Coexistence of sinus bradycardia and junctional tachycardia in a patient with fibrotic atrial cardiomyopathy.

A 65-year-old woman was referred for catheter ablation in the treatment of persistent tachycardia after surgery for atrial fibrillation and mitral regurgitation. Bipolar voltage mapping of both atria revealed that severe and extensive atrial fibrosis isolated the sinoatrial node from the atrioventricular junction and led to the coexistence of sinus bradycardia and persistent junctional tachycardia.

Ivabradine for junctional ectopic tachycardia in post congenital heart surgery.

We report two cases of malignant junctional ectopic tachycardia (JET), in infants following congenital heart surgery. After the failure of conventional therapy the arrhythmia was controlled by oral Ivabradine, a drug which is routinely used to lower heart rate in angina and heart failure in adult practice.

Efficacy of Prophylactic Dexmedetomidine in Preventing Postoperative Junctional Ectopic Tachycardia After Pediatric Cardiac Surgery.

BACKGROUND: Postoperative junctional ectopic tachycardia is one of the most serious arrhythmias that occur after pediatric cardiac surgery, difficult to treat and better to be prevented. Our aim was to assess the efficacy of prophylactic dexmedetomidine in preventing junctional ectopic tachycardia after pediatric cardiac surgery. METHODS AND RESULTS: A prospective controlled study was carried out on 90 children who underwent elective cardiac surgery for congenital heart diseases. Patients were randomized into 2 groups. Group I (dexmedetomidine group): 60 patients received dexmedetomidine; Group II (Placebo group): 30 patients received the same amount of normal saline intravenously. The primary outcome was the incidence of postoperative junctional ectopic tachycardia. Secondary outcomes included bradycardia, hypotension, vasoactive inotropic score, ventilation time, pediatric cardiac care unit stay, length of hospital stay, and perioperative mortality. The incidence of junctional ectopic tachycardia was significantly reduced in the dexmedetomidine group (3.3%) compared with the placebo group (16.7%) with P<0.005. Heart rate while coming off cardiopulmonary bypass was significantly lower in the dexmedetomidine group (130.6±9) than the placebo group (144±7.1) with P<0.001. Mean ventilation time, and mean duration of intensive care unit and hospital stay (days) were significantly shorter in the dexmedetomidine group than the placebo group (P<0.001). However, there was no significant difference between the 2 groups as regards mortality, bradycardia, or hypotension (P>0.005). CONCLUSION: Prophylactic use of dexmedetomidine is associated with significantly decreased incidence of postoperative junctional ectopic tachycardia in children after congenital heart surgery without significant side effects.

Clinical Features and Sites of Ablation for Patients With Incessant Supraventricular Tachycardia From Concealed Nodofascicular and Nodoventricular Tachycardias.

OBJECTIVES: This study sought to describe the clinical features and sites of successful ablation for incessant nodofascicular (NF) and nodoventricular (NV) tachycardias. BACKGROUND: Incessant supraventricular tachycardias have been associated with tachycardia-induced cardiomyopathies and have been previously attributed to permanent junctional reciprocating tachycardias, atrial tachycardias, and atrioventricular nodal re-entrant tachycardias. Incessant concealed NF and NV tachycardias have not been described previously. METHODS: Three cases of incessant concealed NF and NV re-entrant tachycardias were identified from 2 centers. RESULTS: The authors describe 3 cases with incessant supraventricular tachycardia resulting from NV (2 cases) and NF (1 case) pathways. Atrioventricular nodal re-entrant tachycardia was excluded by His synchronous premature ventricular complexes that either delayed or terminated the tachycardia. Ventricular pacing showed constant and progressive fusion in cases 1 and 3. In 2 cases, there was spontaneous initiation with a 1:2 response (cases 1 and 3); the presence of retrograde longitudinal dissociation or marked decremental pathway conduction in cases 1 and 3 sustains these tachycardias. The NV pathway was successfully ablated in the slow pathway region in case 3 and at the right bundle branch in case 1. The NF pathway was successfully ablated within the proximal coronary sinus in case 2. CONCLUSIONS: This is the first report of incessant supraventricular tachycardia using concealed NF or NV pathways. These tachycardias demonstrated spontaneous initiation from sinus rhythm with a 1:2 response and retrograde longitudinal dissociation or marked decremental pathway conduction. Successful ablation was achieved at either right-sided sites or within the coronary sinus.

