Abstract
We report a case of recurrent episodes of Torsades de Pointes arrhythmia in the setting of transiently impaired left ventricular ejection fraction, acute respiratory distress syndrome, transient hypokalaemia and QT-prolonging drugs, in a previously healthy 25-yr-old female patient. In the course of the clinical and genetic work-up this patient was newly diagnosed with a mutation in KCNH2 encoding the alpha-subunit of the human repolarizing potassium channel I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Anti-Bacterial Agents / adverse effects
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Antifungal Agents / adverse effects
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ERG1 Potassium Channel
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Echocardiography / methods
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Erythromycin / adverse effects
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Ether-A-Go-Go Potassium Channels / genetics
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Female
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Fluconazole / adverse effects
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Humans
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Hypokalemia / complications
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Hypokalemia / physiopathology
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Long QT Syndrome / complications
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Long QT Syndrome / congenital*
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Long QT Syndrome / physiopathology
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Mutation / genetics
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Recurrence
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Respiratory Distress Syndrome / etiology*
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Respiratory Distress Syndrome / physiopathology
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Torsades de Pointes / complications*
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Torsades de Pointes / physiopathology
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Treatment Outcome
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Ventricular Dysfunction, Left / complications*
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Ventricular Dysfunction, Left / physiopathology
Substances
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Anti-Bacterial Agents
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Antifungal Agents
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ERG1 Potassium Channel
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Ether-A-Go-Go Potassium Channels
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KCNH2 protein, human
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Erythromycin
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Fluconazole