title: Permanent junctional re-entry tachycardia. A multicentre long-term follow-up study in infants, children and young adults.
abstract: OBJECTIVE
  Permanent junctional re-entry tachycardia is a relatively uncommon form of re-entry tachycardia with antegrade conduction occurring through the atrioventricular node and retrograde conduction over an accessory 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 pharmacological treatment and ablation procedures; evaluation was performed with respect to the patient's symptoms, tachycardia rate, frequency of the tachycardia and left ventricular function.
  
  
  RESULTS
  The long-term follow-up of 32 patients with permanent junctional re-entry tachycardia was evaluated. The first presentation with supraventricular tachycardia occurred between the 27th week of gestation and 27 years. The tachycardia rate ranged from 100 to 250 beats.min-1. During Holter-ECG, permanent junctional re-entry tachycardia was documented as present for over 50% of the time in 24 h in 22 patients (69%). Left ventricular performance was impaired in nine patients (28%) due to a tachycardia-related cardiomyopathy. Symptoms or signs of heart failure were mild to moderate in eight and severe in four patients; 20 patients 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 (mean = 15 x 3) years. Four patients needed no therapy because of the infrequency of permanent junctional re-entry tachycardia episodes. Twenty-five patients initially received antiarrhythmic drugs, which were effective or partially effective in 14 (56%). Eight of them are still on medical therapy; in five treatment was discontinued because of absence of symptoms. Eleven patients had ablation of the accessory pathway during follow-up, three underwent ablation as a primary procedure.
  
  
  CONCLUSIONS
  Permanent junctional re-entry tachycardia in our experience is an arrhythmia with a large variety of clinical symptoms. Patients with a slow tachycardia rate and infrequent episodes of tachycardia may never develop symptoms and therefore do not need any therapy. Patients with frequent permanent junctional re-entry tachycardia, a fast tachycardia rate 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.
authors.affiliation: Department of Pediatric Cardiology, University Children's Hospital, Homburg/Saar, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Lindinger
authors.forename: A
authors.initials: A
authors.lastname: Heisel
authors.forename: G
authors.initials: G
authors.lastname: von Bernuth
authors.forename: T
authors.initials: T
authors.lastname: Paul
authors.forename: H
authors.initials: H
authors.lastname: Ulmer
authors.forename: W
authors.initials: W
authors.lastname: Kienast
authors.forename: H
authors.initials: H
authors.lastname: Pitschner
authors.forename: K
authors.initials: K
authors.lastname: Kuck
authors.forename: W
authors.initials: W
authors.lastname: Hoffmann
mesh_terms: D000293:Adolescent; D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D001283:Atrioventricular Node / Q000503:physiopathology; D017115:Catheter Ablation; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003452:Cryosurgery; D015716:Electrocardiography, Ambulatory; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012086:Reoperation; D012189:Retrospective Studies; D013611:Tachycardia, Atrioventricular Nodal Reentry / Q000503:physiopathology / Q000628:therapy*; D013613:Tachycardia, Ectopic Junctional / Q000503:physiopathology / Q000628:therapy*; D016896:Treatment Outcome; D016277:Ventricular Function, Left / Q000502:physiology
chemical_list: D000889:Anti-Arrhythmia Agents