A Low-Cost Simulation Model for R-Wave Synchronized Atrial Pacing in Pediatric Patients with Postoperative Junctional Ectopic Tachycardia.

BACKGROUND: Postoperative junctional ectopic tachycardia (JET) occurs frequently after pediatric cardiac surgery. R-wave synchronized atrial (AVT) pacing is used to re-establish atrioventricular synchrony. AVT pacing is complex, with technical pitfalls. We sought to establish and to test a low-cost simulation model suitable for training and analysis in AVT pacing. METHODS: A simulation model was developed based on a JET simulator, a simulation doll, a cardiac monitor, and a pacemaker. A computer program simulated electrocardiograms. Ten experienced pediatric cardiologists tested the model. Their performance was analyzed using a testing protocol with 10 working steps. RESULTS: Four testers found the simulation model realistic; 6 found it very realistic. Nine claimed that the trial had improved their skills. All testers considered the model useful in teaching AVT pacing. The simulation test identified 5 working steps in which major mistakes in performance test may impede safe and effective AVT pacing and thus permitted specific training. The components of the model (exclusive monitor and pacemaker) cost less than $50. Assembly and training-session expenses were trivial. CONCLUSIONS: A realistic, low-cost simulation model of AVT pacing is described. The model is suitable for teaching and analyzing AVT pacing technique.

Successful use of intravenous amiodarone in a child with combined postoperative junctional and ectopic tachycardias.

Early postoperative arrhythmias are a known complication of cardiac surgery. It is unusual, however, to encounter postoperative junctional and ectopic atrial tachycardias in the same patient. We describe our experience with a 2-year-old girl who suffered both these tachycardias after repair of a ventricular septal defect, the abnormal rhythms being controlled solely with intravenous administration of amiodarone.

Cryoablation of junctional tachycardia at high risk of atrio-ventricular block.

INTRODUCTION: Transcatheter cryoablation is an alternative option for the treatment of supraventricular tachycardia, due to its very low risk of permanent atrio-ventricular block. However, the overcost of cryocatheter and the high recurrence rate of this emerging technology braked its large use. This study reports the results of an approach using cryoablation for the treatment of junctional tachycardia (JT) in selected patients at high risk of atrio-ventricular (AV) block. PATIENTS AND METHODS: Out of a series of 199 patients with JT treated by catheter ablation, 26 benefited from cryoablation (mean age 32.8+/-15 years, 15 males). The indications were the presence of an accessory pathway with a high risk of atrio-ventricular block (n=7), a slow pathway difficult to ablate, with a risk of atrio-ventricular block (n=7), a recurrence after a RF procedure, during which a transient atrio-ventricular block has occurred (n=4), and finally patients at young age (n=8). RESULTS: The primary success rate was 92%. No permanent AV block has been reported, neither with RF nor with cryoablation. The recurrence rate at 9+/-10 months was at 29% after cryoablation and 8.6% after RF. In case of AV nodal reentrant tachycardia, the additional cost of cryotherapy catheter has been avoided in 76.85% of cases. The use of a cryotherapy catheter and RF catheter has been necessary for the remaining cases. CONCLUSION: This study demonstrates that an approach, reserving cryoablation in selected patients at high risk of AV block is an alternative strategy to "the systematic use" of cryotherapy in the ablation of JT with a high efficacy, an excellent safety and a reduced cost.

Cryoablation of congenital familial ectopic tachycardia with preservation of atrioventricular nodal function in an infant.

