Factors Associated with Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated with COVID-19

Supriya S Jain; Ashraf S Harahsheh; Simon Lee; Geetha Raghuveer; Nagib Dahdah; Michael Khoury; Michael A Portman; Melissa Wehrmann; Arash A Sabati; Marianna Fabi; Deepika Thacker; Nilanjana Misra; Mark D Hicar; Nadine F Choueiter; Matthew D Elias; Audrey Dionne; William B Orr; Jacqueline R Szmuszkovicz; Seda Selamet Tierney; Luis Martin Garrido-Garcia; Frederic Dallaire; Balasubramanian Sundaram; Deepa Prasad; Tyler H Harris; Elizabeth Braunlin; Elisa Fernandez Cooke; Cedric Manlhiot; Pedrom Farid; Brian W McCrindle; International Kawasaki Disease Registry

doi:10.1016/j.cjca.2024.11.027

Factors Associated with Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated with COVID-19

Can J Cardiol. 2024 Nov 30:S0828-282X(24)01233-9. doi: 10.1016/j.cjca.2024.11.027. Online ahead of print.

Authors

Supriya S Jain¹, Ashraf S Harahsheh², Simon Lee³, Geetha Raghuveer⁴, Nagib Dahdah⁵, Michael Khoury⁶, Michael A Portman⁷, Melissa Wehrmann⁸, Arash A Sabati⁹, Marianna Fabi¹⁰, Deepika Thacker¹¹, Nilanjana Misra¹², Mark D Hicar¹³, Nadine F Choueiter¹⁴, Matthew D Elias¹⁵, Audrey Dionne¹⁶, William B Orr¹⁷, Jacqueline R Szmuszkovicz¹⁸, Seda Selamet Tierney¹⁹, Luis Martin Garrido-Garcia²⁰, Frederic Dallaire²¹, Balasubramanian Sundaram²², Deepa Prasad²³, Tyler H Harris²⁴, Elizabeth Braunlin²⁵, Elisa Fernandez Cooke²⁶, Cedric Manlhiot²⁷, Pedrom Farid²⁸, Brian W McCrindle²⁸; International Kawasaki Disease Registry

Collaborators

Affiliations

¹ Department of Pediatrics, Division of Cardiology, New York Medical College-Maria Fareri Children's Hospital at Westchester Medical Center, Valhalla, NY, USA. Electronic address: Sjain7@nymc.edu.
² Division of Cardiology, Department of Pediatrics, Children's National Hospital, The George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
³ The Heart Center, Nationwide Children's Hospital, Columbus, OH, USA.
⁴ Children's Mercy Hospital, Kansas City, MO, USA.
⁵ Division of Pediatric Cardiology, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada.
⁶ Department of Pediatrics, University of Alberta. Edmonton, AB, Canada.
⁷ Seattle Children's Research Institute, Seattle, WA, USA.
⁸ Children's Nebraska, Omaha, NE, USA.
⁹ Phoenix Children's, Phoenix, AZ, USA.
¹⁰ Pediatric Emergency Unit, IRCCS Azienda Ospedaliero-universitaria di Bologna, 40138 Bologna, Italy.
¹¹ Nemours Children's Hospital, Delaware, Wilmington, DE, USA.
¹² Northwell, New Hyde Park, Cohen Children's Medical Center, NY, USA.
¹³ Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA.
¹⁴ Children's Hospital at Montefiore, Bronx, NY, USA.
¹⁵ Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
¹⁶ Department of Cardiology, Boston Children's Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
¹⁷ Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO, USA.
¹⁸ Children's Hospital Los Angeles, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
¹⁹ Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA, USA.
²⁰ Hospital Angeles Lomas, Huixquilucan, Mexico / Instituto Nacional de Pediatria, Mexico City, Mexico.
²¹ Universite de Sherbrooke and Centre de recherche du Centre hospitalier universitaire de Sherbrooke, Sherbrooke, QC, Canada.
²² Kanchi Kamakoti Childs Trust Hospital, Chennai, 600034, India.
²³ Banner Children's Hospital at Desert Medical Center, Mesa, AZ, USA.
²⁴ UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
²⁵ University of Minnesota Masonic Children's Hospital, Minneapolis, MN.
²⁶ Hospital Universitario 12 de Octubre, Madrid, Spain.
²⁷ Blalock-Taussig-Thomas Congenital Heart Center at Johns Hopkins University, Baltimore, MD, USA.
²⁸ Labatt Family Heart Centre, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, ON, Canada.

PMID: 39622342
DOI: 10.1016/j.cjca.2024.11.027

Abstract

Background: While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

Methods: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from 01/2020 to 01/2023. Demographics, clinical features and presentation, management, laboratory values, and outcomes were compared between the diagnosis/shock groups.

Results: Shock at presentation was noted for 19 of 672 (2.8%) KD patients and 653 of 1472 (44%; p<0.001) MIS-C patients. Within both groups, patients with shock were significantly more likely to be admitted to the intensive care unit, receive inotropes and to have greater laboratory abnormalities indicative of hyperinflammation and organ dysfunction, including abnormal cardiac biomarkers. Patients with KD and shock had a greater maximum coronary artery Z score (median +2.62) versus KD patients without shock (+1.36; p<0.001) and MIS-C patients with shock (+1.45; versus +1.32 for those without shock; p<0.001). They were also more likely to have large coronary artery aneurysms. In contrast, MIS-C patients with shock had lower left ventricular ejection fraction (mean 51.6%) versus MIS-C patients without shock (56.6%; p<0.001) and KD patients with shock (56.7%; versus 62.8% for those without shock; p=0.04).

Conclusions: While patients with KD presenting with shock are clinically similar to patients with MIS-C, especially those with shock, they have more severe coronary artery involvement, while MIS-C patients with shock have lower left ventricular ejection fraction.