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Biopsy-proven myocardial sequels in Kawasaki disease with giant coronary aneurysms

Published online by Cambridge University Press:  28 June 2010

Susumu Yonesaka ,
Toru Takahashi ,
Shuji Eto ,
Takumi Sato ,
Katuki Otani ,
Tomomi Ueda ,
Akira Sato ,
Yosuke Kitagawa ,
Yuki Konno  and
Manabu Kinjo
Susumu Yonesaka*
Affiliation:
Department of Nursing, School of Health Sciences, Hirosaki University, 66-1 Honcho, Hirosaki, Japan
Toru Takahashi
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Shuji Eto
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Takumi Sato
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Katuki Otani
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Tomomi Ueda
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Akira Sato
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Yosuke Kitagawa
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Yuki Konno
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
Manabu Kinjo
Affiliation:
Department of Pediatrics, School of Medicine, Hirosaki University, 5-Zaifucho, Hirosaki, Japan
*
Correspondence to: S. Yonesaka, Department of Nursing, School of Health Sciences, Hirosaki University, 66-1 Honcho, Hirosaki, 036-8564 Japan. Tel: +81 172 395947; Fax: +81 172 395947; E-mail: yonesaka@cc.hirosaki-u.ac.jp
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Abstract

Background

Although most Kawasaki disease with giant coronary aneurysms is asymptomatic, conventional investigations might not identify previous lesions, or all Kawasaki disease with giant aneurysms at risk of future myocardial lesions. We evaluated the long-term histopathology of the myocardium, especially of intramural small vessels in asymptomatic Kawasaki disease with giant aneurysms.

Method

The initial study comprised 16 consecutive Kawasaki patients – male-to-female ratio was 12:4 – aged from 2 to 12 years, and in the subsequent study, the same patients were aged from 4.9 to 16 years. Endomyocardial biopsies were histopathologically evaluated. Microangiopathies, mitochondrial abnormalities, and loss or disarray of myofibrils were compared by electron microscopy.

Results

The incidence of histopathological abnormalities such as degeneration, hypertrophy, and inflammatory cell infiltration was quite high in the initial study, and inflammatory cell infiltration, interstitial fibrosis, and disarray were very noticeable at follow-up biopsies. The area of fibrous tissue was significantly higher in patients administered with intravenous immunoglobulin at follow-up biopsies. Electron microscopy showed microangiopathies including microthrombi within intramural small vessels in some patients at follow-up biopsies. The sites of the coronary aneurysms did not seem to have an impact on the biopsy findings, suggesting that the underlying pathophysiology is related to the original disease process.

Conclusions

Whether the abnormalities were due to direct myocardial injury, chronic ischaemia, repeated small-vessel thrombosis, or other problems associated only with biopsies, is difficult to determine. However, this subgroup had residual abnormal lesions in the myocardium. Follow-up should be more aggressive in this group of patients to identify myocardial damage that could be asymptomatic.

Keywords

Acquired cardiac diseasecoronary arterial lesionendomyocardial biopsycardiomyopathy
Type
Original Articles
Information
Cardiology in the Young , Volume 20 , Issue 6 , December 2010 , pp. 602 - 609
Copyright
Copyright © Cambridge University Press 2010

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