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KMID : 0191120140290060879
Journal of Korean Medical Science
2014 Volume.29 No. 6 p.879 ~ p.883
Transient Complete Atrioventricular Block in a Preterm Neonate with Congenital Myotonic Dystrophy: Case Report
Kim Hee-Na

Cho Young-Kuk
Cho Joo-Hyun
Yang Eun-Mi
Song Eun-Song
Choi Young-Youn
Abstract
Congenital myotonic dystrophy (CMD) is an inherited neuromuscular disorder with cardiac rhythm abnormalities that may occur as a child grows. No report has described complete atrioventricular (AV) block detected in a neonate with CMD. We report a floppy infant of 31+4 weeks gestation with complete AV block at birth, who was diagnosed with CMD by Southern analysis. She recovered from complete AV block 32 hr after temporary transcutaneous pacing was applied. To the best our knowledge, this is the first recorded case of a complete AV block accompanied by CMD during the neonatal period. When a newborn has a complete AV block, the physician should consider the possibility of the CMD and conduct a careful physical examination.
KEYWORD
Complete Atrioventricular Block, Congenital Myotonic Dystrophy, Pacing
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