Background: There is limited pharmacologic therapy to reduce the QT interval in hereditary long QT syndrome (LQTS).
Case Summary: We describe a child with Allan-Herndon-Dudley syndrome, Lennox-Gastaut epileptic syndrome (LGS), and LQTS Type 1 (LQTS1). Rufinamide was added to his antiepileptic medications to improve seizure control and was noted to be associated with a marked improvement in electrocardiogram QT interval. To the best of our knowledge, this is the first reported case of successful pharmacologic shortening of the QT interval in LQTS1.
Discussion: This case report highlights the potential benefits of rufinamide, a drug associated with mild QT shortening in normal individuals, to markedly reduce and normalize QT duration in a subject with LQTS1.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7793134 | PMC |
http://dx.doi.org/10.1093/ehjcr/ytaa336 | DOI Listing |
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