronary artery aneurysms is focused on preventing clots and monitoring for signs/symptoms that the heart is not getting enough blood flow. Children with coronary aneurysms require long-term therapy to decrease the likelihood of clotting within the aneurysm.
Aspirin therapy reduces platelet activity. Other anti-clotting medications such as heparin, warfarin (Coumadin), or clopidigrel (Plavix) may be added to aspirin for patients with larger aneurysms. The cardiologist will balance the risks and benefits for the individual child when determining a medication regimen to prevent clotting in coronary aneurysms.
Children who are on long-term aspirin therapy should receive yearly flu shots to reduce the risk of Reye syndrome, a disease linked to the combination of aspirin use (usually high dose) and viral illness, such as chicken pox or influenza. Aspirin therapy should be stopped for six weeks after the chicken pox vaccine is given, but other anti-platelet drugs can be substituted. If a blood clot occurs within a coronary aneurysm, either as detected by echocardiography or by symptoms of a heart attack, doctors will start special treatments to dissolve the clot and to prevent further clotting in order to protect the heart muscle.
The aneurysms formed in Kawasaki disease may heal gradually over time, with the highest likelihood of return to normal for patients whose aneurysms were small to begin with. On the other hand, stenosis (the narrowing or blockage of coronary arteries in patients with aneurysms) tends to worsen with time. Again, the highest likelihood of stenosis occurs in those children with the largest aneurysms (giant aneurysms).
Stress tests, cardiac MRI's and other imaging studies to assess blood flow to the heart should be part of disease management for any child with significant cardiac abnormalities resulting from Kawasaki disease.
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