A 1-year-old child weighing 8 kg with familial congenital junctional ectopic tachycardia that was difficult to treat with antiarrhythmic medications underwent successful cryoablation with preservation of atrioventricular (AV) nodal function. This report describes a strategy of cryoablation in conjunction with a 3-D mapping system and the use of atrial overdrive pacing during cryothermal application to assess AV nodal function.

Transcatheter cryothermal ablation of junctional ectopic tachycardia in the normal heart.

BACKGROUND: Junctional ectopic tachycardia in the normal heart is rare and often is resistant to pharmacologic management. Transcatheter ablation using radiofrequency energy places the AV node at risk. OBJECTIVES: The purpose of this study was to report our experience with transcatheter cryothermal ablation using three-dimensional mapping in six patients with junctional ectopic tachycardia. METHODS: A review of clinical and electrophysiologic data was performed on all patients with structurally normal hearts who underwent cryothermal ablation for treatment of junctional ectopic tachycardia at two institutions. RESULTS: Six patients (age 7.7-36.5 years) underwent attempted transcatheter cryothermal ablation using three-dimensional mapping. Only one patient had achieved arrhythmia suppression on medical management. Cryothermal mapping (-30 degrees C) localized the junctional focus while normal conduction was monitored. The junctional focus was high in the triangle of Koch in four patients and was low in one patient. The sixth patient had only one run of junctional ectopic tachycardia during the procedure and therefore received an empiric cryoablation (-70 degrees C) lesion. Subsequent cryoablation lesions were delivered at and around the junctional focus. In one patient, cryomapping eliminated the junctional focus but resulted in transient complete AV block; therefore, cryoablation was not performed. All patients who received the cryoablation lesions had elimination of their junctional ectopic tachycardia at 6-week follow-up. The patient who did not receive a cryoablation lesion remained in a slower junctional rhythm at follow-up. CONCLUSION: Cryoablation of junctional ectopic tachycardia is safe and effective. Nonetheless, proximity to the His-Purkinje system may preclude success. Empiric cryoablation can be effective; cryotherapy may not yield immediate success, but a delayed salutary effect can follow.

A prospective analysis of the incidence and risk factors associated with junctional ectopic tachycardia following surgery for congenital heart disease.

This study was designed to evaluate the incidence and risk factors associated with the occurrence of junctional ectopic tachycardia (JET) in patients after congenital heart surgery. We prospectively analyzed cardiac rhythm status in 336 consecutive patients undergoing surgery for congenital heart disease at our institution during a 1-year period. The incidence of JET was 8% (27/336). Repairs with the highest incidence of JET were arterial switch operation (3/13, 23%), atrioventricular (AV) canal repair (4/19, 21%), and Norwood repair (2/10, 20%). Compared to patients with no arrhythmias, patients with JET were more likely to be younger (2.75 +/- 2.44 vs 5.38 +/- 7.25 years, p < 0.01), have had longer cardiopulmonary bypass times (126 +/- 50 vs 85 +/- 73, p < 0.01), and have a higher inotrope score (6.26 +/- 7.55 vs 2.41 +/- 8.11, p < 0.01). By multivariate analysis, ischemic time was the only factor associated with JET [odds ratio, 1.01 (confidence interval, 1.005-1.02); p = 0.0014). The presence of JET did not correlate with electrolyte abnormalities. JET is not necessarily related to surgery near the His bundle or hypomagnesemia. Longer ischemic time is the best predictor of JET. Patients undergoing arterial switch operation, AV canal repair, and Norwood repair are at highest risk of postoperative JET and should be considered for prophylactic therapy.

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. METHODS AND 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. CONCLUSION: 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.

Supraventricular tachycardia in children.

