Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

William B Orr¹, Marianna Fabi², Michael Khoury³, Frederic Dallaire⁴, Deepika Thacker⁵, Matthew D Elias⁶, Nadine F Choueiter⁷, Sean M Lang⁸, Nagib Dahdah⁹, Ashraf S Harahsheh¹⁰, Todd T Nowlen¹¹, Mahmoud Alsalehi¹², Nilanjana Misra¹³, Audrey Dionne¹⁴, Paul Dancey¹⁵, Simon Lee¹⁶, Geetha Raghuveer¹⁷, Kambiz Norozi¹⁸, Jacqueline R Szmuszkovicz¹⁹, Tapas Mondal²⁰, Mark D Hicar²¹, Manaswitha Khare²², Seda Tierney²³, Michael A Portman²⁴, Melissa Wehrmann²⁵, Michelle Grcic²⁶, Balasubramanian Sundaram²⁷, Mona El Ganzoury²⁸, Deepa Prasad²⁹, Tyler H Harris³⁰, Ana Caro-Barri³¹, Luis Martin Garrido-Garcia³², Elizabeth Braunlin³³, Daniel Mauriello³⁴, Kimberly E McHugh³⁵, Brian W McCrindle³⁶; Onbehalf of the International Kawasaki Disease Registry

Affiliations

¹ Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO, USA. worr@wustl.edu.
² Pediatric Emergency Unit, IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy.
³ Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
⁴ Department of Pediatrics, Université de Sherbrooke, Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, QC, Canada.
⁵ Nemours Children's Health, Wilmington, DE, USA.
⁶ Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁷ Kravis Children's Hospital at Mount Sinai, New York, NY, USA.
⁸ Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
⁹ Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, QC, Canada.
¹⁰ Division of Cardiology, Department of Pediatrics, Children's National Hospital, The George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
¹¹ Phoenix Children's, Phoenix, AZ, USA.
¹² Kingston Health Sciences Centre, Queens University, Kingston, ON, Canada.
¹³ Division of Pediatric Cardiology, Cohen Children's Medical Center, Northwell Health, New Hyde Park, NY, USA.
¹⁴ Department of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
¹⁵ Janeway Children's Health and Rehabilitation Centre, St. John's, NL, Canada.
¹⁶ Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
¹⁷ Children's Mercy Hospital, Kansas City, MO, USA.
¹⁸ Department of Pediatrics, Schulich School of Medicine and Dentistry, Western University, Children's Health Research Institute, London, ON, Canada.
¹⁹ Children's Hospital of Los Angeles, Los Angeles, CA, USA.
²⁰ Division of Cardiology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, ON, Canada.
²¹ Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA.
²² University of California San Diego/Rady Children's Hospital, San Diego, CA, USA.
²³ Lucile Packard Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
²⁴ Seattle Children's Research Institute, Seattle, WA, USA.
²⁵ Children's Nebraska, Omaha, NE, USA.
²⁶ The Heart Center at Nationwide Children's Hospital, Columbus, OH, USA.
²⁷ Kanchi Kamakoti Childs Trust Hospital, Chennai, India.
²⁸ Ain Shams University, Cairo, Egypt.
²⁹ Banner Children's Hospital, Mesa, AZ, USA.
³⁰ UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
³¹ Hospital Universitario 12 de Octubre, Madrid, Spain.
³² Hospital Angeles Lomas, Hacienda de Las Palmas, Mexico, Mexico.
³³ University of Minnesota, Minneapolis, MN, USA.
³⁴ Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA.
³⁵ Medical University of South Carolina, Charleston, SC, USA.
³⁶ Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

PMID: 40353863
DOI: 10.1007/s00246-025-03888-4

Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

William B Orr et al. Pediatr Cardiol. 2025.

. 2025 May 12.

doi: 10.1007/s00246-025-03888-4. Online ahead of print.

