Abstract
Patients with congenital QT interval prolongation are at risk of ventricular arrhythmias and sudden death. It has been suggested that the susceptibility to arrhythmias in these syndromes may be related to the abnormal dynamics of ventricular repolarisation. The dynamics of ventricular repolarisation, including assessment of the effect of changing heart rate on the QT interval and the duration of the right ventricular monophasic action potential, were studied in eight patients with congenital long QT syndromes. The effects of altered sympathetic tone on these dynamics were investigated with isoprenaline, propranolol, and left stellate ganglion block. The rate adaptation of the QT interval was abnormal in only a few patients and in some patients this feature may be related to the severity of the condition. These abnormalities may be exaggerated by isoprenaline and lessened by propranolol and left stellate ganglion block. Monophasic action potential dynamics were normal in all patients. The hypothesis that impaired QT rate adaptation may play a role in the genesis of ventricular arrhythmias in these syndromes is not, in general, supported by the present data. However, in patients with impaired adaptation the normalisation of QT dynamics after beta blockade and left stellate ganglion block was consistent with the efficacy of these forms of treatment.