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


METHODS
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.
Yonesaka
Susumu
S
Department of Nursing, Hirosaki University, Japan. yonesaka@cc.hirosaki-u.ac.jp
Takahashi
Toru
T
Eto
Shuji
S
Sato
Takumi
T
Otani
Katuki
K
Ueda
Tomomi
T
Sato
Akira
A
Kitagawa
Yosuke
Y
Konno
Yuki
Y
Kinjo
Manabu
M
D000293:Adolescent; D001707:Biopsy, Needle; D009202:Cardiomyopathies / Q000150:complications / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003323:Coronary Aneurysm / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D003324:Coronary Artery Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D004699:Endocardium / Q000473:pathology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D009206:Myocardium / Q000473:pathology*; D011859:Radiography
D016756:Immunoglobulins, Intravenous