Variability of action potential duration in pharmacologically induced long QT syndrome type 1.

Long QT Syndrome (LQTS) is a congenital disorder associated with life-threatening arrhythmias. LQT1, a type of LQTS affecting the slow delayed rectifier potassium current, shows a higher incidence of arrhythmia associated with sympathetic stimulation than other types of LQTS. LQT1 patients show increased variability of repolarization with epinephrine infusion, as measured from the 12-lead ECG. We investigate the variability of repolarization measured as action potential duration (APD) in the rabbit left ventricle: how APD variability is affected by pacing rate, transmural location, LQT1 induced by chromanol 293b, and epinephrine infusion. Chromanol preferentially changes APD variability in the midwall. Infusing epinephrine returns the variability to near-control levels. These results differ substantially from clinical studies and show the need for further study.