Long Term Effects of Kawasaki Disease

New information has led us to believe that for a subset of patients who had some abnormalities of the echocardiogram in childhood, there can be cardiovascular complications years after the acute phase of illness as a result of inflammation and scarring of the heart and blood vessels. This scarring takes years, sometimes decades, to develop and is often clinically silent. As a result of the scarring, the blood vessels of the heart can narrow (coronary artery stenosis), which can lead to reduced blood flow to the heart muscle. Aside from the coronary arteries, in patients with persistent or resolved aneurysms, there is the possibility of heart problems including irregular heart beat and decreased heart muscle function as a late complication of the heart muscle inflammation (myocarditis) during acute KD.

However, the limited information that doctors have today was collected largely from studies that were completed before the routine use of IVIG to treat acute KD. To date, there has been no study of the long-term outcomes of KD patients treated with IVIG during the acute phase of illness. And so, the natural history and late cardiovascular complications of KD under this current treatment regimen remain largely unknown. Based on current information, patients whose echocardiogram was always normal in childhood have no known increased risk of cardiovascular complications in adulthood as a result of their KD.

Based on current information, we recommend that young adults with a known history of coronary artery damage during acute KD consider having a CT Calcium Score performed as a screening test for coronary artery damage. The test must be ordered by a physician and we are recommending this test for all young adults who had abnormalities detected by echocardiogram at some point during the course of their initial KD. The screening test could also be considered for young adults who do not know the results of their cardiac evaluation in childhood. A negative test (calcium score of 0) is reassuring if performed at least 10 years after the initial KD illness and suggests that no further evaluation is necessary. A positive test (calcium score>0) suggests that some injury to the arterial wall may have occurred and should be investigated further. Currently, insurance companies are declining to pay for the test and the cost to the family is variable but costs $100 in San Diego. Please contact us if you have questions about our recommendations for this testing.

Kawasaki Disease Research Study

The Kawasaki Disease Research Center is currently conducting a research study to better define the long-term cardiovascular outcomes of KD patients. The research study involves filling out a questionnaire about your health-related history. You will also be invited to complete a short follow-up health questionnaire every few years.

If you are an adult or young adult with a childhood history of KD and are interested in learning more about the study, please take a moment to read the flyer on the main page, or feel free to contact us directly at adultkd@ucsd.edu.

Adult KD Articles