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Original Investigation
May 7, 2018

Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events

Author Affiliations
  • 1Department of Cardiology, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan
  • 2Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan
  • 3Division of Clinical Research Planning, Department of Development Strategy, Center for Clinical Research and Development, National Center for Child Health and Development, Tokyo, Japan
  • 4Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan
  • 5Department of Pediatrics, Nippon Medical School, Tokyo, Japan
  • 6Department of Pediatrics, NTT Sapporo Medical Center, Hokkaido, Japan
  • 7Department of Pediatric Cardiology and Nephrology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
  • 8Department of Pediatrics, Graduate School of Medicine, University of Toyama, Toyama, Japan
  • 9Division of Clinical Research Planning, Department of Development Strategy, Center for Department of Pediatrics, Nagoya University Hospital, Aichi, Japan
  • 10Department of Pediatrics, Mie University Graduate School of Medicine, Mie, Japan
  • 11Department of Pediatrics, Niigata City General Hospital, Niigata, Japan
  • 12Department of Pediatrics, Gunma University Graduate School of Medicine, Gunma, Japan
  • 13Department of Pediatric Cardiology, Ibaraki Children's Hospital, Ibaraki, Japan
  • 14Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan
  • 15Department of Pediatrics, Niigata University, Niigata, Japan
  • 16Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
  • 17Department of Pediatrics, Kurashiki Central Hospital, Okayama, Japan
JAMA Pediatr. 2018;172(5):e180030. doi:10.1001/jamapediatrics.2018.0030
Key Points

Questions  Is the internal diameter z score associated with time-dependent coronary events in patients with Kawasaki disease with coronary artery aneurysms?

Findings  This cohort study surveyed 1006 patients with Kawasaki disease younger than 19 years who received a coronary angiography and found that the 10-year event-free survival rate for coronary events was 100%, 94%, and 52% in men and 100%, 100%, and 75% in women for small, medium, and large aneurysms, respectively. Large aneurysms, male sex, and resistance to intravenous immunoglobulin therapy were associated with coronary events.

Meaning  Careful management is essential for men and treatment-resistant patients with large coronary artery aneurysms based on the internal diameter z score.

Abstract

Importance  Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA).

Objective  To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA.

Design, Setting, and Participants  This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011.

Main Outcomes and Measures  The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications.

Results  Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1–15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7–4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4–3.6) were significantly associated with CE.

Conclusions and Relevance  Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.

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