THE LONG QT SYNDROME - AN ELECTIVE INDICA TION FOR BETA-BLOCKER THERAPY - RESULTS IN 5 CASES

The authors report 5 cases of the long QT syndrome. The patients had m alaises and syncopes triggered by exercise or emotional stress, occurr ing before 10 years of age. The diagnosis was delayed by an average of 38 months (range : 2 to 72 months) from the first symptom and systema tic familial study which was positive in 4 of the 5 cases. The correct ed QT intervals varied from 0.46 to 0.56 second. All patients were pre scribed the betablocker nadolol, resulting in total regression of symp toms with no deaths during an average follow-up period of 40 months (r ange : 26 to 64 months). The long QT syndrome is a rare hereditary dis order comprising several entities. Recent genetic advances have improv ed our understanding of the condition. All have an idiopathic prolonga tion of the QT interval (> 0.44 second) in common with a predispositio n to syncope due to torsades de pointes which may cause sudden death. The spontaneous arrhythmic mortality, which was early and high, has be en remarkably decreased by betablocker therapy, from 73 % to 6 % at 10 years. The authors find nadolol to be extremely reliable and well tol erated, and recommended it as the drug of choice in this indication.