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The Long-term Cardiac and Noncardiac Prognosis of Kawasaki Disease: A Systematic Review
Jennifer J Y Lee1,2,3,4, Ethan Lin5, Jessica Widdifield2,6,4
1Department of Pediatrics.
Insights
Children with Kawasaki Disease (KD) have over 90% survival rates up to 30 years. Major adverse cardiovascular events (MACE) are noted in those with coronary artery aneurysms (CAA), but long-term risks for others require further study.
Area of Science:
- Pediatric Cardiology
- Immunology
- Public Health
Background:
- Kawasaki Disease (KD) is a critical childhood illness, but long-term risks for non-cardiac diseases and cardiac complications, especially in patients without coronary artery aneurysms (CAA), remain unclear.
- Understanding the long-term prognosis of KD is vital for patient management and public health strategies.
Purpose of the Study:
- To comprehensively evaluate the long-term mortality and prognosis of children following a diagnosis of Kawasaki Disease.
- To synthesize evidence on cardiac and non-cardiac outcomes in KD survivors.
Main Methods:
- A systematic review and meta-analysis of controlled trials and observational studies.
- Inclusion criteria required a minimum 1-year follow-up and reported mortality, major adverse cardiovascular events (MACE), or other chronic diseases.
- Data extraction focused on sample size, age at diagnosis, presence of CAA, follow-up duration, and outcomes.
Main Results:
- Seventy-four studies involving KD patients were analyzed, reporting on mortality, cardiac, and non-cardiac outcomes.
- Survival rates exceeded 90% up to 30 years post-diagnosis.
- Major adverse cardiovascular events (MACE)-free survival varied significantly, particularly in patients with coronary artery aneurysms (CAA), with notable risks of early atherosclerosis and increased incidence of allergic diseases.
Conclusions:
- Kawasaki Disease survivors demonstrate high long-term survival rates (>90% up to 30 years).
- Major adverse cardiovascular events (MACE) are a concern for KD patients with coronary artery aneurysms (CAA).
- The long-term cardiac prognosis for KD patients without CAA and the risk of non-cardiac comorbidities require further investigation due to study limitations.
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.
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