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American Academy of Pediatrics
Case Report

Prolonged QTc Interval in Association With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

Jason R. Wiles, Nancy Leslie, Timothy K. Knilans and Henry Akinbi
Pediatrics June 2014, 133 (6) e1781-e1786; DOI: https://doi.org/10.1542/peds.2013-1105
Jason R. Wiles
aPerinatal Institute, Division of Neonatology;
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Nancy Leslie
bDivision of Human Genetics;
cDepartment of Pediatrics; and
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Timothy K. Knilans
cDepartment of Pediatrics; and
dThe Heart Institute, University of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
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Henry Akinbi
aPerinatal Institute, Division of Neonatology;
cDepartment of Pediatrics; and
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Abstract

Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency is the most common disorder of mitochondrial fatty acid oxidation. We report a term male infant who presented at 3 days of age with hypoglycemia, compensated metabolic acidosis, hypocalcemia, and prolonged QTc interval. Pregnancy was complicated by maternal premature atrial contractions and premature ventricular contractions. Prolongation of the QTc interval resolved after correction of metabolic derangements. The newborn screen was suggestive for MCAD deficiency, a diagnosis that was confirmed on genetic analysis that showed homozygosity for the disease-associated missense A985G mutation in the ACADM gene. This is the first report of acquired prolonged QTc in a neonate with MCAD deficiency, and it suggests that MCAD deficiency should be considered in the differential diagnoses of acute neonatal illnesses associated with electrocardiographic abnormality. We review the clinical presentation and diagnosis of MCAD deficiency in neonates.

  • medium-chain acyl-coenzyme A dehydrogenase deficiency
  • MCAD
  • neonate
  • prolonged QT
  • Accepted November 27, 2013.
  • Copyright © 2014 by the American Academy of Pediatrics

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Pediatrics
Vol. 133, Issue 6
1 Jun 2014
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Prolonged QTc Interval in Association With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
Jason R. Wiles, Nancy Leslie, Timothy K. Knilans, Henry Akinbi
Pediatrics Jun 2014, 133 (6) e1781-e1786; DOI: 10.1542/peds.2013-1105

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Prolonged QTc Interval in Association With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
Jason R. Wiles, Nancy Leslie, Timothy K. Knilans, Henry Akinbi
Pediatrics Jun 2014, 133 (6) e1781-e1786; DOI: 10.1542/peds.2013-1105
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Subjects

  • Fetus/Newborn Infant
    • Neonatology

Keywords

  • medium-chain acyl-coenzyme A dehydrogenase deficiency
  • MCAD
  • neonate
  • prolonged QT
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