Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population

Abstract

Background The natural history of coronary artery aneurysms ( CAA ) after intravenous immunoglobulin ( IVIG ) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease ( KD ) patients and identify factors associated with major adverse cardiac events ( MACE ) and CAA regression. Methods and Results We evaluated all KD patients with CAA at 2 centers from 1979 to 2014. Factors associated with CAA regression, maximum CA z ‐score over time ( z Max), and MACE were analyzed. We performed a matched analysis of treatment effect on likelihood of CAA regression. Of 2860 KD patients, 500 (17%) had CAA , including 90 with CAA z ‐score >10. Most (91%) received IVIG within 10 days of illness, 32% received >1 IVIG , and 27% received adjunctive anti‐inflammatory medications. CAA regression occurred in 75%. Lack of CAA regression and higher CAA z Max were associated with earlier era, larger CAA z ‐score at diagnosis, and bilateral CAA in univariate and multivariable analyses. MACE occurred in 24 (5%) patients and was associated with higher CAA z ‐score at diagnosis and lack of IVIG treatment. In a subset of patients (n=132) matched by age at KD and baseline CAA z ‐score, those receiving IVIG plus adjunctive medication had a CAA regression rate of 91% compared with 68% for the 3 other groups ( IVIG alone, IVIG ≥2 doses, or IVIG ≥2 doses plus adjunctive medication). Conclusions CAA regression occurred in 75% of patients. CAA z ‐score at diagnosis was highly predictive of outcomes, which may be improved by early IVIG treatment and adjunctive therapies.