Background/Purpose: Coronary artery abnormalities (CAA) are a dreaded yet potentially preventable stigmata of Kawasaki disease (KD). Giant CAA are rare but at higher risk of complications and adverse outcomes. We aimed to evaluate the medium-term outcomes of children with KD with giant coronary aneurysms (Z score >10).

Methods: KD cases registered in our tertiary care centre were retrospectively reviewed, and data of children with giant CAA was analysed.

Results: Of the 34 KD cases in our register (2018-2024), we encountered 5 children (14.7%) with giant coronaries. All children presented as incomplete KD, with delayed immunomodulation and IVIg resistance. One patient had extensive systemic vascular involvement beyond the coronaries. The high proportion of children with giant coronaries as opposed to the reported literature might be due to a referral bias. The diagnosis of KD and initiation of immunomodulation took a median (IQR) of 12 (10-14) days. All received IVIg and oral steroids, with IV methylprednisolone pulse in two. All but one received primary intensification with Infliximab. CAAs persisted in all, noted during the 22 patient-years of follow-up. There was one mortality owing to a cardiovascular event. Our experience, although limited, was concordant with published literature suggesting that male sex, aneurysms at onset, delayed IVIG therapy and IVIg resistance are heralds of giant CAAs in KD. Similarly, systemic vascular involvement was associated with a poor outcome.

Conclusion: Incomplete KD may have a high propensity to develop giant coronaries, possibly refractory to immunomodulation. As is evident in our series, delayed diagnosis and immunomodulation (12 (10-14) days) are important factors determining CAA prognosis. Although rare, giant and systemic arterial aneurysms in KD are life-threatening and difficult to manage. They may progress despite appropriate immunomodulation.

CT images of children with Kawasaki disease with giant coronaries

Series of children with Kawasaki disease with giant coronaries

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/giant-coronaries-in-children-with-kawasaki-disease-a-single-centre-experience/