Long-term Cardiovascular Outcomes in Survivors of Kawasaki Disease

BACKGROUND AND OBJECTIVE: Kawasaki disease (KD) may result in coronary aneurysm formation, but there is incomplete knowledge regarding its long-term effects. Our objective was to quantify the longer-term rates of adverse cardiac events in a modern North American KD cohort. METHODS: Using the Kaiser Permanente Northern California population, we performed a retrospective cohort study in patients with a history of KD versus matched patients without KD. Chart review was used to confirm the diagnosis of KD and all outcomes of interest, including acute coronary syndrome, coronary revascularization, heart failure, ventricular arrhythmia, valve disease, aortic aneurysm, and all-cause mortality. All outcomes occurring at age ≥15 years were included in the primary analysis. Outcome rates were compared between the 2 groups by using Cox proportional hazards analysis. RESULTS: The study included 546 KD patients and 2218 matched patients without KD. Seventy-nine percent of the KD patients received intravenous immunoglobulin and 5% had persistent coronary aneurysm. The average follow-up time was 14.9 years. Only 2 KD patients experienced outcomes after age 15 (0.246 events per 1000 person-years) compared with 7 events in the non-KD group (0.217 events per 1000 person-years), a nonsignificant difference (hazard ratio: 0.81; 95% confidence interval: 0.16–4.0). Within the KD subgroup, persistent coronary aneurysm predicted the occurrence of adverse events ( P = .007). CONCLUSIONS: This is the largest US study of longer-term cardiac outcomes after KD and reveals a low rate of adverse cardiovascular events through age 21. Additional validation studies, including studies with longer-term follow-up, should be performed.