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Congenital junctional ectopic tachycardia in children and adolescents: a 20 year experience based study
  1. B Sarubbi1,
  2. B Musto2,
  3. V Ducceschi1,
  4. A D‘Onofrio2,
  5. C Cavallaro2,
  6. F Vecchione2,
  7. C Musto2,
  8. R Calabrò1
  1. 1Second University of Naples, Division of Paediatric Cardiology, Monaldi Hospital, Naples, Italy
  2. 2Division of Cardiology, Monaldi Hospital
  1. Correspondence to:
    Berardo Sarubbi, MD, PhD, Via Tino di Camaino, 23, 80128 Napoli, Italy;

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Junctional ectopic tachycardia (JET) is a rare arrhythmia presenting either as a primary form1 or more often as a transient phenomenon immediately after heart surgery.2 Congenital JET usually occurs in the first six months of life as a persistent arrhythmia, associated in up to 60% of cases with cardiomegaly and/or heart failure.1

Congenital JET has been described in sporadic case reports or larger multicentre populations. The purpose of this study is to examine retrospectively the clinical presentation and outcome of a cohort of nine patients with congenital JET referred during a 20 year period time to a single unit and all medically treated.


Nine patients (two male, seven female) suffering from persistent sustained JET were referred to our hospital between 1980 and 1999. All the patients underwent a complete medical history, physical examination, 12 lead ECG, 24 hour Holter recording, and echocardiogram.

The diagnosis of JET was based on electrocardiographic evidence of a narrow complex tachycardia and atrioventricular dissociation.

All the patients underwent medical treatment according to the following regimen:

  • Digoxin (5–10 μg/kg/day orally) was given for controlling the symptoms of cardiac failure, in the presence of cardiomegaly and/or decreased left ventricular function

  • Propafenone (10–15 mg/kg/day orally) was used in all patients, alone or in combination with digoxin, as first step of treatment

  • Amiodarone (loading dose 12–27 mg/kg/day for seven days, followed by a maintenance dose of 5–12 mg/kg/day orally) was the next drug administered if propafenone proved ineffective after four days of treatment.

A Ic antiarrhythmic drug (propafenone or flecainide) was administered in combination with amiodarone when the latter proved ineffective alone in controlling the arrhythmia. This combination of drugs was also used in an attempt to reduce the dose of amiodarone, and prevent signs of …

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