Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events
Authors: Masaru Miura, Tohru Kobayashi, Tetsuji Kaneko, Mamoru Ayusawa, Ryuji Fukazawa, Naoya Fukushima, Shigeto Fuse, Kenji Hamaoka, Keiichi Hirono, Taichi Kato, Yoshihide Mitani, Seiichi Sato, Shinya Shimoyama, Junko Shiono, Kenji Suda, Hiroshi Suzuki, Jun Maeda, Kenji Waki, Hitoshi Kato, Tsutomu Saji, Hiroyuki Yamagishi, Aya Ozeki, Masako Tomotsune, Makiko Yoshida, Yohei Akazawa, Kentaro Aso, Shouzaburoh Doi, Yoshi Fukasawa, Kenji Furuno, Yasunobu Hayabuchi, Miyuki Hayashi, Takafumi Honda, Norihisa Horita, Kazuyuki Ikeda, Masahiro Ishii, Satoru Iwashima, Masahiro Kamada, Masahide Kaneko, Hiroshi Katyama, Yoichi Kawamura, Atushi Kitagawa, Akiko Komori, Kenji Kuraishi, Hiroshi Masuda, Shinichi Matsuda, Satoshi Matsuzaki, Sayaka Mii, Tomoyuki Miyamoto, Yuji Moritou, Noriko Motoki, Kiyoshi Nagumo, Tsuneyuki Nakamura, Eiki Nishihara, Yuichi Nomura, Shohei Ogata, Hiroyuki Ohashi, Kenichi Okumura, Daisuke Omori, Tetsuya Sano, Eisuke Suganuma, Tsutomu Takahashi, Shinichi Takatsuki, Atsuhito Takeda, Masaru Terai, Manatomo Toyono, Kenichi Watanabe, Makoto Watanabe, Masaki Yamamoto, Kenichiro Yamamura
Journal: JAMA Pediatrics (2018)

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