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.

Hemodynamic profile effects of PM101 amiodarone formulation in patients with post-operative tachyarrhythmias.

Amiodarone may be considered for patients with junctional ectopic tachycardia refractory to treatment with sedation, analgesia, cooling, and electrolyte replacements. There are currently no published pediatric data regarding the hemodynamic effects of the newer amiodarone formulation, PM101, devoid of hypotensive agents used in the original amiodarone formulation. We performed a single-center, retrospective, descriptive study from January 2012 to December 2020 in a pediatric ICU. Thirty-three patients were included (22 male and 11 female) between the ages of 1.1 and 1,460 days who developed post-operative junctional ectopic tachycardia or other tachyarrhythmias requiring PM101. Data analysis was performed on hemodynamic parameters (mean arterial pressures and heart rate) and total PM101 (mg/kg) from hour 0 of amiodarone administration to hour 72. Adverse outcomes were defined as Vasoactive-Inotropic Score >20, patients requiring ECMO or CPR, or patient death. There was no statistically significant decrease in mean arterial pressures within the 6 hours of PM101 administration (p > 0.05), but there was a statistically significant therapeutic decrease in heart rate for resolution of tachyarrhythmia (p < 0.05). Patients received up to 25 mg/kg in an 8-hour time for rate control. Average rate control was achieved within 11.91 hours and average rhythm control within 62 hours. There were four adverse events around the time of PM101 administration, with three determined to not be associated with the medication. PM101 is safe and effective in the pediatric cardiac surgical population. Our study demonstrated that PM101 can be used in a more aggressive dosing regimen than previously reported in pediatric literature with the prior formulation.

A Comparative Effectiveness Systematic Review and Meta-analysis of Drugs for the Prophylaxis of Junctional Ectopic Tachycardia.

BACKGROUND: Junctional Ectopic Tachycardia (JET) is an arrhythmia originating from the AV junction, which may occur following congenital heart surgery, especially when the intervention is near the atrioventricular junction. OBJECTIVE: The aim of this systematic review and meta-analysis is to compare the effectiveness of amiodarone, dexmedetomidine, and magnesium in preventing JET following congenital heart surgery. METHODS: This meta-analysis was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) statement, where 11 electronic databases were searched from the date of inception to August 2020. The incidence of JET was calculated with the relative risk of 95% Confidence Interval (CI). Quality assessment of the included studies was assessed using the Consolidated Standards of Reporting Trials (CONSORT) 2010 statement. RESULTS: Eleven studies met the predetermined inclusion criteria and were included in this meta-analysis. Amiodarone, dexmedetomidine, and magnesium significantly reduced the incidence of postoperative JET [Amiodarone: risk ratio 0.34; I2= 0%; Z=3.66 (P=0.0002); 95% CI 0.19-0.60. Dexmedetomidine: risk ratio 0.34; I2= 0%; Z=4.77 (P<0.00001); 95% CI 0.21-0.52. Magnesium: risk ratio 0.50; I2= 24%; Z=5.08 (P<0.00001); 95% CI 0.39-0.66]. CONCLUSION: All three drugs have shown promising results in reducing the incidence of JET. Our systematic review found that dexmedetomidine is better in reducing the length of ICU stays as well as mortality. In addition, dexmedetomidine also has the least pronounced side effects among the three. However, it should be noted that this conclusion was derived from studies with small sample sizes. Therefore, dexmedetomidine may be considered as the drug of choice for preventing JET.

Sotalol versus amiodarone for postoperative junctional tachycardia after congenital heart surgery.

BACKGROUND: Junctional ectopic tachycardia (JET) is a common arrhythmia after congenital heart disease surgery. There is variability in the choice of antiarrhythmic therapy, with amiodarone used commonly. Intravenous (IV) sotalol is a newly available agent that may be useful for JET. OBJECTIVE: The purpose of this study was to evaluate the safety and efficacy of IV sotalol for postoperative JET and compare outcomes with IV amiodarone. METHODS: This is a retrospective single-center study of all patients who received IV sotalol or IV amiodarone for postoperative JET at Texas Children's Hospital from December 15, 2015, to December 15, 2020. Data included antiarrhythmic efficacy, hemodynamics, and adverse effects. Successful JET control was defined as a decrease in JET rate to <170 beats/min (or decrease by >20%), or conversion to sinus rhythm, with persistent control over 24 hours without requiring alternative antiarrhythmics or mechanical support. RESULTS: A total of 32 patients (median age 71 days; interquartile range 17-221 days) received IV amiodarone (n = 20 [62%]) or IV sotalol (n = 12 [38%]) for postoperative JET. Amiodarone was successful in treating JET in 75% of cases; sotalol was successful in 83%. The JET rate decreased faster over the first 90 minutes after a sotalol bolus (25 beats/min per hour) than after an amiodarone bolus (8 beats/min per hour) (P < .01); no heart rate difference was seen after 24 hours. Amiodarone infusion was discontinued early because of hypotension/bradycardia in 2 patients; this was not required in any patients receiving sotalol. CONCLUSION: For children with postoperative JET, both IV sotalol and amiodarone are safe and efficacious. IV sotalol may lead to a faster improvement in heart rate.

