title: Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population.
abstract: 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.
  
  
  
  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 (zMax), 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 zMax 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).
  
  
  
  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.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA kevin.friedman@cardio.chboston.org.
authors.forename: Kevin G
authors.initials: KG
authors.lastname: Friedman
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Gauvreau
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA.
authors.forename: Akiko
authors.initials: A
authors.lastname: Hamaoka-Okamoto
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA.
authors.forename: Alexander
authors.initials: A
authors.lastname: Tang
authors.affiliation: Department of Pediatrics, University of California San Diego, La Jolla, CA Rady Children's Hospital San Diego, San Diego, CA.
authors.forename: Erika
authors.initials: E
authors.lastname: Berry
authors.affiliation: Department of Pediatrics, University of California San Diego, La Jolla, CA Rady Children's Hospital San Diego, San Diego, CA.
authors.forename: Adriana H
authors.initials: AH
authors.lastname: Tremoulet
authors.affiliation: Department of Pediatrics, University of California San Diego, La Jolla, CA Rady Children's Hospital San Diego, San Diego, CA.
authors.forename: Vidya S
authors.initials: VS
authors.lastname: Mahavadi
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Annette
authors.initials: A
authors.lastname: Baker
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Sarah D
authors.initials: SD
authors.lastname: deFerranti
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: David R
authors.initials: DR
authors.lastname: Fulton
authors.affiliation: Department of Pediatrics, Harvard Medical School, Boston, MA Rady Children's Hospital San Diego, San Diego, CA.
authors.forename: Jane C
authors.initials: JC
authors.lastname: Burns
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002318:Cardiovascular Diseases / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003323:Coronary Aneurysm / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D001026:Coronary Artery Bypass / Q000706:statistics & numerical data; D054059:Coronary Occlusion / Q000453:epidemiology; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D053208:Kaplan-Meier Estimate; D016013:Likelihood Functions; D016015:Logistic Models; D008279:Magnetic Resonance Imaging; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000188:drug therapy*; D015999:Multivariate Analysis; D009203:Myocardial Infarction / Q000453:epidemiology; D062645:Percutaneous Coronary Intervention / Q000706:statistics & numerical data; D016016:Proportional Hazards Models; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D012307:Risk Factors; D017180:Tachycardia, Ventricular / Q000453:epidemiology; D014057:Tomography, X-Ray Computed; D014481:United States
chemical_list: D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: Kawasaki disease; cardiovascular outcomes; coronary aneurysm