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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.

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