The mechanisms causing different supraventricular tachycardias can be identified with the aid of the 12-lead ECG using Tipple's approach. The main aims of this retrospective study were to use the 12-lead ECG to determine the underlying mechanisms of supraventricular arrhythmias and to evaluate the effectiveness of the treatment modalities used. Forty-one patients were included in the study. The main findings were: nine of the 41 patients had atrial tachycardias while junctional tachycardia occurred in 32/41 of our patients. The underlying mechanisms causing the junctional tachycardias were: AVNRT (n = 21), AVRT (n = 10) and JET (n = 1). Of the 10 patients presenting with AVRT, eight were less than one year old. AVNRT occurred more often in the older age group (>1 year of age). Fifteen of the 41 patients had spontaneous cessation of their supraventricular tachycardia. The drug most commonly used during the acute and long-term phases was digoxin. Amiodarone was used in six patients with an 80% success rate. In the early 80s verapamil was used in five patients with a 100% success rate. It is important to note that verapamil is no longer used in children due to its side effects. Lately, adenosine phosphate is the drug of choice in most supraventricular tachycardias. The management of supraventricular tachycardias in paediatric practice is mainly based on clinical studies and individual experience. Care must therefore be taken to choose medication regimens that are likely to be effective with the minimum risk of potentiating abnormal haemodynamics or conduction.

Reporte de un caso de tauicardia reciprocante de la unión o taquicardia de Coumel

La taquicardia reciprocante de la unión o taquicardia de coumel fue descrita por primera vez hace 30 años. Previamente se consideraba como una taquicardia originada en la aurícula. El substrato anatómico más aceptado involucra una vía accesoria con conducción ventrículo atrial con propiedades decrementales. Es por lo general una tauicardia de personas jóvenes que por ser frecuentemente incesante puede producir taquicardiomiopatía y en el electrocardiograma de superficie se carcteriza por la presencia de ritmo sinusal sin onda delta. tres patologías comparten el patrón electrocardiográfico de taquicardias de complejos estrechos con intervalo RP>PR y onda P negativa en derivaciones inferiores (taquicardia atrial, taquicardia por reentrada intranodal variedad no común y taquicardia reciprocante de la unión). El diagnóstico correcto de la taquicardia tiene consecuencias importantes para el paciente. El presente caso ilustra cómo hacer el diagnóstico diferencial correcto entre estas tres arritmias con la ayuda del estudio electrofisiológico, cómo demostrar las propiedades decrementales de la vía accesoria y cómo un paciente joven refractario al tratamiento farmacológico puede ser curado utilizando ablación con radiofrecuencia, con una mínima morbilidad.

Effect of adenosine and verapamil in catecholamine-induced accelerated atrioventricular junctional rhythm: insights into the underlying mechanism.

Accelerated AV junctional rhythm is postulated to be due to enhanced automaticity of a high AV junctional focus. The adenosine response of this rhythm was tested in 17 patients (7 males, 12-83 years). The indications of electrophysiology study were nonspecific palpitation (n = 5), unexplained syncope (n = 6), postablation of accessory pathways (n = 4), and postmodification of AV nodal reentry tachycardia (n = 2). The sinus node and AV nodal functions were normal. Pacing and programmed electrical stimulation failed to induce any arrhythmia at baseline. The accelerated junctional rhythm (cycle length = 553 +/- 134 ms) was initiated spontaneously in all patients after isoproterenol infusion (1-2 micrograms/min). It was not suppressible by overdrive pacing. Cessation of isoproterenol infusion terminated the rhythm in all patients. Adenosine (6 mg) reproducibly terminated the accelerated junctional rhythm in all patients. In six patients, adenosine suppressed the junctional rhythm without producing AV nodal block. In the other 11 patients, the junctional rhythm was terminated prior to the occurrence of AV nodal block. Verapamil was tested in ten patients and 5 mg of intravenous verapamil terminated the junctional rhythm in all patients. In conclusion, the mechanism of catecholamine-induced accelerated AV junctional rhythm is most likely enhanced automaticity, and catecholamine-induced accelerated AV junctional automaticity is sensitive to adenosine and verapamil. Adenosine appears to have differential effects on catecholamine-enhanced AV junctional automaticity and AV nodal conduction. This suggests that, under catecholamine stimulation, adenosine may have different mechanisms of action on AV nodal conduction and automaticity.

