creators_name: Sarubbi, Berardo creators_name: Vergara, Pasquale creators_name: D’Alto, Michele creators_name: Calabrò, Raffaele editors_name: Singh, Balbir editors_name: Lokhandwala, Yash editors_name: Francis, Johnson editors_name: Gupta, Anup type: journalp datestamp: 2005-04-24 lastmod: 2011-03-11 08:55:59 metadata_visibility: show title: Congenital Junctional Ectopic Tachycardia: Presentation And Outcome ispublished: pub subjects: ipej full_text_status: public keywords: Junctional Ectopic Tachycardia, Congenital abstract: 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 “post-operative” 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. 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