The Long-term Cardiac and Noncardiac Prognosis of Kawasaki Disease: A Systematic Review

Abstract

CONTEXT It is uncertain if children with Kawasaki Disease (KD) are at risk for non-cardiac diseases and if children with KD but without coronary artery aneurysms (CAA) are at risk for long-term cardiac complications. OBJECTIVE To determine the long-term mortality and prognosis of children after KD. DATA SOURCES Medline, Embase, and the Cochrane Central Register. STUDY SELECTION Controlled trials and observational studies were included if they included children with KD and reported mortality, major adverse cardiovascular events (MACE), chronic cardiac or other disease over an average follow-up of ≥1 year. DATA EXTRACTION Data extracted included sample size, age at diagnosis, the proportion with coronary artery aneurysms (CAA), follow-up duration, and outcome(s). RESULTS Seventy-four studies were included. Thirty-six studies reported mortality, 55 reported a cardiac outcome, and 12 reported a noncardiac outcome. Survival ranged from 92% to 99% at 10 years, 85% to 99% at 20 years, and 88% to 94% at 30 years. MACE-free survival, mostly studied in those with CAA, varied from 66% to 91% at 10 years, 29% to 74% at 20 years, and 36% to 96% at 30 years. Seven of 10 studies reported an increased risk in early atherosclerosis. All 6 included studies demonstrated an increased risk in allergic diseases. LIMITATIONS Our study may have missed associated chronic comorbidities because short-term studies were excluded. The majority of outcomes were evaluated in East-Asian patients, which may limit generalizability. Studies frequently excluded patients without CAA and did not compare outcomes to a comparison group. CONCLUSIONS Studies demonstrate >90% survival up to 30 years follow-up. MACE is observed in children with CAA, but is not well studied in those without CAA.