1/48. Junctional ectopic tachycardia evolving into complete heart block. Transition from congenital junctional ectopic tachycardia to complete AV block was observed in an 8 month old girl, over a 36 hour period, during initial hospital admission. Two years later she had evidence of a rapidly increasing left ventricular end diastolic diameter, associated with lowest heart rates during sleep of < 30 beats/min. A transvenous permanent pacemaker was therefore implanted. This finding supports the idea that a pathological process in the area of the AV junction, initially presenting as junctional ectopic tachycardia may later extend to sudden complete atrioventricular block. ( info) |
2/48. Paired ventricular pacing: an alternative therapy for postoperative junctional ectopic tachycardia in congenital heart disease. Junctional ectopic tachycardia (JET) is one of the most life-threatening postoperative arrhythmias in children with congenital heart disease, and medical management is difficult. Paired ventricular pacing (PVP) may provide a safe alternative mode of management. We evaluated the safety and efficacy of PVP for the management of postoperative JET in patients with congenital heart disease. A retrospective collection of data was done from 1981-1995. PVP was successfully tried in five postoperative patients (age range: 37 days to 22 years, median: 10 months). Onset of JET was 3-60 hours (mean /- SD, 19 /- 23 hours) postoperatively. The maximal JET rate was 261 /- 39 beats/min. PVP was used as the first line of management in three patients and was successful in all patients. It resulted in an instantaneous increase in blood pressure from 66 /- 9 to 94 /- 15 mmHg (42% increase) and was required for 12 /- 14 hours (range 2-36 hours). No complications were noted. Therefore, in our experience, this is a safe alternative modality for the control of postoperative JET. ( info) |
| Junctional ectopic tachycardia is an arrhythmia seen principally in infants and children, in adults it is even more rare and is difficult to treat with antiarrhythmic drugs and is associated with a poor prognosis. To our knowledge, there is not information about treatment with nonpharmacological methods in adults. We report two adult patients with junctional ectopic tachycardia who underwent successful radiofrequency catheter ablation of the automatic focus located in the His bundle region. The tachycardia was eliminated in both patients with AV block in one and the AV conduction was preserved in the other. After six and 48 months of follow-up both patients are asymptomatic and free of recurrences. ( info) |
| Junctional ectopic tachycardia (JET) is a rare cardiac arrhythmia characterized by atrio-ventricular dissociation, a high rate junctional escape rhythm and poor clinical tolerance in neonates and infants. sudden infant death has been reported. The intra-uterine presentation of this arrhythmia is unknown. We report a familial form of JET with antenatal diagnosis. A sustained tachycardia at a rate of 170 beats/min with a 1:1 conduction was diagnosed in a hydropic fetus at a gestational age of 32 weeks. The older brother had presented with prenatal hydrops and junctional ectopic tachycardia was diagnosed at birth. Assuming that this arrhythmia was a JET, amiodarone was given to the mother in order to control the fetal tachycardia. The arrhythmia persisted with a 1/1 pattern but at a slower ventricular rate (140 beats/min). The ECG performed at birth revealed a narrow QRS tachycardia with a ventricular rate of 180 beats/min and a 1/1 retrograde conduction. amiodarone therapy was continued with the addition of propanolol. Postnatal echocardiography revealed normal chambers and left ventricular dysfunction with a left ventricular shortening fraction of 17 per cent. Subsequent ECGs and Holter monitoring demonstrated typical electrocardiographic features of JET. Both parents had a normal ECG and Holter monitoring. A fetal tachycardia of moderately high rate with a 1/1 retrograde conduction and poor cardiac tolerance can be due to JET. In such cases, the use of amiodarone can be considered as a first line drug. ( info) |
5/48. Successful radiofrequency ablation in an infant with drug-resistant permanent junctional reciprocating tachycardia. Over the past decade, the technique of radiofrequency ablation has evolved substantially. Currently, most forms of cardiac arrhythmias seen in children can be treated with good long-term results and low risk of adverse outcome. Curative arrhythmia treatment with this technique, however, is still uncommon in neonates and infants. Reported here is our experience in the management of an 8-week-old with drug-resistant permanent junctional reciprocating tachycardia. ( info) |
6/48. Transient QT prolongation with torsades de pointes tachycardia after ablation of permanent junctional reciprocating tachycardia. INTRODUCTION: catheter ablation with radiofrequency energy is a curative therapy in patients with permanent junctional reciprocating tachycardia (PJRT). methods AND RESULTS: For the first time, we report a case of transient QT prolongation with torsades de pointes tachycardia 18 hours after successful radiofrequency energy ablation of PJRT in a 25-year-old woman with tachycardia-induced cardiomyopathy. Of note, the torsades de pointes occurred in the absence of bradycardia, electrolyte disturbances, or QT-prolonging drugs. This patient initially was thought to have a hereditary long qt syndrome that was unmasked by PJRT ablation. Therefore, the patient received an implantable defibrillator in addition to beta-blocker therapy, which was discontinued 6 months later. Surprisingly, the QT interval completely normalized within 1 week after PJRT ablation, and the patient remained free of arrhythmias during a follow-up period of 4.5 years. CONCLUSION: patients with incessant tachyarrhythmias should undergo ECG monitoring for at least 24 hours following successful radiofrequency catheter ablation because transient QT prolongation with torsades de pointes may occur even in the absence of bradycardia, QT-prolonging drugs, or electrolyte disturbances. ( info) |
7/48. Congestive heart failure treated by the upgrade from VVI to DDD pacing. The case is presented of an elderly woman with normal left ventricular (LV) systolic function and VVI pacing complicated by severe congestive heart failure. The symptoms and findings of congestive heart failure became refractory to medical treatment and resolved with the upgrade of the VVI to a DDD system. Right heart catheterization during VVI pacing showed increased mean pulmonary capillary wedge and right atrial pressures both being normalized under DDD pacing. This case report illustrates the need to consider permanent physiological pacing in elderly patients, even in presence of normal LV systolic function, to ensure AV synchrony when the atrium can be paced, since diastolic LV dysfunction is quite common in these subjects. ( info) |
8/48. catheter ablation of atrioventricular junction via retrograde route in a patient with single ventricle. Radiofrequency catheter ablation of the atrioventricular junction (AVJ) was performed by the retrograde route in a 19-year-old woman with atrial fibrillation and single ventricle following the bidirectional Glenn procedure. Two energy applications resulted in complete atrioventricular block and dependence on an epicardial ventricular pacemaker. ( info) |
| The congenital form of junctional ectopic tachycardia has never been reported during the prenatal period. We describe a case in which the diagnosis could be ascertained in utero with the superior vena cava/ascending aorta Doppler approach. The diagnosis was based on the evidence of ventricular tachycardia with atrioventricular (AV) dissociation and the simultaneous occurrence of atrial and ventricular contractions during an episode of tachycardia with 1:1 AV relationship. ( info) |
10/48. Atrial pacing during radiofrequency ablation of junctional ectopic tachycardia--a useful technique for avoiding atrioventricular bloc. Radiofrequency catheter ablation (RFCA) was performed on a 5-year-old boy with congenital junctional ectopic tachycardia (JET) that was refractory to medical management. Because of the lack of retrograde atrial depolarization during tachycardia, radiofrequency energy was delivered during atrial overdrive pacing to confirm the presence of preserved atrioventricular (AV) conduction. Although the procedure was complicated by complete right bundle branch block after ablation of the para-Hissian region, the patient regained sinus rhythm accompanied by normal AV conduction. Rapid atrial pacing during RFCA of JET may be safely used to avoid AV block. ( info) |