Kawasaki%20disease Management
Monitoring
Long-Term Follow-Up
W/o Detectable Coronary Lesions
- Children w/ no known cardiac sequelae during the 1st month of Kawasaki disease appear to return to their previous state of health, w/o signs or symptoms of cardiac impairment
- Altered lipid metabolism persists beyond clinical resolution of the disease
- Lower myocardial flow reserve & higher total coronary resistance have been found in those w/ history of Kawasaki disease w/ normal epicardial coronary arteries
Regression of Coronary Lesions
- Coronary artery lesions resulting from Kawasaki disease change dynamically w/ time
- The likelihood that an aneurysm will resolve appears to be determined by:
- Initial size: smaller aneurysms have a greater likelihood of regression
- <1 year of age at onset of the disease
- Fusiform morphology
- Location in distal coronary segment
Persistent Coronary Artery Abnormalities
- Stenotic lesions that are secondary to marked myointimal proliferation are frequently progressive
- Highest rate of progression to stenosis occurs in patients w/ large aneurysms
- Worst prognosis occurs in children w/ giant aneurysms since thrombosis is promoted by sluggish blood flow w/in massively dilated vascular space & the frequent occurrence of stenotic lesions at the proximal or distal end of the aneurysms
Cardiovascular Risk Stratification
- Stratification of patients according to their relative risk of myocardial ischemia allows for patient management to be individualized
- The risk level for a given patient w/ coronary artery involvement may change over time because of the changes in coronary artery morphology
- No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness; dilatation subsides w/in 30 days after onset
- Suggest periodic assessment & counseling about known cardiovascular risk factors every 5 years
Risk Level II - Patients w/ transient coronary artery ectasia or dilatation, which disappears w/in the initial 6-8 weeks after the onset of illness
- No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness
- Recommend risk assessment & counseling at 3-5 year intervals
Risk Level III - Patients w/ solitary small-to-medium (>3 mm but <6 mm) coronary artery aneurysm in >1 coronary arteries on echocardiography or angiography, & aneurysm persists or worsens beyond 30 days after onset
- Long term antiplatelet therapy w/ low-dose Aspirin should be administered until the aneurysms regress
- No restriction on physical activity in patients <11 years of age
- For patients 11-20 years of age, stress tests w/ myocardial perfusion evaluation may be useful to guide recommendations for physical activity
- Due to risk of bleeding, participation in competitive collision or high-impact sports is discouraged in children receiving antiplatelet therapy
- Annual follow-up by a pediatric cardiologist w/ echocardiogram (ECG) is recommended
- Stress test w/ myocardial perfusion imaging is recommended every 2 years in patients >10 years of age
- Coronary angiography is indicated if myocardial ischemia is demonstrated by non-invasive tests
Risk Level IV - Patients w/ ≥1 large coronary artery aneurysm (≥6 mm) including giant aneurysms & patients in whom a coronary artery contains multiple (segmented) or complex aneurysms w/o obstruction
- Long term antiplatelet therapy is recommended
- Adjunctive therapy w/ Warfarin w/ a target International Normalized Ratio (INR) of 2-2.5 is recommended for patients w/ giant aneurysms
- Low molecular weight Heparin may be considered as an alternative to Warfarin for infants & toddlers
- Recommendation about physical activity should be guided by annual stress tests w/ myocardial perfusion evaluation
- Collision or high-impact sports should be discouraged
- Participation in non contact dynamic or recreational sports is encouraged if there is no evidence of stress-induced myocardial ischemia
- Cardiology evaluation w/ echocardiogram (ECG) should be done at 6 months intervals
- Stress tests w/ myocardial perfusion evaluation should be performed annually
- Monitor for known risk factors of atherosclerosis; counsel family accordingly
- Cardiac catheterization w/ selective coronary angiography should be performed 6-12 months after recovery from the acute illness, or sooner if clinically indicated
- Follow-up angiography may be indicated if noninvasive studies suggest myocardial ischemia
- For females of childbearing age, reproductive counseling is strongly recommended
Risk Level V - Patients w/ coronary artery obstruction confirmed by angiography
- Long-term antiplatelet therapy, w/ or w/o adjunctive therapy w/ Warfarin anticoagulation, is recommended
- Beta-blockers should be considered to reduce myocardial oxygen consumption
- Recommendation about dynamic physical activities should be based on the patient’s response to stress testing
- Collision or high-impact sports should be discouraged
- Patients should avoid a sedentary lifestyle
- Cardiology evaluation w/ an echocardiogram (ECG) should be obtained at 6 months intervals
- Stress tests w/ myocardial perfusion evaluation should be performed annually
- Monitor patient for known risk factors of atherosclerosis; counsel family accordingly
- Cardiac catheterization w/ selective coronary angiography is recommended to address the therapeutic options of bypass grafting or catheter intervention & to identify the extent of collateral perfusion
- Repeat cardiac catheterization may be indicated when new onset or worsening myocardial ischemia is suggested by noninvasive diagnostic testing or clinical presentation
- For females of childbearing age, reproductive counseling is strongly recommended
Prognosis
- Depends on cardiac involvement & its long-term sequelae
- Coronary artery aneurysms develop in 20-40% of untreated children or in patients treated w/ only Aspirin, which may eventually lead to rupture in adulthood, ischemic heart disease & myocardial infarction (MI)
- Aneurysms might be demonstrated in other extraparenchymal muscular arteries except the central nervous system (CNS) arteries
Prevention
- Schedule for administration of inactivated childhood vaccines should not be interrupted
- It may be necessary to reschedule administration of live viral vaccines (eg measles, varicella-containing vaccines) to at least 9-11 months after intravenous immunoglobulin (IVIG) administration
- If the risk of exposure to measles is high, patients may be vaccinated earlier & then reimmunized ≥11 months after intravenous immunoglobulin (IVIG) administration if the patient has an inadequate serological response
- Annual influenza vaccination is recommended in patients who are on long-term therapy w/ Aspirin to reduce the risk of Reye’s syndrome