Table 1.

Medical and procedural treatment options in LQTS

Medical treatment	Notes
β-Adrenergic blockers	Documented to reduce risk of cardiac events
Labetolol	Described in limited clinical situations. Has both α- and β-adrenergic blockage actions, less specific antisympathetic effects
Verapamil	Hypothesized to decrease calcium channel– mediated heterogeneity of repolarization
Nicorandil	Potassium channel opener decreases heterogeneity of repolarization and shortens action potential duration in LQT1 and LQT2 (and other K+-channel mutation mediated LQTS)
Mexiletine	Sodium channel blockade thought to decrease gain-of function mutation in LQT3
Lidocaine	Blocks inactivated sodium channels, thought to decrease sodium leak current in LQT3

Procedural treatment	Notes
ICD placement	Recommended for patients with aborted cardiac arrest or VT on medical treatment, other indications (see text)
Pacemaker placement	Recommended for rate smoothing in pause-related VT
Left cardiac sympathetic denervation	Decreases frequency of cardiac events. May decrease frequency of ICD defibrillation
Electrophysiologic study/catheter ablation	Case reports of successful ablation of arrhythmogenic foci. Not in common diagnostic or therapeutic use.

VT = Ventricular tachycardia, ICD = Implantable cardioverter-defibrillator