Ivabradine for congenital ectopic tachycardia in newborn.

INTRODUCTION: Congenital junctional ectopic tachycardia is a rare arrhythmia that occurs in patients without previous cardiac surgery. In this report, we wanted to present a 6-hour-old newborn with congenital junctional ectopic tachycardia resistant to conventional anti-arrhythmic medications, who was successfully treated with ivabradine and amiadarone combination. CASE: A six-hour-old newborn girl was hospitalised in neonatal ICU because transient tachypnoea of the newborn. She was tachycardic, and supraventricular tachycardia was noticed. There was no answer to the adenosine esmolol treatment; even synchronised direct cardioversion intravenous amiodarone was started. Junctional ectopic tachycardia was diagnosed. We have added propranolol to the treatment and followed patient for 2 days. On the fourth day, junctional ectopic tachycardia rhythm still persisted; therefore, ivabradine treatment was added to the treatment. On the following day, the heart rhythm was slowed to 110/min, and propranolol was stopped; intravenous amiodarone treatment was changed to the oral form. The rhythm turned into sinus; two days after starting ivabradine and oral amiodarone. CONCLUSION: Tachyarrhythmia originating in the atrioventricular node and atrioventricular junction including the bundle of His complex are junctional ectopic tachycardia. Congenital junctional ectopic tachycardia is rare, and it is mostly resistant to the conventional treatment.Ivabradine is a new anti-arrhythmic agent, used extensively to decrease sinus rate in the treatment of cardiac failure. Ivabradine may be an option for the resistant congenital ectopic tachycardia.

Lyme carditis presenting as paroxysmal junctional tachycardia and complete atrioventricular block in an adolescent.

We describe a case of a previously healthy adolescent who presented with junctional tachycardia and complete atrioventricular (AV) block due to Lyme carditis. The simultaneous presence of these findings suggested significant inflammation of the AV junction. Junctional tachycardia, particularly if seen in a patient with conduction abnormalities and potential tick exposure, should increase suspicion for Lyme carditis.

Atypical fetal junctional ectopic tachycardia: a case report and literature review.

BACKGROUND: Junctional ectopic tachycardia (JET) is caused by ectopic rhythms, originating in the atrioventricular node, typically with heart rate between 200 and 250 bpm. Herein, we present a case of fetal JET with normal fetal heart rate and a review of nine cases. CASE PRESENTATION: A 32-year-old, gravida 2, para 1, woman in whom fetal JET could not be diagnosed prenatally because the fetal heart rate was within the normal range. The fetus was diagnosed with premature restriction of the foramen ovale, and a cesarean section was performed, owing to the right heart overload that was characterized by fetal ascites and abnormal fetal Doppler velocity. Postnatally, the female neonate was diagnosed with JET on a 12-lead electrocardiogram, which revealed a neonatal heart rate of 158 bpm with narrow QRS and atrioventricular dissociation. After failure to respond to amiodarone therapy, she was treated with flecainide, which controlled the JET rate from 120 to 150 bpm. Fetal tachycardia with ventriculo-atrial (VA) dissociation or 1:1 VA conduction with a shorter VA interval than that of atrioventricular reentrant tachycardia confirmed the diagnosis of fetal JET. CONCLUSIONS: JET should be suspected even in the absence of tachycardia in patients with ductus venosus and pulmonary vein retrograde flow or tricuspid and mitral regurgitation without a cardiac anomaly, as tachycardia might sometimes be intermittent in cases of JET.

Perioperative junctional ectopic tachycardia associated with congenital heart disease: risk factors and appropriate interventions.

The risk factors and the appropriate interventions for perioperative junctional ectopic tachycardia (JET) in congenital heart disease (CHD) surgery have not been sufficiently investigated despite the severity of this complication. This study aimed to examine the risk factors and interventions for perioperative JET. From 2013 to 2020, 1062 surgeries for CHD (median patient age: 4.3 years, range 0.0-53.0) with or without a cardiopulmonary bypass (CPB) were performed at Hokkaido University, Japan. We investigated the correlation between perioperative JET morbidity factors, such as age, genetic background, CPB/aortic cross-clamp (ACC) time, use of inotropes and dexmedetomidine, STAT score, and laboratory indices. The efficacy of JET therapies was also evaluated. Of the 1062 patients, 86 (8.1%) developed JET. The 30-day mortality was significantly high in JET groups (7% vs. 0.8%). The independent risk factors for JET included heterotaxy syndrome [odds ratio (OR) 4.83; 95% confidence interval (CI) 2.18-10.07], ACC time exceeding 90 min (OR 1.90; CI 1.27-2.39), and the use of 3 or more inotropes (OR 4.11; CI 3.02-5.60). The combination of anti-arrhythmic drugs and a temporary pacemaker was the most effective therapy for intractable JET. Perioperative JET after CHD surgery remains a common cause of mortality. Inotrope use was a risk factor for developing JET overall surgery risk. In short ACC surgeries, heterotaxy syndrome could increase the risk of JET, which could develop even without inotrope use in long ACC surgeries. It is crucial not to delay the treatment in cases with unstable hemodynamics caused by this arrhythmia. It is recommended to reduce numbers not dose of inotropes.

