Congenital QT interval prolongation. A review with a survey of three families

The prolonged QT interval syndrome without hearing loss (Romano-Ward syndrome) is described in three families with 48 affected members. Syncope or dizziness caused by different ventricular tachyarrhythmias were the presenting symptoms in the symptomatic patients. Four of the subjects died suddenly. Right stellate ganglionectomy was performed in one patient in order to abolish the ventricular dysrhythmia. beta-blockers are considered the drug of choice in patients with hereditary prolonged QT interval; if the beta-blockers fail to abolish the syncopal attacks of severe bradycardia complicates the clinical course, a pharmacological blockade of the stellate ganglia should be performed and its results carefully evaluated in order to establish whether a stellate ganglionectomy is justified.