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, 2003). LQTS has genetic causes in at least 75% of individuals diagnosed with LQTS, including the possibility of a mutation in one of 12 associated genes (Ackerman et al., 2011). Patients with an inherited form of LQTS often have a history of syncope (loss of consciousness) in the absence of other causes, such as QT-prolonging medications, cardiomyopathies, myocardial ischemia, or electrolyte imbalances. The circumstances under which syncope occurs may further suggest a specific subtype of LQTS. Increased risk of cardiac ventricular arrhythmic events is triggered by exercise in patients with LQT1 and auditory Isotretinoin stimulation such as a loud sudden noise in patients with LQT2. Patients with LQT3 usually have arrhythmias during rest or sleep when the heart rate is slow. Patients with a history of aborted cardiac arrest or life-threatening ventricular arrhythmias are considered high risk (> 10% risk) for a subsequent life-threatening cardiac event (Priori et al., 2003). Further workup and possible genetic testing is warranted in patients with a history of syncope within the past 2 years, especially if this website recurrent, or a prolongation of corrected QT (QTc) is noted on the ECG (Ackerman et al., 2011). Affected individuals without a history of syncope and with QTc duration �� 0.50 s can be assigned a low risk (Kinase Inhibitor Library earlier age than women (Priori et al., 2003). This is believed to be due to cardioprotective effects of estrogen in women. Depending on the subtype of LQTS, medical therapy may include beta-blockers, an implantable cardioverter defibrillator (ICD), or lifestyle modification to avoid triggers such as certain forms of exercise (i.e., swimming [LQT1]), sudden auditory triggers (LQT2), and avoidance of QT-prolonging drugs, in all subtypes. Knowledge of the LQTS genotype may be used to tailor the management plan. For example, patients with LQT3 benefit less from beta-blockers and more from the placement of an ICD to prevent SCD (Lehnart et al., 2007). Recently, the Heart Rhythm Society and the European Heart Rhythm Association developed consensus recommendations on genetic testing for channelopathies and cardiomyopathies (Ackerman et al., 2011). The expert panel recommended genetic counseling for all patients and relatives with channelopathies and cardiomyopathies. Genetic counseling should include discussion of the risks, benefits, and options available for clinical or genetic testing. Treatment decisions, however, should not rely solely on an individual's genetic test result but rather comprehensive clinical evaluation and family history.