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.

Junctional Ectopic Tachycardia Localization and Procedural Approach using Cryoablation.

BACKGROUND: Idiopathic junctional ectopic tachycardia (JET) may still be difficult to control with antiarrhythmic therapy. Transcatheter ablation can be challenging and may be associated with a high risk of unintended atrioventricular block. The objective of this manuscript is to report the procedural technique, the location of the successful ablation, and the procedural characteristics while utilizing 3D mapping for cryoablation of JET. METHODS: A retrospective analysis was performed on all patients who had undergone cryothermal ablation for the treatment of JET at a single center. Patient, arrhythmia, and procedural information and long-term outcomes were evaluated. RESULTS: Thirteen patients with JET were treated by cryothermal ablation. The JET arrhythmia burden varied greatly, generally with inadequate control on medications. Left ventricular dilation was present in three patients, and one patient had dilated cardiomyopathy. The median age at the time of procedure was 13 years, with median weight of 54.1 kg. The ectopic focus was ablated in 11/13 patients within the lower 2/3 of the triangle of Koch (TOK) with cryotherapy. Ablations, which were not successful, low in the TOK were associated with substantially longer procedures, and had a higher risk of recurrence. There was late resolution of the arrhythmia in two of three acutely unsuccessful ablations. There were no complications. CONCLUSION: In the majority of patients JET can be safely ablated with the use of cryotherapy. Foci not identified in the lower 2/3 of the TOK are associated with longer procedures, more lesions, and decreased chance for long-term success.

Virus myocarditis in a 1-month-old boy presenting as two types of paroxysmal supraventricular tachycardia.

Herein we describe the case of a 1-month-old boy with acute viral myocarditis, who presented with two kinds of paroxysmal supraventricular tachycardia, and who was cured after medical treatment. He was brought to the emergency room with poor feeding due to fever. On the third day of hospitalization, a narrow QRS tachycardia (180-200 beats/min) was detected. Echocardiography showed a high echoic area at the atrial septum around the atrioventricular node. The patient was clinically diagnosed with acute myocarditis. The narrow QRS tachycardia was diagnosed as incessant junctional ectopic tachycardia. The patient was treated with propranolol and landiolol. The frequency of the tachycardia decreased, but a different narrow QRS tachycardia was detected on the 15th day of hospitalization on electrocardiogram (220 beats/min), which was ascribed to atrioventricular nodal re-entrant tachycardia. Atenolol was effective for the tachycardia. At 2 years follow up, cardiac function was normal and tachycardia had not recurred.

Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.

Junctional ectopic tachycardia(JET) is a rare childhood arrhythmia originating from the area adjacent to the atrioventricular(AV) node. It often occurs after surgical procedures like repair of Tetralogy of Fallot, atrioventricular septal defect and ventricular septal defect, which are all performed in that area. While AV block (AVB) can occur after JET, it is very rare for late JET occurring after early postoperative AVB to be followed by normal sinus rhythm (NSR). There is no information in the literature related to the pathophysiology of this phenomenon. In this text, we present 4 patients who developed complete AV block(CAVB) in the early postoperative period (within the first 24h) after JET in late period (>72h) and returned to NSR with first-degree AV block and then NSR during follow-up. Based on these cases, we hypothesize that there is a link between late JET after early postoperative CAVB and return to NSR.

Junctional tachycardia in a child with non-rheumatic fever streptococcal pharyngitis.

Accelerated junctional rhythm has been reported in children in the setting of acute rheumatic fever; however, we describe a hitherto unreported case of isolated junctional tachycardia in a child with streptococcal pharyngitis, not meeting revised Jones criteria for rheumatic fever. A previously healthy, 9-year-old girl presented to the emergency department with complaints of sore throat, low-grade fever, and intermittent chest pain. She was found to have a positive rapid streptococcal antigen test. The initial electrocardiogram showed junctional tachycardia with atrioventricular dissociation in addition to prolonged and aberrant atrioventricular conduction. An echocardiogram revealed normal cardiac anatomy with normal biventricular function. The patient responded to treatment with amoxicillin for streptococcal pharyngitis. The junctional tachycardia and other electrocardiogram abnormalities resolved during follow-up.

