115 - FACTORS ASSOCIATED WITH EARLY REGRESSION OF GIANT CORONARY ARTERY ANEURYSMS IN KAWASAKI DISEASE

BACKGROUND. KD patients with giant coronary artery aneurysms (CAA) have high risks of cardiovascular complications. In some patients, giant CAAs regress to small CAA or normal sized coronaries, but this varies between patients, and factors that favor early regression of giant CAAs remain unclear. In a retrospective single-centre study, we investigated patient factors and treatment characteristics that correlated with earlier regression of giant CAAs.

METHODS. We included all KD patients who developed giant aneurysms (defined as CAA with maximal coronary artery z score ≥ +10, as per 2017 American Heart Association guidelines), treated at a UK tertiary centre between 2003-2022. Demographic and clinical information was extracted. Patients were divided into two groups as follows. In the early regression (ER) group, CAA reduced to z-score < +5 within 12 months. In the late regression (LR) group, z-score remained ≥ +5 at 12 months. Numerical data were analysed with unpaired t tests and categorical data with Fisher’s exact test. Factors associated with early regression were analysed with univariate logistic regression. P value < 0.05 was considered significant.

RESULTS. 19 children met the inclusion criteria. Median age was 1.4 years (range 0.16-9), 14/19 were males and 10/19 non-white. The median duration of fever at presentation was 12 days (range 4-21). 12/19 (63%) of patients presented with incomplete KD. All patients received IVIG and anticoagulation treatment (low molecular weight heparin, and subsequently warfarin if giant CAA persisted). Adjunctive anti-inflammatory therapy included high-dose steroids (5/19), infliximab 5mg/kg (15/19), anakinra (8/19) and/οr ciclosporin (4/19). The ER and LR groups included 10 and 9 children respectively. The maximum z-score of CAA, duration of fever at presentation and day of IVIG treatment did not differ between the groups. The ER group received adjunctive treatment with infliximab sooner after IVIG [mean ± SD: 2.4 (±1.5) vs 7.1 (±5.4) days, p =0.04), with an odds ratio favoring ER of 18.0 (Confidence Interval (CI): 1.3-232.6]) for patients treated within 4 days. The ER group also had a shorter duration of inflammation (days with CRP >10mg/dL) [mean ± SD]: 16.8 (±4.5) vs 28.3 (±13.8) days, p=0.03, with odds ratio of 0.01 (CI 0.01-0.88) for patients with CRP >10mg/dL for > 4 weeks.

CONCLUSIONS. In our centre, there was heterogenous management of KD with giant CAA during the study period, with varying outcomes. Earlier treatment with infliximab after IVIG and short duration of inflammation were significant factors favoring earlier regression of giant CAA.