Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms

Elif Seda Selamet Tierney, Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, School of Medicine, Palo Alto, CA. Electronic address: tierneys@stanford.edu.
Kyle Runeckles, Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Adriana H. Tremoulet, Department of Pediatrics, University of California San Diego, Rady Children's Hospital-San Diego, San Diego, CA.
Nagib Dahdah, Division of Pediatric Cardiology, Centre Hospitalier Universitaire Ste-Justine, University of Montreal, Montreal, Quebec, Canada.
Michael A. Portman, Seattle Children's Research Institute, Seattle, WA.
Andrew S. Mackie, Stollery Children's Hospital, Edmonton, Alberta, Canada.
Ashraf S. Harahsheh, Pediatrics-Cardiology, Children's National Hospital/George Washington University School of Medicine, Washington, DC.
Sean M. Lang, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Nadine F. Choueiter, Children's Hospital at Montefiore, New York, NY.
Jennifer S. Li, Duke University Medical Center, Durham, NC.
Cedric Manlhiot, Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Tisiana Low, Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Mathew Mathew, Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Kevin G. Friedman, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Geetha Raghuveer, Children's Mercy Hospital, Kansas City, MO.
Kambiz Norozi, Department of Pediatrics, Western University, London, Canada.
Jacqueline R. Szmuszkovicz, Children's Hospital of Los Angeles, Los Angeles, CA.
Brian W. McCrindle, Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.

Document Type

Journal Article

Publication Date

1-1-2022

Journal

The Journal of pediatrics

Volume

240

DOI

10.1016/j.jpeds.2021.08.072

Keywords

Kawasaki; management; variation

Abstract

OBJECTIVE: To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). STUDY DESIGN: Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. RESULTS: We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.5-4.99 in 848, 5.0-9.99 in 349, and ≥10.0 (large/giant) in 430 patients. All centers reported IVIG and acetylsalicylic acid (ASA) as primary therapy and use of additional IVIG or steroids as needed. In 23 out of 30 centers, (77%) infliximab was also used; 11 of these 23 centers reported using it in <10% of their patients, and 3 centers used it in >20% of patients. Nonsteroidal anti-inflammatory agents were used in >10% of patients in only nine centers. Beta-blocker (8.8%, all patients) and abciximab (3.6%, all patients) were mainly prescribed in patients with large/giant CAAs. Statins (2.7%, all patients) were mostly used in one center and only in patients with large/giant CAAs. ASA was the primary antiplatelet modality for 99% of patients, used in all centers. Clopidogrel (18%, all patients) was used in 24 centers, 11 of which used it in >50% of their patients with large/giant CAAs. CONCLUSIONS: In the IKDR, IVIG and ASA therapy as primary therapy is universal with common use of a second dose of IVIG for persistent fever. There is practice variation among centers for adjunctive therapies and anticoagulation strategies, likely reflecting ongoing knowledge gaps. Randomized controlled trials nested in a high-quality collaborative registry may be an efficient strategy to reduce practice variation.

Department

Pediatrics

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