Uso de la ivrabadina para el tratamiento de la taquicardia ectópica de la unión congénita / Use of ivabradine for the treatment of congenital junctional ectopic tachycardia

Resumen La taquicardia ectópica de la unión en su variante congénita es una taquiarritmia pediátrica poco frecuente, que por su naturaleza incesante y su refractariedad a los agentes farmacológicos tradicio nales lleva asociada una alta morbimortalidad. Se presentan los casos clínicos de dos pacientes pediátricos con diagnóstico de taquicardia ectópica de la unión congénita, que mostraron respuesta inadecuada a las alternativas de tratamiento habituales y que, en consecuencia, desarrollaron miocardiopatía dilatada y disfunción ventricular secundaria a la taquicardia sostenida. En ambos se utilizó ivabradina como alternativa farmacológica innovadora pare el control de ésta con excelente respuesta clínica.

Abstract The congenial form of junctional ectopic tachycardia is a rare variant of pediatric tachyarrhythmia that due to its incessant nature and its refractoriness to the traditionally used antiarrhythmic agents has a high morbimortality The clinical cases of two patients with a diagnosis of congenital junctional ectopic tachycardia with inadequate response to the regular pharmacological options, who developed dilated cardiomyopathy and ventricular dysfunc tion secondary to sustained tachycardia, are presented. In both ivrabadine, a new innovative option was used with excellent clinical response.

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

OBJECTIVES: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children. BACKGROUND: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard. METHODS: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm. RESULTS: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group. CONCLUSIONS: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).

[Use of ivabradine for the treatment of congenital junctional ectopic tachycardia]. / Uso de la ivrabadina para el tratamiento de la taquicardia ectópica de la unión congénita.

The congenial form of junctional ectopic tachycardia is a rare variant of pediatric tachyarrhythmia that due to its incessant nature and its refractoriness to the traditionally used antiarrhythmic agents has a high morbimortality The clinical cases of two patients with a diagnosis of congenital junctional ectopic tachycardia with inadequate response to the regular pharmacological options, who developed dilated cardiomyopathy and ventricular dysfunction secondary to sustained tachycardia, are presented. In both ivrabadine, a new innovative option was used with excellent clinical response.

La taquicardia ectópica de la unión en su variante congénita es una taquiarritmia pediátrica poco frecuente, que por su naturaleza incesante y su refractariedad a los agentes farmacológicos tradicionales lleva asociada una alta morbimortalidad. Se presentan los casos clínicos de dos pacientes pediátricos con diagnóstico de taquicardia ectópica de la unión congénita, que mostraron respuesta inadecuada a las alternativas de tratamiento habituales y que, en consecuencia, desarrollaron miocardiopatía dilatada y disfunción ventricular secundaria a la taquicardia sostenida. En ambos se utilizó ivabradina como alternativa farmacológica innovadora pare el control de ésta con excelente respuesta clínica.

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.

Fetal Persistent junctional reciprocating tachycardia : a diagnostic and a therapeutic challenge.

A mother presented with a fetus at 22±1 weeks of gestation with a sustained supraventricular tachycardia  (SVT) at initially 186 beat per minute (bpm). The fetal M-mode echocardiography showed a 1/1 atrio ventricular ratio (with short atrioventricular (AV) interval and a long ventriculo-atrial (VA) interval, suggesting a Persistent junctional reciprocating tachycardia (PJRT) . Upon  initial present no signs of heart failure or hydrops  were noted and treament was initiated with amiodarone and  digoxin . Fetus heart rate slowed  .Postnatal electrocardiogram  Confirmed  the diagnosis of PJRT New born was put on amiodarone and proparonal). Sinus rhythm was rapidly achieved 9 days later .The patient doing well at  10 months of age with maintain of sinus rhythm. Conclusion: our case report illustrates  a particular  form of  JRT   diagnosed  prenatal PJRT  , characterized  by  a good clinical tolerance, its absence of evolution towards cardiomyopathy  and its rapid and unusual response to antiarrhythmics.