Paradoxical responses to pacing maneuvers differentiating atrioventricular node reentrant tachycardia and junctional tachycardia.

A 40-year-old female presented at our hospital because of heart palpitations. During an electrophysiological study, atrioventricular (AV) conduction showed dual AV nodal physiology. Three types of supraventricular tachycardia (SVT) were induced. The initiation of SVT was reproducibility dependent on a critical A-H interval prolongation. An early premature atrial contraction during SVT repeatedly advanced the immediate His potential with termination of the tachycardia, indicating AV node reentrant tachycardia (AVNRT). However, after atrial overdrive pacing during SVT without termination of the tachycardia, the first return electrogram resulted in an AHHA response, consistent with junctional tachycardia. The mechanism of paradoxical responses to pacing maneuvers differentiating AVNRT and junctional tachycardia was discussed.

Impact of Different Diagnostic Criteria on the Reported Prevalence of Junctional Ectopic Tachycardia After Pediatric Cardiac Surgery.

OBJECTIVES: Junctional ectopic tachycardia is a frequent complication after pediatric cardiac surgery. A uniform definition of postoperative junctional ectopic tachycardia has yet to be established in the literature. The objective of this study is to analyze differences in the general and age-related prevalence of postoperative junctional ectopic tachycardia according to different diagnostic definitions. DESIGN: Data files and electrocardiograms of 743 patients (age, 1 d to 17.6 yr) who underwent surgery for congenital heart disease during a 3-year period were reviewed. The prevalence of postoperative junctional ectopic tachycardia in this cohort was determined according to six different definitions identified in the literature and one definition introduced for analytical purposes. Agreement between the definitions was analyzed according to Cohen κ coefficients. A receiver operating characteristic analysis was performed to determine the ability of different definitions to discriminate between patients with increased postoperative morbidity and without. SETTING: A university-affiliated tertiary pediatric cardiac PICU. PATIENTS: Infants and children who underwent heart surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The prevalence of postoperative junctional ectopic tachycardia ranged from 2.0% to 8.3% according to the seven different definitions. Even among definitions for which the general prevalence was almost equal, the distribution according to age varied. Most definitions used a frequency criterion to define postoperative junctional ectopic tachycardia. Definitions based on a fixed frequency criterion did not identify cases of postoperative junctional ectopic tachycardia in patients older than 12 months. The grade of agreement was moderate or poor between definitions using a fixed or dynamic frequency criterion and those not based on a critical heart rate (κ = 0.37-0.66). In the receiver operating characteristic analysis, the definition with a fixed frequency criterion of 180 beats/min or an age-related frequency criterion according to the 95th percentile showed the optimal cut-off value to determine increased postoperative morbidity. CONCLUSIONS: Different definitions of junctional ectopic tachycardia after pediatric cardiac surgery lead to relevant differences in the reported prevalence and age distribution pattern. A uniform definition of postoperative junctional ectopic tachycardia is needed to provide comparable study results and to improve the diagnosis of junctional ectopic tachycardia in pediatric patients.

Delayed cardiac dysrhythmias after fingolimod administration.

A 51-year-old woman with relapsing-remitting multiple sclerosis was initiated on fingolimod. She developed a Mobitz Type I (Wenckebach)second-degree atrioventricular (AV) heart block during the initial 6-hour monitoring. She was transferred to the emergency department for further monitoring, where she went into a junctional tachycardia then went back into a Mobitz Type I AV block. The patient was symptomatic with a heart rate nadir of 38 beats per minute and treated with atropine. Junctional tachycardia has not been previously reported with fingolimod use. Patients may require extended cardiac monitoring after fingolimod administration.

Junctional ectopic tachycardia after congenital heart surgery.

