Late occurrence of adenosine-sensitive focal junctional tachycardia in complex congenital heart disease.
BACKGROUND
Although supraventricular tachycardia in complex congenital heart disease (CHD) has been reported after surgical repair, its exact electrophysiologic identification has been limited to intraatrial reentrant tachycardia (IART). Moreover, junctional tachycardia (JT) has not previously been described as a cause of late postoperative arrhythmia.


RESULTS
Since 1993, a total of 12 patients with congenital heart disease presented with paroxysmal focal JT. The patients with only typical immediate postoperative junctional ectopic tachycardia were excluded. Medical records, standard electrocardiography and Holter monitoring were reviewed. An intracardiac electrophysiologic (EP) study was performed in 11 patients. Ten patients were in post-Fontan status (5.7% of total Fontan survivors). Focal JT occurred more frequently in heterotaxy syndrome among the Fontan survivors (7/52 vs. 3/124; P < 0.05). The commonest anatomy of the atrioventricular (AV) junction was complete AV canal in 8 patients. EP characteristics of focal JT were as follows: (1) various tachycardia mechanisms were identified (increased automaticity or a triggered mechanism in 6/11, and focal reentry in 5/11, including one concealed nodofascicular pathway) (2) ventriculoatrial conduction during tachycardia was either dissociation (7/12) or variable (5/12) (3) All JTs were terminated by adenosine. Class III antiarrhythmic agent was effective in 5/6. His bundle ablation was performed in one Fontan patient, who already had pacemaker because of accompanying intractable IART and sinus node dysfunction.


CONCLUSIONS
Focal JT may be a source of late term supraventricular tachycardia in patients with complex CHD. The tachycardia mechanism was either automatic/triggered or reentrant. In all patients, JT was effectively terminated by adenosine.
Bae
Eun-Jung
EJ
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea.
Noh
Chung-Il
CI
Choi
Jung-Yun
JY
Yun
Yong-Soo
YS
Kim
Woong-Han
WH
Lee
Jeong-Ryul
JR
Kim
Yong-Jin
YJ
D000241:Adenosine; D000293:Adolescent; D000328:Adult; D000889:Anti-Arrhythmia Agents; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D013613:Tachycardia, Ectopic Junctional / Q000150:complications* / Q000503:physiopathology; D013617:Tachycardia, Supraventricular / Q000209:etiology* / Q000503:physiopathology
D000889:Anti-Arrhythmia Agents; D000241:Adenosine
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