The long QT syndrome; effects of drugs and left stellate ganglion block.

Four patients, two with congenital QT prolongation (Romano-Ward syndrome) and two with acquired idiopathic QT prolongation not related to bradycardia, drug toxicity, electrolyte imbalance, or neurological disorder were investigated for the onset of recurrent palpitations and/or syncope. The effects on the measured QT interval of intravenously administered propranolol (QTp), an infusion of isoproterenol (QTi) and left stellate ganglion block (QTs) were assessed at identical atrial paced rates and during sinus rhythm, corrected for rate change (QTc). Propranolol shortened the QTc in all patients. The QTp shortened only in those with congenital QT prolongation. Isoproterenol lengthened the QTc in the three patients studied. However, the QTi lengthened in the congenital syndrome whereas it shortened in the acquired syndrome. The QTs was uninfluenced by left stellate ganglion block in all patients. It is suggested that the congenital and acquired forms can be differentiated by pharmacological interventions and that the efficacy of propranolol in the former may result from its ability not only to increase the threshold for ventricular fibrillation, but also its ability to shorten the QT interval.