[Prevention of polymorphous ventricular tachycardia in patients with congenital long QT interval syndrome]

Patients with the Romano-Ward long QT interval syndrome run a high risk of sudden cardiac death. Beta-blockers of the sympathetic nerve are effective treatment. Some patients die suddenly despite this treatment. The treatment of choice is a combination of beta-blockers of the sympathetic nerve and cardiostimulation. The authors describe a group of their own five patients from three families with the Romano-Ward syndrome. The course was favourable. The stimulation rate needed for normalization of the QT interval and a favourable clinical development was 78 +/- 6 imp./min. Based on data in the literature and their own experience the authors recommended combined treatment with beta-blockers and cardiostimulation in patients with the Romano-Ward syndrome, if monotherapy with beta-blockers is not effective.