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Acute Dilated Cardiomyopathy in a Patient with Deficiency of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase

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Abstract

Deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase (LCHADD) is a rare inborn error of metabolism. It is associated with hypertrophic cardiomyopathy and less frequently with dilated cardiomyopathy. The incidence and pathophysiology of cardiac involvement in LCHADD is poorly understood. This report describes the acute decompensation of a 3-year-old girl who had LCHADD with rapidly developing dilated cardiomyopathy. A review of the literature and possible causes of cardiomyopathy in LCHADD are explored.

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Authors and Affiliations

  1. Department of Pediatrics, Nationwide Children’s Hospital Heart Center, 700 Children’s Drive, Room ED 622, Columbus, OH, 43205-2696, USA

    Peter C. Dyke II, Laura Konczal, Dennis Bartholomew, Kim L. McBride & Timothy M. Hoffman

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  1. Peter C. Dyke II
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  2. Laura Konczal
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  3. Dennis Bartholomew
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  4. Kim L. McBride
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  5. Timothy M. Hoffman
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Correspondence to Timothy M. Hoffman.

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Dyke, P.C., Konczal, L., Bartholomew, D. et al. Acute Dilated Cardiomyopathy in a Patient with Deficiency of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase. Pediatr Cardiol 30, 523–526 (2009). https://doi.org/10.1007/s00246-008-9351-8

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  • DOI: https://doi.org/10.1007/s00246-008-9351-8

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