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

• Published in: Pacing and clinical electrophysiology 2012-03, Vol.35 (3), p.e62-e64
• Main Authors: SHAH, DIPAK P., BAEZ‐ESCUDERO, JOSE L., WEISBERG, IAN L., BESHAI, JOHN F., BURKE, MARTIN C.
• Format: Article
• Language: English
• 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
• ISSN: 0147-8389; 1540-8159
• DOI: 10.1111/j.1540-8159.2010.02913.x

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)