Congenital Junctional Ectopic Tachycardia: Presentation And Outcome

Introduction Junctional ectopic tachycardia (JET) is a rare type of supraventricular arrhythmia. Even if its management has improved in recent years, it remains a great challenge for the cardiologist. Two are the possible clinical presentations of this arrhythmia: as a primary idiopathic disorder during infancy, configuring the so called "congenital" JET, or more often as a transient phenomenon immediately after surgery for congenital heart disease, giving rise to the "postoperative" variety. The congenital form, firstly described as a distinct entity by Coumel et al. in 19761, usually occurs in the first six months of life presenting as a persistent sustained form, lasting up to 90% of the time. Its clinical presentation may be dramatic, being associated in up to 60% of cases with cardiomegaly and/or heart failure. Congenital JET is hampered by high mortality. Secondary dilated cardiomyopathy, ventricular fibrillation and sudden cardiac death have also been reported 2,3.