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.

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.

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: Recognition and Modern Management Strategies.

Junctional ectopic tachycardia is a common dysrhythmia after congenital heart surgery that is associated with increased perioperative morbidity and mortality. Risk factors for development of junctional ectopic tachycardia include young age (neonatal and infant age groups); hypomagnesemia; higher-complexity surgical procedure, especially near the atrioventricular node or His bundle; and use of exogenous catecholamines such as dopamine and epinephrine. Critical care nurses play a vital role in early recognition of dysrhythmias after congenital heart surgery, assessment of hemodynamics affecting cardiac output, and monitoring the effects of antiarrhythmic therapy. This article reviews the underlying mechanisms of junctional ectopic tachycardia, incidence and risk factors, and treatment options. Currently, amiodarone is the pharmacological treatment of choice, with dexmedetomidine increasingly used because of its anti-arrhythmic properties and sedative effect.

The Impact of Time to Rate Control of Junctional Ectopic Tachycardia After Congenital Heart Surgery.

BACKGROUND: Junctional ectopic tachycardia (JET) after congenital heart disease (CHD) surgery is often self-limiting but is associated with increased risk of morbidity and mortality. Contributing factors and impact of time to achieve rate control of JET are poorly described. METHODS: From January 2010 to June 2015, a retrospective, single-center cohort study was performed of children who developed JET after CHD surgery . We classified the cohort into two groups: patients who achieved rate control of JET in ≤24 hours and in >24 hours. We examined factors associated with time to rate control and compared clinical outcomes (mortality, duration of mechanical ventilation, length of intensive care unit [ICU], and hospital stay) between the two groups. RESULTS: Our cohort included 27 children, with a median age of 3 (interquartile range: 0.7-38] months. The most common CHD lesions were ventricular septal defect (n = 10, 37%), tetralogy of Fallot (n = 7, 25.9%), and transposition of the great arteries (n = 4, 14.8%). In all, 15 (55.6%) and 12 (44.4%) patients achieved rate control of JET in ≤24 hours and >24 hours, respectively. There was a difference in median mechanical ventilation time (97 [21-145) vs 311 [100-676] hours; P = .013) and ICU stay (5.0 [2.0-8.0] vs 15.5 [5.5-32.8] days, P = .023) between the patients who achieved faster rate control than those who didn't. There was no difference in length of hospital stay and mortality between the groups. CONCLUSION: Our study demonstrated that time to achieve rate control of JET was associated with increased duration of mechanical ventilation and ICU stay.

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.

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.

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.

Nonpharmacologic control of postoperative supraventricular arrhythmias using AV nodal fat pad stimulation in a young animal open heart surgical model.

BACKGROUND: Supraventricular arrhythmias (junctional ectopic tachycardia [JET] and atrial tachyarrhythmias) frequently complicate recovery from open heart surgery in children and can be difficult to manage. Medical treatment of JET can result in significant morbidity. Our goal was to develop a nonpharmacological approach using autonomic stimulation of selective fat pad (FP) regions of the heart in a young canine model of open heart surgery to control 2 common postoperative supraventricular arrhythmias. METHODS AND RESULTS: Eight mongrel dogs, varying in age from 5 to 8 months and weighting 22±4 kg, underwent open heart surgery replicating a nontransannular approach to tetralogy of Fallot repair. Neural stimulation of the right inferior FP was used to control the ventricular response to supraventricular arrhythmias. Right inferior FP stimulation decreased baseline AV nodal conduction without altering sinus cycle length. AV node Wenckebach cycle length prolonged from 270±33 to 352±89 ms, P=0.02. Atrial fibrillation occurred in 7 animals, simulating a rapid atrial tachyarrhythmias. FP stimulation slowed the ventricular response rate from 166±58 to 63±29 beats per minute, P<0.001. Postoperative JET occurred in 7 dogs. FP stimulation slowed the ventricular rate during postoperative JET from 148±31 to 106±32 beats per minute, P<0.001, and restored sinus rhythm in 7/7 dogs. CONCLUSIONS: Right inferior FP stimulation had a selective effect on the AV node, and slowed the ventricular rate during postoperative JET and atrial tachyarrhythmias in our young canine open heart surgery model. FP stimulation may be a useful new technique for managing children with JET and atrial tachyarrhythmias.

