Permanent junctional reciprocating tachycardia in children: A multicenter experience

doi:10.1016/j.hrthm.2014.04.033

Heart Rhythm

Volume 11, Issue 8, August 2014, Pages 1426-1432

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Background

Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published.

Objective

To describe the presentation and clinical course of PJRT in children.

Methods

This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010.

Results

The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients.

Conclusion

PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.

Introduction

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of supraventricular tachycardia that occurs predominantly in infants and children.¹ The arrhythmia substrate is an accessory pathway with slow, decremental retrograde conduction² that is commonly located in the posteroseptal region of the atrioventricular (AV) junction.³ In the majority of affected patients, PJRT is incessant and may lead to tachycardia-induced cardiomyopathy (TIC), which is reversible with a sustained period of rate or rhythm control.

There are several studies on the course and treatment of PJRT in children,4, 5, 6, 7, 8, 9 but they do not reflect management and outcomes in the current era. In previous case series, PJRT was controlled with antiarrhythmic medications in up to 25% of the pediatric patients, and spontaneous resolution was uncommon.5, 8, 9 Disappointing outcomes from medical management have resulted in the use of catheter ablation as primary therapy and for PJRT that is refractory to antiarrhythmic medications.4, 5, 6, 7, 8 Pediatric catheter ablation has evolved rapidly over the past decade, and modern technologies such as cryoablation and electroanatomic mapping have improved outcomes and decreased the risk of adverse events, the latter primarily reducing the need for fluoroscopy use during the procedure.¹⁰

The objective of this study was to describe the clinical course and outcomes of treatment of PJRT in children in the modern era to reflect these therapeutic advances in catheter ablation technology.

Section snippets

Methods

This is a retrospective cohort study of patients with PJRT from 11 pediatric centers. Participating centers were solicited through the Pediatric and Congenital Electrophysiology Society. Approval from the local ethics committee was obtained at each center.

Clinical presentation and PJRT at diagnosis

There were 194 patients (98 male patients) included in the study. The median age at diagnosis was 3.2 months (IQR 0.1–116.1 months). PJRT cases were observed in a unimodal distribution peaking in infancy (Figure 1). There were 110 patients (57%) diagnosed with PJRT in infancy (aged <1 year), including 53 patients (27%) presenting with fetal tachycardia. PJRT presented with symptoms in 134 of 194 patients (69%), while in the remaining 31% of the patients it was an incidental finding on physical

Discussion

This study, the largest to date, describes the clinical course and outcomes of PJRT detailing treatment strategies in the current era. Similar to prior studies,5, 8 these data show that the incidence is highest in infancy, that there is a low likelihood of spontaneous resolution, and that the risk of TIC is significant. One of the important findings of this study is that despite the 90% success rate of catheter ablation and low risk of serious complications, it is the initial treatment for only

Conclusion

This international multicenter review of PJRT provides a comprehensive assessment of medical treatment options, suggesting that PJRT may be amenable to medical control, but is unlikely to resolve spontaneously. The success of catheter ablation as a treatment of PJRT exceeds that provided by medication. Compared with previous studies of catheter ablation for this arrhythmia, the present study shows that the risk of recurrence or major complications is lower. Catheter ablation for PJRT should be

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Permanent junctional reciprocating tachycardia (PJRT) is a rare form of congenital arrhythmia occurring predominantly in infants and children. Prenatal presentation is frequently characterized by incessant tachycardia leading to dilated cardiomyopathy (DCM). Some patients can have a normal heart rate which leads to a delayed diagnosis. We report a case of a neonate who was presented prenatally with DCM, fetal hydrops, and no signs of fetal arrhythmia. Diagnosis of PJRT was established after delivery with characteristic electrocardiographic patterns. Successful conversion to sinus rhythm with digoxin and amiodarone was achieved three months later. At 16 months of age, both echocardiography and electrocardiography were normal.
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: This work was funded by a grant from the Rare Disease Foundation, Vancouver, Canada. Dr Kubus was supported by MH CZ – DRO, University Hospital Motol, Prague, Czech Republic.

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Permanent junctional reciprocating tachycardia in children: A multicenter experience

Background

Objective

Methods

Results

Conclusion

Introduction

Section snippets

Methods

Clinical presentation and PJRT at diagnosis

Discussion

Conclusion

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Biomed Inform

Am J Cardiol

Rev Esp Cardiol