2020European Heart Journal - Case ReportsOpen access

Antiepileptic rufinamide and QTc interval shortening in a patient with long QT syndrome: case report

Tapas Mondal, Kristen Sullivan, Syam Divakaramenon, Robert M. Hamilton

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Abstract

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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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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Available abstract

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.

Key concepts: QT interval, Long QT syndrome, Medicine, Short QT syndrome, Lennox–Gastaut syndrome, Epilepsy, Anesthesia, Internal medicine

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