Kawasaki%20disease Management

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

• 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