Abstract 189: Natural History of Coronary Artery Aneurysms in Kawasaki Disease in US population and Risk Factors for Persistent Aneurysms
Background: The late natural history of coronary artery aneurysms (CAA) after IVIG treatment in the US is not well described.
Methods: We evaluated all KD patients (pts) at 2 centers from 1984-2014. Entry criteria were: 1) IVIG treatment; 2) CAA, defined as LAD or RCA z-score ≥ 3 or Japanese Ministry of Health criteria; and 3) ≥1 follow-up (f/u) echo. Kaplan Meier curves evaluated time to CAA regression (z < 2.5) and Cox regression examined factors associated with persistent CAA and major adverse cardiac events (MACE= death, MI, CABG, PCI, occluded CA).
Results: Of 2592 KD pts, 408 (15%) met entry criteria and were 72% male; 54% white, 21% Asian, 7% black, 18% other race. Median age at fever onset was 1.8 y [IQR: 0.7-4.4y], 74% had complete KD, and fever days before 1st IVIG were 7d [IQR 6-10d]. IVIG retreatment occurred in 35% and adjunctive anti-inflammatory therapy in 37%, both increased over time (p<.001). LAD and RCA CAA occurred in 31%, LAD alone in 47% and RCA alone in 22%. Median z-scores at CAA diagnosis were: LAD 3.61 (IQR 3.1-5.1) and RCA 3.1 (1.7-4.1) with 93 (23%) pts having giant CAA (z ≥10).
Over median f/u of 2.6 y (0.01-29.0y), 313 (77%) had CAA regression at median of 1.1 mo (IQR =0.3-16.9mo). Univariate risk factors for CAA persistence were z ≥ 8 at diagnosis (HR 0.22, p <.001), earlier era (2010-2014 HR =1.0 , 2000-2009 HR = 0.60, 1990-1999 HR =0.38, 1984-1989=0.15, all p<.001), multi-vessel CAA (LCA alone HR =1, RCA alone HR =0.99 p=.99, LCA+RCA HR=0.35, p<.001) IVIG retreatment (HR 0.68, p=.003), IVIG treatment >Day 10 (HR 0.46, p<0.001), adjunctive anti-inflammatory therapy (HR 0.65, p=.03), and non-Asian race (HR 0.64, p=.001). Multivariable model for persistent CAA included earlier era (p<.001), z-score >8 at diagnosis (p<.001), multi-vessel CAA (<.001) and treatment after Day 10 (p=.01). MACE occurred in 25 (1%) pts, including 3 deaths, with univariate risk factors of CAA z-score ≥ 8 (p<.001), earlier era (p<.001), treatment after 10 d (p=.03), and non-Asian race (p=.03)
Conclusion: Most CAA regress within the first year after treatment. Persistent CAA are more likely in pts with larger CAA, multi-vessel CAA and late IVIG treatment. In pts with large CAA, time to regression has gotten shorter in more recent eras, possibly related to greater use of adjunctive therapies.
Author Disclosures: K.G. Friedman: None. A. Hamaoko-Okamoto: None. K. Gauvreau: None. S. de Ferranti: None. A. Baker: None. D. Fulton: None. A. Tremoulet: None. A. Tang: None. E. Berry: None. V.S. Mahavadi: None. J. Burns: None. J. Newburger: None.
- © 2015 by American Heart Association, Inc.