[High frequency catheter ablation in young patients with permanent junctional reentry tachycardia and ectopic atrial tachycardia]. / Hochfrequenzstrom-Katheterablation bei jungen Patienten mit permanenter junktionaler Reentry-Tachykardie und atrialer ektoper Tachykardie.

Ten young patients with a median age of 10.5 (range: 6 to 31) years suffering from the permanent form of junctional reciprocating tachycardia (PJRT; n = 7) and ectopic atrial tachycardia (AET; n = 3) were treated by transcatheter radiofrequency current application. Indications for interventional therapy were failure of medical therapy after a median of 3 antiarrhythmic drugs in all patients, syncope in 1, and impaired left ventricular function in 4 patients (PJRT n = 3, AET n = 1). The intervention was primarily successful in all patients. Median fluoroscopy time during the ablation procedure was 17 (10 to 70) minutes. A median of 3 (1 to 14) radiofrequency (500 kHz) pulses was delivered at a target temperature of 70 degrees C or with 30 W for 30 s. One patient had a recurrence of PJRT and was successfully treated by a repeat ablation. After a median follow-up of 14 (2 to 61) months, all patients are free of drug treatment and in normal sinus rhythm. Left ventricular function has normalized in patients with follow-up > 6 months. Radiofrequency catheter ablation was a safe and effective therapy in young patients with drug-resistant forms of chronic-permanent supraventricular tachycardia.

Permanent junctional re-entry tachycardia. A multicentre long-term follow-up study in infants, children and young adults.

AIMS: Permanent junctional re-entry tachycardia is a relatively uncommon form of re-entry tachycardia with antegrade conduction occurring through the atrioventricular node and retrograde conduction over an accessory pathway usually located in the postero-septal region. It was the aim of the study to investigate the course of permanent junctional re-entry tachycardia with particular regard to the effectiveness of pharmacological treatment and ablation procedures; evaluation was performed with respect to the patient's symptoms, tachycardia rate, frequency of the tachycardia and left ventricular function. METHODS AND RESULTS: The long-term follow-up of 32 patients with permanent junctional re-entry tachycardia was evaluated. The first presentation with supraventricular tachycardia occurred between the 27th week of gestation and 27 years. The tachycardia rate ranged from 100 to 250 beats.min-1. During Holter-ECG, permanent junctional re-entry tachycardia was documented as present for over 50% of the time in 24 h in 22 patients (69%). Left ventricular performance was impaired in nine patients (28%) due to a tachycardia-related cardiomyopathy. Symptoms or signs of heart failure were mild to moderate in eight and severe in four patients; 20 patients showed no clinical impairment. Follow-up time was 1 to 31 (mean 10) years; current age of the patients ranged from 1.5 months to 35 (mean = 15 x 3) years. Four patients needed no therapy because of the infrequency of permanent junctional re-entry tachycardia episodes. Twenty-five patients initially received antiarrhythmic drugs, which were effective or partially effective in 14 (56%). Eight of them are still on medical therapy; in five treatment was discontinued because of absence of symptoms. Eleven patients had ablation of the accessory pathway during follow-up, three underwent ablation as a primary procedure. CONCLUSION: Permanent junctional re-entry tachycardia in our experience is an arrhythmia with a large variety of clinical symptoms. Patients with a slow tachycardia rate and infrequent episodes of tachycardia may never develop symptoms and therefore do not need any therapy. Patients with frequent permanent junctional re-entry tachycardia, a fast tachycardia rate and impaired left ventricular function need effective therapy. In infancy and early childhood medical therapy is recommended as a first option, whereas in older and symptomatic patients catheter ablation is an effective and safe procedure.