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 |
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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. |
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ISSN: | 1328-8067 1442-200X |
DOI: | 10.1111/ped.12983 |