1. In a systematic review of 74 studies reporting Kawasaki disease (KD) outcomes over years of follow-up, overall mortality estimates ranged from 0% to 6%.
2. Long-term outcomes were generally poorer in KD patients with coronary artery aneurysms, with estimated rates of survival without a major adverse cardiac event ranging from 36% to 96% at 30 years of follow-up.
Evidence Rating Level: 3 (Average)
Study Rundown: Kawasaki disease (KD) is an idiopathic acute systemic vasculitis that occurs in young children. When left untreated, it causes coronary artery aneurysms (CAAs) in about 25% of cases, and is therefore an important cause of chronic, acquired cardiac disease. This systematic review aimed to assess the long-term morbidity and mortality associated with KD and in particular the cardiovascular prognosis in patients who have KD without CAA. 74 studies of more than 72,000 patients were included in the review. 31% of studies had a follow-up duration between 1 and 5 years, while 15% had more than 15 years of follow-up. In studies reporting mortality, mortality rates were estimated to be between 0 and 6%. In patients with CAA, estimated mortality rates ranged from 0 to 25%. Rates of major adverse cardiac event-free survival in patients with CAA were estimated to be between 72 and 100% at 5 years follow-up and between 36 and 96% at 30 years. Most studies examining rates of early atherosclerosis found an increased risk in KD patients. The heterogeneity of the included studies and their outcomes prevented pooled statistical analysis in this review, which greatly limits the interpretation of the findings. Cardiac outcomes in KD patients without CAA were also found to be understudied, preventing the formation of any significant conclusions on this population. This review represents generally good prognoses for the overall KD population though with high variation between individual study samples.
Relevant Reading: Diagnosis, treatment, and long-term management of Kawasaki disease
In-Depth [systematic review]: Observational studies published between 1982 and 2020 reporting mortality as well as cardiac and noncardiac outcomes in children diagnosed with KD at 18 years old or younger were included in the review. CAA was treated as a risk factor for cardiac events rather than a cardiac outcome. 36 studies reported mortality, 55 a cardiac outcome, and 12 a noncardiac outcome. The majority of the mortality and cardiac outcome studies did not include a control group without KD. The prevalence of myocardial infarction ranged from 0 to 7% in studies of KD patients overall, and from 0 to 35% in patients with CAA. Most studies showed no increased risk of hypertension but increased risk of allergic disease in the overall KD population.
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