SHORT-COUPLED VARIANT OF TORSADE-DE-POINTES - A NEW ELECTROCARDIOGRAPHIC ENTITY IN THE SPECTRUM OF IDIOPATHIC VENTRICULAR TACHYARRHYTHMIAS

Background Torsade de pointes is characterized not only by its particu lar ECG pattern but by its context of congenital or acquired long QT s yndrome and the long coupling interval of the initial premature beat. Methods and Results We observed 14 patients aged 34.6+/-10 years (mean +/-SD) with no structural heart disease who presented with syncope rel ated to a typical ECG aspect of torsade de pointes. However, there was no evidence of long QT syndrome, and the torsade had the unusual part icularity of an extremely short coupling interval of the first beat or of the isolated premature beats (245+/-28 milliseconds). In 10 cases they deteriorated into ventricular fibrillation. Four patients had a f amilial history of sudden death. Only 2 patients had a tachyarrhythmia inducible by programmed stimulation. At Holter recordings the heart r ate variability was globally and significantly depressed, the vagal li mb of the autonomic nervous system being predominantly affected. Durin g a mean follow-up of 7 years there were 5 deaths (4 sudden). Nine pat ients are alive, 3 with implanted defibrillators and 6 treated with ve rapamil alone. Unlike the other types of antiarrhythmic agents includi ng beta-blockers and amiodarone, verapamil is in our experience the on ly drug apparently active on the arrhythmias; however, it does not pre vent sudden death. Conclusions The short-coupled variant of torsade de pointes should be identified because of their ECG pattern and the ris k of sudden death in young adults with no structural heart disease.