PURPOSE: In this literature review, we try to give anesthesiologists a better understanding about Junctional Ectopic Tachycardia (JET), a narrow complex tachycardia that frequently occurs during and after surgery for congenital heart disease. SOURCE: Information was found in the databases of Pubmed, Science Direct, Medline and the Cochrane Library, by using the mesh terms "Tachycardia, Ectopic Junctional", combined with "Diagnosis", "Etiology", "Physiopathology", "Complications" and "Therapy". The publication date of the articles ranged from 1990 to 2012. PRINCIPAL FINDINGS: Risk factors for the development JET are surgery near the AV node, a duration of cardiopulmonary bypass longer than 90 minutes, young age, the use of inotropic drugs and hypomagnesaemia. The diagnosis of Junctional Ectopic Tachycardia can be made on a 12-lead ECG, demonstrating a narrow-complex tachycardia with inverted P-waves and VA dissociation. Adenosine administration and an atrial electrocardiogram can help to confirm the diagnosis. If JET has a minimal impact on the hemodynamic status of the patient, risk factors should be avoided and the adrenergic tonus should be reduced. Hemodynamic unstable JET can be treated by amiodarone, hypothermia and pacing. Extracorporeal membrane oxygenation (ECMO) and radiofrequency or cryoablation are treatment options for life-threatening and resistant JET. CONCLUSION: JET is the most frequent arrhythmia during and after congenital cardiac surgery. The ECG is the only available method to diagnose JET, demonstrating inverted P-waves and VA-dissociation. Amiodarone seems to be the most effective treatment option, because it can restore sinus rhythm and reduces the JET rate.

Narrow irregular QRS tachycardia with AV dissociation: what is the mechanism?

A 39-year-old woman with no structural heart disease and frequent episodes of sudden onset palpitations was referred for the electrophysiological study. During the study, a slightly irregular narrow QRS tachycardia with AV dissociation was repeatedly induced and spontaneously terminated. Apparently, irregular cycles and termination of the tachycardia were related to the dissociated sinus rhythm: atrial depolarizations timed when the AV junction was refractory were able to reset the tachycardia, while early atrial depolarizations caused its termination. This observation was enough to diagnose the tachycardia mechanism in our case.

Response to atrial extrastimulus during supraventricular tachycardia: what is the mechanism?

We describe a case illustrating the potential challenges in distinguishing AV nodal reentry tachycardia (AVNRT) from automatic junctional tachycardia (JT). While an early atrial extrastimulus advanced the next His and ventricular depolarization without tachycardia termination, suggesting JT, other features indicated the correct diagnosis of AVNRT. This teaching case demonstrates a novel exception to a recently reported diagnostic pacing maneuver and illustrates the importance of considering response to multiple maneuvers in reaching a diagnosis of SVT mechanism.

Mechanistic heterogeneity of junctional ectopic tachycardia in adults.

Spontaneous junctional ectopic tachycardia (JET) in adults is rare, and the electrophysiologic mechanism has not been definitively established. Two patients who presented with JET, not associated with cardiac surgery, were evaluated and studied in the electrophysiology laboratory, and electrophysiologic and pharmacologic maneuvers were performed to assess the mechanisms of tachycardia. The junctional tachycardia in Patient 1 manifested characteristics consistent with a triggered mechanism, and was sensitive to adenosine. The junctional tachycardia in Patient 2 manifested characteristics consistent with abnormal automaticity, and was insensitive to adenosine. This is a rare clinical example of abnormal automaticity. These two cases demonstrate that JET may be due to multiple mechanisms, with data consistent with triggered activity and abnormal automaticity.

Clinical risk prediction score for postoperative accelerated junctional rhythm and junctional ectopic tachycardia in children with congenital heart disease.

BACKGROUND: Accelerated junctional rhythm (AJR) and junctional ectopic tachycardia (JET) are common postoperative arrhythmias associated with morbidity/mortality. Studies suggest that pre- or intraoperative treatment may improve outcomes, but patient selection remains a challenge. OBJECTIVES: The purpose of this study was to describe contemporary outcomes of postoperative AJR/JET and develop a risk prediction score to identify patients at highest risk. METHODS: This was a retrospective cohort study of children aged 0-18 years undergoing cardiac surgery (2011-2018). AJR was defined as usual complex tachycardia with ≥1:1 ventricular-atrial association and junctional rate >25th percentile of sinus rate for age but <170 bpm, whereas JET was defined as a rate >170 bpm. A risk prediction score was developed using random forest analysis and logistic regression. RESULTS: Among 6364 surgeries, AJR occurred in 215 (3.4%) and JET in 59 (0.9%). Age, heterotaxy syndrome, aortic cross-clamp time, ventricular septal defect closure, and atrioventricular canal repair were independent predictors of AJR/JET on multivariate analysis and included in the risk prediction score. The model accurately predicted the risk of AJR/JET with a C-index of 0.72 (95% confidence interval 0.70-0.75). Postoperative AJR and JET were associated with prolonged intensive care unit and hospital length of stay, but not with early mortality. CONCLUSION: We describe a novel risk prediction score to estimate the risk of postoperative AJR/JET permitting early identification of at-risk patients who may benefit from prophylactic treatment.