• Jana-Katharina Dieks, Sophia Klehs, Matthias J Müller, Thomas Paul, and Ulrich Krause.
• Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University, Göttingen, Germany. Electronic address: jana.dieks@med.uni-goettingen.de.
• Heart Rhythm. 2016 Jun 1; 13 (6): 1297-302.

BackgroundTreatment of congenital junctional ectopic tachycardia (JET) is often challenging. In the majority of patients affected, a combination of ≥2 antiarrhythmic drugs is required for JET control.ObjectiveThe purpose of this study was to assess the efficacy and safety of adjunctive ivabradine therapy for pediatric congenital JET.MethodsSince January 2015, 5 consecutive patients aged 10 days to 3.5 years (median 8 weeks) were treated with adjunctive ivabradine for congenital JET. All patients had previously undergone antiarrhythmic therapy with unsatisfactory control of JET. Ivabradine was administered orally at an initial dosage of 0.05-0.1 mg/kg/d divided into 2 single doses and was increased up to 0.28 mg/kg/d if necessary.ResultsIn all 5 patients, ivabradine proved to be successful in controlling JET. Complete suppression of JET and conversion into sinus rhythm were achieved in 4 of 5 patients. The remaining patient had effective heart rate control with persistent slow JET. Mean heart rate was reduced by 31% compared to pre-ivabradine (P = .03) as assessed by 24-hour Holter monitoring. Echocardiography revealed improvement of left ventricular function in all 3 patients with previously impaired left ventricular function. No significant side effects of ivabradine were encountered during median follow-up of 135 days (range 37-203 days).ConclusionIn our group of patients with congenital JET, adjunctive treatment with ivabradine resulted in effective and safe rhythm/heart rate control and therefore may be recommended early in the course of this rare inborn tachyarrhythmia.Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

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