Background/Purpose: Kawasaki Disease (KD) is a common childhood vasculitis. Coronary artery aneurysms (CAAs) develop in approximately 25% of untreated KD patients. However, there is limited data on the long-term cardiovascular events following childhood KD diagnosis, especially in the post-IVIG era. We aimed to determine the cumulative incidence and incidence rate of myocardial infarction (MI), heart failure (HF), and cardiac arrest, referred to as cardiovascular events and mortality following KD diagnosis.

Methods: We performed a literature search using 5 databases until 2022. We retrieved English articles for review including randomized-controlled trials, cross-sectional studies, case-control studies, cohort studies, and abstracts. The search produced 181 full-text articles for data extraction (Figure 1). The Newcastle-Ottawa Scale assessed study quality focusing on participant selection, comparability, and measurement of outcome or ascertainment of exposure. The study was submitted to PROSPERO on 23 November 2019 and registered on 28 April 2020.

Results: Among 392,848 KD patients across 181 studies, there were 1548 and 1090 cases of cardiovascular events (MI, heart failure, or cardiac arrest) and death, respectively. The median follow-up period of studies included was 60 months. Meta-analysis generated a cumulative incidence of cardiovascular events of 0.85% and death of 0.24%. Subgroup analysis for CAA presence and IVIG use was performed. Among studies where all participants had CAAs, the cumulative incidence was 3.5% and 0.99% for cardiovascular events and death, respectively. For studies where none or few of the participants had CAAs, the cumulative incidence was 0.02% and 0.03% for cardiovascular events and death, respectively (Figure 2). Among studies where all participants received IVIG, the cumulative incidence was 0.05% and 0.14% for cardiovascular events and death, respectively. For studies where none or few of the participants received IVIG the cumulative incidence was 2.3% and 0.63% for cardiovascular events and death, respectively (Figure 3).

Conclusion: This study comprises the largest systematic review and meta-analysis of long-term cardiovascular outcomes and mortality following childhood KD diagnosis. The low prevalence of mortality (< 1%) and lower prevalence of cardiovascular events for patients without CAAs can provide reassurance to families. Those with CAAs, however, have higher prevalence of cardiovascular events which highlights the need for CAA surveillance, monitoring, and potential intervention to prevent development of these events. Further studies are needed to better define the risk of long-term cardiovascular events and mortality following childhood KD diagnosis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-effect-of-coronary-artery-aneurysms-and-ivig-use-on-long-term-cardiovascular-disease-and-mortality-following-kawasaki-disease-in-childhood-a-systematic-review-and-meta-analysis/