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Published on: January 16, 2019
Autoptic Findings in Cases of Sudden Death Due to Kawasaki Disease
Giacomo Visi1, Federica Spina1, Fabio Del Duca2
1Department of Surgical Pathology, Medical, Molecular and Critical Area, Institute of Legal Medicine, University of Pisa, 56126 Pisa, Italy.
Insights
Kawasaki disease (KD) can lead to fatal cardiac complications, primarily acute myocardial infarction and coronary artery aneurysm rupture, especially in young individuals. Autopsies are crucial for understanding KD
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Pathology
Background:
- Kawasaki disease (KD) is a leading cause of acquired pediatric heart disease.
- While often self-limiting, KD can cause severe cardiovascular complications, including coronary artery aneurysms (CAA) and acute myocardial infarction (AMI).
- The etiology of KD remains unknown, hindering targeted prevention and treatment strategies.
Purpose of the Study:
- To review and synthesize autoptic and histopathological data from literature concerning deaths associated with Kawasaki disease.
- To identify the primary causes of mortality and affected demographics in fatal KD cases.
- To highlight the underrepresentation of autopsies in reported fatal KD cases.
Main Methods:
- Systematic literature review of scientific publications reporting autoptic and histopathological findings in fatal KD cases.
- Selection of 54 publications detailing 117 cases based on titles and abstracts.
- Analysis of reported causes of death and patient demographics.
Main Results:
- The majority of deaths were attributed to acute myocardial infarction (41.03%), coronary artery aneurysm rupture (11.97%), arrhythmia (8.55%), and acute coronary syndrome (8.55%).
- Fatalities predominantly affected individuals aged 20 years or younger (69.23%), consistent with coronary artery involvement.
- A small proportion of KD deaths undergo autoptic examination and are documented in the literature.
Conclusions:
- Sudden death in Kawasaki disease is often linked to cardiovascular complications, particularly those involving the coronary arteries.
- There is a critical need for increased autopsies in fatal KD cases to elucidate underlying molecular mechanisms.
- Further autopsies can inform the development of novel therapeutic protocols and improved prevention strategies for Kawasaki disease.
Abstract:
Kawasaki disease (KD) is the second-most-common childhood vasculitis, and its etiology is still unknown today. Even though the acute illness is usually self-limiting, sometimes, it can generate complications, such as coronary artery aneurysms (CAA), acute myocardial infarction (AMI), heart failure, or arrhythmias, and can rarely cause sudden or unexpected deaths. We present a review of the literature, which collects autoptic and histopathological data relating to many of the cases of these deaths. On the basis of the titles and abstracts, we selected 54 scientific publications for a total of 117 cases. Among them, as expected, the majority of the deaths were due to AMI (41.03%), arrhythmia (8.55%), acute coronary syndrome (8.55%), and CAA rupture (11.97%), involving mostly 20-year-olds or younger individuls (69.23%). This is not surprising since the CAs are the most involved arteries. Gross autoptic and histopathological findings are reported in the paper. Our work revealed that, when compared with the incidence of KD, only a few cases suffered from sudden death, underwent an autoptic examination, and were then described in the literature. We suggest that researchers should perform autopsies to gain a better understanding of the molecular pathways involved in KD so as to propose further innovative therapeutic protocols or implement more appropriate prevention schemes.
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