Left ventricular non‐compaction revealed by aortic regurgitation due to Kawasaki disease in a boy with LDB3 mutation

Kawasaki disease (KD) is an acute febrile illness of childhood characterized by systemic vasculitis, especially coronary arteritis. Aortic valve regurgitation (AVR) is a relatively common complication. There have been no reports to date of heart failure and left ventricular non‐compaction (LVNC) aft...

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Veröffentlicht in:Pediatrics international 2016-08, Vol.58 (8), p.797-800
Hauptverfasser: Hachiya, Akira, Motoki, Noriko, Akazawa, Yohei, Matsuzaki, Satoshi, Hirono, Keiichi, Hata, Yukiko, Nishida, Naoki, Ichida, Fukiko, Koike, Kenichi
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Sprache:eng
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Zusammenfassung:Kawasaki disease (KD) is an acute febrile illness of childhood characterized by systemic vasculitis, especially coronary arteritis. Aortic valve regurgitation (AVR) is a relatively common complication. There have been no reports to date of heart failure and left ventricular non‐compaction (LVNC) after acute KD, although the precise etiology of this condition remains unclear. A 6‐month‐old boy with KD was admitted to hospital. Despite high‐dose i.v. gammaglobulin for dilation of the coronary artery, moderate AVR appeared, and thereafter he developed heart failure. A rough, dense LV myocardium indicated LVNC. On genetic testing a heterogenous 163G > A substitution changing a valine to isoleucine in LIM domain binding protein 3 (LDB3) was identified. Additional cardiac stress, such as that caused by AVR and/or KD might have triggered cardiac failure in the form of LVNC due to LDB3 mutation.
ISSN:1328-8067
1442-200X
DOI:10.1111/ped.12983