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.

Taquicardia ectópica congénita de la unión: Tratamiento farmacológico en el primer año de vida / Congenital junctional ectopic tachycardia: Pharmacologic management during infancy

La forma congénita de la taquicardia ectópica de la unión (TEU) es una arritmia poco frecuente que suele presentar dificultades en su manejo farmacológico, con altas tasas de morbilidad y mortalidad. El objetivo de este trabajo fue informar la experiencia en el seguimiento y el tratamiento de esta forma de taquicardia supraventricular en pacientes menores de un año. Se identificaron siete pacientes con TEU congénita en 28 meses de seguimiento entre 2008 y 2010. El diagnóstico fue realizado en el primer día de vida en cuatro pacientes y dentro de los 150 días de vida en los 3 restantes. Sólo dos presentaron miocardiopatía dilatada. Ninguno presentó cardiopatía estructural. Se utilizó amiodarona en todos los pacientes, en un caso como única droga, asociándose a propanolol en cuatro. En un paciente se asoció flecainida a estos dos fármacos y en otro se la combinó con amiodarona. En un tiempo de seguimiento con un rango de 1-28 meses (media 12.2 meses, mediana 9.75 meses) en tres de los pacientes se consiguió obtener ritmo sinusal alternante con taquicardia nodal lenta; ninguno presentó efectos adversos secundarios a la medicación, ni deterioro de la función ventricular. Hubo sólo una muerte en el grupo estudiado. En conclusión, la combinación de fármacos antiarrítmicos (amiodarona más propranolol y eventualmente flecainida) constituye una alternativa válida para un adecuado control de la TEU congénita en pacientes menores de un año de edad.

Congenital junctional ectopic tachycardia (JET) is a rare arrhythmia that can be refractory to medical therapy with high morbidity and mortality rates. The aim of this study was to report our experience with pharmacologic management of congenital JET in infants. Seven patients with congenital JET were identified between 2008 and 2010. Only two of them presented dilated cardiomyopathy. There were no congenital structural defects. Amiodarone was given to all the patients, as single therapy in one, and in combination with propranolol in four. In one patient flecainide was administered together with amiodarone and propranolol, and in another patient was used combined with amiodarone. During follow- up with an average time of 12.2 months (median 9.75 months, range 1-28 months), sinus rhythm alternating with slow junctional tachycardia was successfully achieved in 3 patients; no side effects were detected. There was only one death in our study group. The combination of different antiarrhythmics (amiodarone plus propranolol, and eventually flecainide) is a valid option for rhythm control and management of JET in infants.

Isolated junctional tachycardia in a child after noncardiac surgery: an uncommon clinical presentation.

Isolated junctional tachycardia is rare in children but has been reported after cardiac surgery. To date, it has not been reported after noncardiac surgery. This report describes the case of a 3-year-old boy with no cardiac history who experienced transient junctional tachycardia after a right pyeloplasty. Medical therapy was not prescribed initially due to lack of symptoms. However, symptomatic junctional tachycardia recurred, prompting institution of oral beta-blocker therapy. Isolated junctional tachycardia should not be overlooked in the differential diagnosis of pediatric supraventricular tachycardia after noncardiac surgery.

Isolated junctional tachycardia in a patient with pheochromocytoma: an unusual presentation of an uncommon disease.

Pheochromocytoma is a rare disease, which often presents a diagnostic challenge due to a variable symptom presentation complex. We report the case of a child who presented with isolated symptoms of palpitations and was found to have junctional tachycardia with subsequent development of cardiomyopathy. Further evaluation revealed that she had an extra-adrenal pheochromocytoma, which was the cause of her arrhythmia and subsequent symptomatology.