Premium
Left ventricular non‐compaction revealed by aortic regurgitation due to Kawasaki disease in a boy with LDB3 mutation
Author(s) -
Hachiya Akira,
Motoki Noriko,
Akazawa Yohei,
Matsuzaki Satoshi,
Hirono Keiichi,
Hata Yukiko,
Nishida Naoki,
Ichida Fukiko,
Koike Kenichi
Publication year - 2016
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12983
Subject(s) - medicine , cardiology , kawasaki disease , heart failure , vasculitis , regurgitation (circulation) , etiology , artery , disease
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.