Permanent junctional reciprocating tachycardia in children: a multicenter study on clinical profile and outcome

Guy Vaksmann ^1*, Christine D'Hoinne ², Vincent Lucet ³, Sophie Guillaumont ⁴, Jean-Marc Lupoglazoff ⁵, Alain Chantepie ⁶, Isabelle Denjoy ⁷, Elisabeth Villain ⁸ and François Marçon ⁹

¹ Clinique de la LOUVIERE, Pediatric Cardiology, France
² Pediatric cardiology, France
³ Le chateau des Cotes, France
⁴ Department of Pediatric Cardiology; Montpellier, France
⁵ Department of pediatric Cardiology; Hôpital Robert Debré, France
⁶ Department of Pediatric Cardiology; Tours, France
⁷ Department of Cardiology, Lariboisiere Hospital, France
⁸ Department of Pediatric Cardiology; Necker Hospital
⁹ Department of Pediatric Cardiology; Nancy, France

^* To whom correspondence should be addressed. E-mail: guy.vaksmann{at}wanadoo.fr.

Accepted 7 April 2005

Abstract

Objectives: The aim of this study was to investigate the clinical profile, natural history and optimal management of permanent reciprocating tachycardia (PJRT) in children.

Background: Persistent or permanent junctional reciprocating tachycardia (PJRT) is an uncommon arrhythmia characterized by an incessant orthodromic tachycardia with anterograde conduction over the atrioventricular node, and retrograde ventriculoatrial conduction via an accessory pathway with slow and decremental conduction. The arrhythmia is commonly incessant from birth or infancy and may lead to a tachycardia-induced cardiomyopathy that is reversible with rate control. Due to the rareness of the reports on natural history of PJRT, little is known about the clinical course and the optimal management of this chronic tachycardia.

Methods and results: Eighty-five patients meeting the ECG criteria for PJRT were included in a retrospective multicenter study. Age at diagnosis varied from birth to 20 years (median age: 3 months). Follow-up ranged from 0.1 to 26.0 (median 8.2) years. At the time of referral, 24 of 85 patients (28%) had congestive heart failure (CHF) that was resolved with medical treatment in all patients. Eighty three patients received drug treatment initially. Amiodarone and Verapamil were the most effective with a success rate of 84% to 94% alone or in association with digoxin. Radiofrequency ablation of the accessory pathway was performed in 18 patients. There was a trend for a relation between age at ablation and the result of the procedure, failures being more frequent in younger patients (p=.14). Two patients with persistent left ventricular dysfunction on echocardiography, but no symptoms of CHF, died suddenly 1 month and 3 years after diagnosis. Spontaneous resolution of PJRT occurred in 19 patients (22%), age at diagnosis of PJRT was not predictor of spontaneous resolution.

Conclusions: PJRT is a potentially lethal arrhythmia in children with tachycardia-induced cardiomyopathy. Spontaneous resolution of tachycardia is not uncommon. Antiarhythmic treatment is often effective. Radiofrequency ablation should be performed in older children or when rate control is not achieved, especially in patients with persistent left ventricular dysfunction.

Keywords: supraventricular tachycardia, children, outcome, permanent junctional reciprocating tachycardia

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