78 - EARLY USE OF ADJUNCTIVE THERAPY TO REDUCE CORONARY ARTERY DAMAGE FROM KAWASAKI DISEASE IN HIGH-RISK INFANTS LESS THAN 6 MONTHS OF AGE: A TWO-CENTER EXPERIENCE

Background:    

Children with KD who are untreated or receive late treatment after 10 days of illness are at increased risk of CA abnormalities. Infants < 6 months of age with KD are at highest risk of this cardiac complication regardless of timing of treatment.  

Method:    

In this two-center, retrospective study, we analyzed all infants < 6 months of age admitted to Rady Children’s Hospital San Diego (n=49) and to Children’s Hospital Colorado (n=23) with KD meeting AHA criteria who were treated within 10 days from fever onset between 1/1/2014-12/1/2023. Data were extracted from our REDCap database at the Kawasaki Disease Research Center in La Jolla, CA and from medical records at both institutions. Parents gave signed consent.  

Results:     

Seventy-two infants (54.1% female; 25 Hispanic, 14 Asian, 13 Caucasian, 11 Mixed, 5 Black, 2 Other, 1 Asian Pacific Islander, 1 unknown) met inclusion criteria. A coronary artery Z-score >2.0 in the left anterior descending (LAD) and/or right coronary artery (RCA) was documented on the initial echo in 40/72 (55.6%) patients. Initial Z-scores for the proximal LAD and/or RCA were as follows: >2 to ≤2.5, n= 18; >2.5 to ≤5, n= 36; >5 to ≤10, n=4; >10, n=1. Initial therapy was intensified in 39/40 (97.5%) patients with Z score >2.0: IVIG+infliximab (IFX), n=23; IVIG+IFX+cyclosporine (CSA), n=4; IVIG+IVIG+IFX, n=3; IVIG+ IFX+anakinra (ANA), n=3; IVIG+IFX+ANA+CSA, n=3; IVIG+IVIG+IFX+CSA, n=1; IVIG+IVIG, n=1; IVIG+IFX+IFX, n=1. Therapy was intensified in 17 additional patients because of increasing trend in Z-score, n=9; IVIG-resistance, n=5; severity of disease, n=3. 

Echos at the closest interval to one-year follow-up, or last echo in the system were assessed for CA outcome. The median time from the initial echo was 13.1 months (IQR=1,13 months). At that time, eleven patients had residual coronary dilation. Eight patients had mild LAD dilation: Z-score >2 to ≤2.5, n=5; LAD Z-score >2.5 to 3, n=3. Three patients had mild RCA dilation: Z-score >2 to ≤2.5, n=2; RCA Z-score 2.7, n=1. Despite average Zmax LAD +2.6 (range +0.1 to +12.4) and average Zmax RCA +2.6 (range +0.1 to +11.9), no patients in this high-risk cohort developed persistent large/giant aneurysms. 

Conclusions:    

Timely diagnosis and intensification of initial therapy for patients with CA Z score >2 may have resulted in the favorable outcome in this cohort of infants < 6 months of age managed in two different institutions.