Ranolazine Safely Decreases Ventricular and Atrial Fibrillation in Timothy Syndrome (LQT8)

Long QT eight (LQT8), otherwise known as Timothy syndrome (TS), is a genetic disorder causing hyper‐activation of the L‐type calcium channel Cav 1.2. This calcium load and the resultant increase in the QT interval provide the substrate for ventricular arrhythmias. We previously presented a case in a...

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Veröffentlicht in:Pacing and clinical electrophysiology 2012-03, Vol.35 (3), p.e62-e64
Hauptverfasser: SHAH, DIPAK P., BAEZ‐ESCUDERO, JOSE L., WEISBERG, IAN L., BESHAI, JOHN F., BURKE, MARTIN C.
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container_issue 3
container_start_page e62
container_title Pacing and clinical electrophysiology
container_volume 35
creator SHAH, DIPAK P.
BAEZ‐ESCUDERO, JOSE L.
WEISBERG, IAN L.
BESHAI, JOHN F.
BURKE, MARTIN C.
description Long QT eight (LQT8), otherwise known as Timothy syndrome (TS), is a genetic disorder causing hyper‐activation of the L‐type calcium channel Cav 1.2. This calcium load and the resultant increase in the QT interval provide the substrate for ventricular arrhythmias. We previously presented a case in a patient with TS who had a profound decrease in his burden of ventricular arrhythmias after institution of an L‐type calcium channel blocker. Although this patient's arrhythmia burden had decreased, he displayed an increasing burden of atrial fibrillation and still had bouts of ventricular fibrillation requiring defibrillator therapy. Basic research has recently shown that ranolazine, a multipotent ion‐channel blocker, may be of benefit in patients with LQT8 syndrome. This case report details the decrease of atrial fibrillation and ventricular fibrillation events in our LQT8 patient with the addition of ranolazine. (PACE 2010; 1–3)
doi_str_mv 10.1111/j.1540-8159.2010.02913.x
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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Acetanilides - therapeutic use
Adult
Anti-Arrhythmia Agents - therapeutic use
Atrial Fibrillation - drug therapy
Autistic Disorder
Drug Therapy, Combination
electrophysiology—clinical
Humans
long QT
Long QT Syndrome - drug therapy
Male
pharmacology
Piperazines - therapeutic use
Ranolazine
Syndactyly - drug therapy
Treatment Outcome
Ventricular Fibrillation - drug therapy
Verapamil - therapeutic use
title Ranolazine Safely Decreases Ventricular and Atrial Fibrillation in Timothy Syndrome (LQT8)
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