A survey of the 3-decade outcome for patients with giant aneurysms caused by Kawasaki disease.
BACKGROUND
Our purpose was to determine the outcome in patients with a more-than-20-year history of giant coronary aneurysms (GAs) caused by Kawasaki disease (KD).


METHODS
Between 2010 and 2011, the incidence and outcome of cardiac events (CEs) in patients with GA was surveyed by questionnaire by the Kinki area Society of KD research. Death, acute myocardial infarction (AMI), coronary artery bypass grafting (CABG), percutaneous coronary catheter intervention, syncope, and ventricular tachycardia were considered as CEs. Survival rate and CE-free rate were analyzed by the Kaplan-Meier method.


RESULTS
We enrolled 245 patients (187 were male, 58 were female), 141 with bilateral GA and 104 with unilateral GA. The interval between the onset of acute KD to the time of survey ranged from 0.2 to 51 years, and the median was 20 years. Death, AMI, and CABG occurred in 15 (6%), 57 (23%), and 90 patients (37%), respectively. The CE-free rate and the survival rate at 30 years after KD were 36% (95% CI 28-45) and 90% (95% CI 84-94), respectively. The 30-year survival rate for bilateral GA was 87% (95% CI 78-93), and for unilateral GA, it was 96% (95% CI 85-96; hazard ratio 4.60, 95% CI 1.27-29.4, P = .027). The 30-year survival rate in patients with AMI was 49% (95% CI 27-71), and the 25-year survival rate in patients undergoing CABG was 92% (95% CI 81-98).


CONCLUSIONS
The outcome differed significantly between bilateral GA and unilateral GA. The results focus attention on the need to preserve myocardial perfusion, especially in high-risk patients with bilateral GA. An understanding of the optimal CABG would be useful in bilateral GA.
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan. Electronic address: etsuda@hsp.ncvc.go.jp.
Etsuko
E
Tsuda
Department of Pediatric Cardiology and Nephrology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Kenji
K
Hamaoka
Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Hiroyuki
H
Suzuki
Department of Pediatric Cardiology, Hyogo Prefectural Amagasaki Hospital, Amagasaki, Japan.
Hisanori
H
Sakazaki
Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan.
Yosuke
Y
Murakami
Department of Pediatrics, Shiga Medical University, Shiga, Japan.
Masao
M
Nakagawa
Department of Pediatrics, Kochi Medical University, Nankoku, Japan.
Hisashi
H
Takasugi
Department of Pediatric Cardiology, Nara Hospital, Kinki University, Nara, Japan.
Muneo
M
Yoshibayashi
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003323:Coronary Aneurysm / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology*; D017023:Coronary Angiography; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000175:diagnosis; D011159:Population Surveillance; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D015996:Survival Rate / Q000639:trends; D055815:Young Adult