PERMANENT JUNCTIONAL REENTRY TACHYCARDIA - A MULTICENTER LONG-TERM FOLLOW-UP-STUDY IN INFANTS, CHILDREN AND YOUNG-ADULTS

Aims Permanent junctional re-entry tachycardia is a relatively uncommo n form of re-entry tachycardia with antegrade conduction occurring thr ough the atrioventricular node and retrograde conduction over an acces sory pathway usually located in the postero-septal region. It was the aim of the study to investigate the course of permanent junctional re- entry tachycardia with particular regard to the effectiveness of pharm acological treatment and ablation procedures; evaluation was performed with respect to the patient's symptoms, tachycardia rate, frequency o f the tachycardia and left ventricular function. Methods and Results T he long-term follow-up of 32 patients with permanent junctional re-ent ry tachycardia was evaluated. The first presentation with supraventric ular tachycardia occurred between the 27th week of gestation and 27 ye ars. The tachycardia rate ranged from 100 to 250 beats. min(-1). Durin g Holter-ECG, permanent junctional re-entry tachycardia was documented as present for over 50% of the time in 24 h in 22 patients (69%). Lef t ventricular performance was impaired in nine patients (28%) due to a tachycardia-related cardiomyopathy. Symptoms or signs of heart failur e were mild to moderate in eight and severe in four patients; 20 patie nts showed no clinical impairment. Follow-up time was 1 to 31 (mean 10 ) years; current age of the patients ranged from 1.5 months to 35 (mea n=15.3) years. Four patients needed no therapy because of the infreque ncy of permanent junctional re-entry tachycardia episodes. Twenty-five patients initially received antiarrhythmic drugs, which were effectiv e or partially effective in 14 (56%). Eight of them are still on medic al therapy; in five treatment was discontinued because of absence of s ymptoms. Eleven patients had ablation of the accessory pathway during follow-up, three underwent ablation as a primary procedure. Conclusion Permanent junctional re-entry tachycardia in our experience is an arr hythmia with a large variety of clinical symptoms. Patients with a slo w tachycardia rate and infrequent episodes of tachycardia may never de velop symptoms and therefore do not need any therapy. Patients with fr equent permanent junctional re-entry tachycardia, a fast tachycardia r ate and impaired left ventricular function need effective therapy. In infancy and early childhood medical therapy is recommended as a first option, whereas in older and symptomatic patients catheter ablation is an effective and safe procedure.