Kawasaki Disease or Incomplete Kawasaki Disease Clinical Pathway — Emergency Department and Inpatient

Abnormal Echocardiogram Subsequent Treatment

Guidelines for assessment and management for patients of all ages with abnormal echocardiogram results (any coronary artery Z score of ≥ 2.5)

For all patients, monitor and manage as indicated based on response to initial treatment.

All patients with abnormal echo Consult Rheumatology.
Start prednisolone 2 mg/kg divided BID x 5 days then taper (if not already started on steroids)
Response to Initial Treatment Management and/or Treatment
  • Resistant
  • Fever > 38.0
  • OR
  • lingering clinical symptoms
    > 24-36 hours after completion of IVIG
  • Re-treat with IVIG 2 gram/kg
  • Re-check labs (CRP, CBC, LFTs, reticulocyte count, LDH) 12-24 hours after second dose of IVIG.
  • Discuss timing of repeat echo with Cardiology
  • If persistent fever or symptoms after IVIG, discuss further adjunctive treatment with Rheumatology.
  • Recommended subsequent adjunctive treatments for persistent symptoms after 24 hours of steroids:
    • Infliximab 5 mg/kg (consider repeat dose if persistent fever/symptoms 24 hours after first dose)
    • Consider Pulse methylprednisolone 30 mg/kg/dose Q24 (max 1 gram) x 3 days
  • Responsive
  • Afebrile < 38.0
  • AND
  • clinically improving ~48 hours after completion of IVIG (or adjunctive treatment)
  • Consider discharge but discuss with Cardiology and Rheumatology.
  • Consider repeat echocardiogram prior to discharge if significant coronary findings.