Authors

Affiliations

¹ Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO, USA. worr@wustl.edu.
² Pediatric Emergency Unit, IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy.
³ Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
⁴ Department of Pediatrics, Université de Sherbrooke, Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, QC, Canada.
⁵ Nemours Children's Health, Wilmington, DE, USA.
⁶ Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁷ Kravis Children's Hospital at Mount Sinai, New York, NY, USA.
⁸ Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
⁹ Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, QC, Canada.
¹⁰ Division of Cardiology, Department of Pediatrics, Children's National Hospital, The George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
¹¹ Phoenix Children's, Phoenix, AZ, USA.
¹² Kingston Health Sciences Centre, Queens University, Kingston, ON, Canada.
¹³ Division of Pediatric Cardiology, Cohen Children's Medical Center, Northwell Health, New Hyde Park, NY, USA.
¹⁴ Department of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
¹⁵ Janeway Children's Health and Rehabilitation Centre, St. John's, NL, Canada.
¹⁶ Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
¹⁷ Children's Mercy Hospital, Kansas City, MO, USA.
¹⁸ Department of Pediatrics, Schulich School of Medicine and Dentistry, Western University, Children's Health Research Institute, London, ON, Canada.
¹⁹ Children's Hospital of Los Angeles, Los Angeles, CA, USA.
²⁰ Division of Cardiology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, ON, Canada.
²¹ Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA.
²² University of California San Diego/Rady Children's Hospital, San Diego, CA, USA.
²³ Lucile Packard Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
²⁴ Seattle Children's Research Institute, Seattle, WA, USA.
²⁵ Children's Nebraska, Omaha, NE, USA.
²⁶ The Heart Center at Nationwide Children's Hospital, Columbus, OH, USA.
²⁷ Kanchi Kamakoti Childs Trust Hospital, Chennai, India.
²⁸ Ain Shams University, Cairo, Egypt.
²⁹ Banner Children's Hospital, Mesa, AZ, USA.
³⁰ UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
³¹ Hospital Universitario 12 de Octubre, Madrid, Spain.
³² Hospital Angeles Lomas, Hacienda de Las Palmas, Mexico, Mexico.
³³ University of Minnesota, Minneapolis, MN, USA.
³⁴ Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA.
³⁵ Medical University of South Carolina, Charleston, SC, USA.
³⁶ Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

PMID: 40353863
DOI: 10.1007/s00246-025-03888-4

Abstract

Non-coronary artery systemic arterial aneurysms (SAAs) are rare and an under-reported sequelae of Kawasaki disease (KD). We hypothesize that practices regarding SAA screening and management vary widely among experts and published literature. A survey was sent to members of the International KD Registry regarding their experiences and practices with SAAs in KD patients. For comparison, a systematic scoping review was conducted using PRISMA methodology, from which 25 reports with 83 patients were included. Results from each were compared. Surveys were completed by 48 (56%) of 86 IKDR investigators; 35 (73%) respondents had > 10 years of experience caring for KD patients. However, 33% of respondents had not cared for a patient with SAA. Features prompting assessment for SAA included demographics, presence and degree of coronary artery (CA) involvement, and clinical features, including prolonged/persistent fever, progressing/persistent elevation of inflammatory markers, and resistance to standard treatment. Features prompting screening were somewhat concordant with the characteristics of patients with SAA identified in the scoping review. From the survey, the initial preferred assessment included computed tomographic angiography (48%), ultrasound (29%), and magnetic resonance imaging (24%). In contrast, assessment of patients with SAA from the scoping review commonly used multiple imaging modalities. SAA often regressed, but associated complications included thrombosis, calcification, stenosis, occlusion, and collateral formation. While SAA is a known but rare complication of acute KD, there remains a gap in evidence regarding which patients are at risk, best practices for screening and management, and outcomes. Prospective cohort studies are needed.

Keywords: Aneurysms; Kawasaki disease; Mucocutaneous lymph node syndrome; Pediatrics; Surveillance.

PubMed Disclaimer

Conflict of interest statement

Declarations. Conflict of interest: The authors have no conflicts of interest to disclose, and no funding was secured for this study.

References

1. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M et al (2017) Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 135(17):927–999 - DOI
1. Burns JC, Glodé MP (2004) Kawaski syndrome. The Lancet 364(9433):533–544 - DOI
1. Kawasaki T (1967) Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Jpn J Allergy 16:178–222
1. Orenstein JM, Shulman ST, Fox LM, Baker SC, Takahashi M, Bhatti TR et al (2012) Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. PLoS ONE 7(6):e38998 - DOI - PubMed - PMC
1. Zhao Q-m, Chu C, Wu L, Liang X-c, Sun S-n, He L et al (2019) Systemic artery aneurysms and Kawasaki disease. Pediatrics. https://doi.org/10.1542/peds.2019-2254 - DOI - PubMed - PMC

LinkOut - more resources

Full Text Sources
- Springer
Miscellaneous
- NCI CPTAC Assay Portal

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

Affiliations

Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

Authors

Affiliations

Abstract

Conflict of interest statement

References

LinkOut - more resources

Full Text Sources

Miscellaneous