Junctional ectopic tachycardia in six paediatric patients

Abstract

The presenting features and treatment responses of six children with junctional ectopic tachycardia are evaluated. Two of the patients were siblings and both presented in early childhood with cardiopulmonary failure. The elder sibling died, the surviving sibling was controlled on a combination of amiodarone, digoxin, and sotalol. The remaining four patients presented in later childhood with tachycardia induced cardiomyopathy. Two of the patients were diagnosed incidentally and have normalised their myocardial function on sotalol therapy. The other two presented in congestive cardiac failure. Radiofrequency His bundle ablation and insertion of a permanent pacemaker to control the arrhythmia was undertaken in the elder of the two patients. The remaining patient has had marginal recovery of myocardial function on a combination of amiodarone and sotalol treatment. Improvement in myocardial function may take several months and is dependent on control of the tachycardia in some patients. Sotalol, when used as single or combination treatment, was partially successful in four cases in reducing heart rate. None of the patients reverted to sinus rhythm.

Pediatric Nonpost-Operative Junctional Ectopic Tachycardia

“…Many patients required Ͼ2 medications, either sequentially or in combination, to eventually see a salutary drug effect on JET rate. As in prior studies, our findings support the use of amiodarone for the treatment of JET (3,5). For patients for whom medications were thought to be effective for tachycardia conversion or rate control, amiodarone was the most frequently cited medication.…”

“…The mortality rate for JET has been previously reported by Villain et al (5) to be 34%, with 2 of 9 being sudden unexplained deaths in infants thought to have the JET rate controlled with medications. In contrast, Cilliers et al (3) showed no deaths among 9 patients with JET. In our population, we report a 4% mortality; all deaths occurred among patients presenting at age Յ6 months.…”

“…Junctional rates can be quite variable, and range from AJR to the more rapid JET. In review of prior literature pertaining to nonpost-operative JET, there appears to be no specific heart rate cut-off for the diagnosis of AJR versus JET (3)(4)(5)14,17). In creating a precise definition of JET for this manuscript, a survey of the authors was conducted with 3 proposed definitions for JET versus AJR based on prior studies in similar but different tachyarrhythmias: 1) JET defined by a junctional rate Ͼ95th percentile of heart rate for age; this definition was utilized in a study of the efficacy of amiodarone in a largely post-operative congenital heart disease population (24); 2) JET defined by a junctional rate of Ͼ170 beats/min; this definition was utilized in a study evaluating the treatment of JET in the post-operative congenital heart disease population (25); or 3) JET defined by Ͼ10% to 20% of the surrounding sinus heart rate; this type of definition was utilized to differentiate accelerated ventricular rhythm from ventricular tachycardia (26).…”

“…The mechanism of the tachycardia is thought to be abnormal automaticity arising from the region of the atrioventricular junction. Congenital JET presents in infancy and is associated with high morbidity and mortality (1)(2)(3)(4)(5)(6)(7). JET presenting beyond the first 6 months of life has a variable presentation.…”

Pharmacologic Management of Arrhythmias in Patients with Congenital Heart Disease

“…[78] However, there has been limited efficacy in restoring sinus rhythm. In extreme cases where pharmacologic therapy was unsuccessful in rate control, radio-frequency catheter ablation has been performed; however, this is often complicated by complete AV block and requires pacemaker implantation.…”