Ivabradine as an Adjunct for Refractory Junctional Ectopic Tachycardia Following Pediatric Cardiac Surgery: A Preliminary Study.

OBJECTIVES: Junctional ectopic tachycardia (JET) is a relatively common narrow complex rhythm typically characterized by atrioventricular dissociation or retrograde atrial conduction in a 1:1 pattern. Junctional ectopic tachycardia can be a life-threatening disorder, causing severe hemodynamic compromise and increased morbidity and mortality. The treatment of refractory JET can be very difficult, even with multimodal therapeutic interventions. The purpose of this study was to assess the role of ivabradine in cases of JET refractory to amiodarone and esmolol. METHODS: A total of 480 congenital heart surgeries were carried out at our center in 2017. Twenty (4.16%) patients had postoperative JET. Among these, five infants, aged 7 to 12 months (median: 8 months), had refractory JET. These patients (three tetralogy of Fallot, one ventricular septal defect, one complete atrioventricular septal defect) were treated with oral ivabradine in the dose range of 0.1 to 0.2 mg/kg/12 h as an adjunct to amiodarone. RESULTS: All five patients achieved rate reduction and eventual conversion to sinus rhythm. Mean duration to achieve heart rate of <140 bpm after initiation of ivabradine therapy was 16.8 hours (±7.2 hours), while mean duration to achieve sinus rhythm was 31.6 hours (±13.6 hours). No patient had any recurrence of JET. No patient exhibited any hemodynamic derangement nor side effects attributable to oral ivabradine. CONCLUSION: Oral ivabradine has the potential to be used as an adjunct to amiodarone in the treatment of JET in infants after surgery for congenital heart disease.

Ivabradine in Post-operative Junctional Ectopic Tachycardia (JET): Breaking New Ground.

Junctional ectopic tachycardia (JET) is the commonest tachyarrhythmia in the early post-operative period in children undergoing open-heart surgery. It frequently leads to hemodynamic instability and needs to be managed aggressively. Amiodarone is the first-line agent along with non-pharmacological interventions. We report our initial experience with the use of Ivabradine in post-operative JET. A retrospective case records review of children with post-operative JET during the period from June 2018 to May 2019 was performed. Eight patients with post-operative JET were treated with Ivabradine during this period. The first patient was initially treated with Amiodarone. All eight patients responded to Ivabradine. The initial response was rate control permitting overdrive pacing. One patient had recurrence of JET 10 h after Ivabradine and after return to sinus rhythm. Amiodarone was administered along with the second dose of Ivabradine resulting in remission to sinus rhythm. Ivabradine appears to be an effective alternative to Amiodarone in children with post-operative JET based on our initial clinical experience.

Ivabradine is an effective antiarrhythmic therapy for congenital junctional ectopic tachycardia-induced cardiomyopathy during infancy: Case studies.

Junctional ectopic tachycardia (JET) is a rare form of arrhythmia that is most commonly seen during infancy. JET is continuous and incessant, characterized by persistently high heart rates that may result in impaired cardiac function and tachycardia-induced cardiomyopathy. Despite the availability of multiple antiarrhythmic treatments, including flecainide and amiodarone, management of JET is generally very difficult. Catheter ablation has a high risk of atrioventricular block and it may require the placement of a pacemaker. Ivabradine, also known as a cardiac pacemaker cell inhibitor, is a new-generation antiarrhythmic used to treat sinus tachycardia and angina pectoris in adult patients. In this article, we present three cases of subjects with infantile congenital JET who were admitted to our clinic with a tachycardia-induced cardiomyopathy. The age of the subjects ranged from 52 days to 10 months. Although the cases of tachycardia could not be controlled by multiple antiarrhythmics, including a combination of amiodarone and flecainide combined with either propranolol or digoxin, they were rapidly converted into sinus rhythm with an ivabradine treatment of 0.1-0.2 mg/kg/day. No cardiac or other side effects were observed during ivabradine treatment, and left ventricular functions and rhythms improved within 24 hours. These three cases therefore provide hope that ivabradine may be a suitable standard initial treatment for congenital JET. However, additional research is needed to confirm the validity of these results in other circumstances.

Landiolol Hydrochloride Rapidly Controls Junctional Ectopic Tachycardia After Pediatric Heart Surgery.

OBJECTIVES: Junctional ectopic tachycardia is a supraventricular tachyarrhythmia with atrioventricular dissociation that causes life-threatening postsurgical conditions in pediatric heart patients. This study evaluates the efficacy of landiolol hydrochloride for managing junctional ectopic tachycardia. DESIGN: A single-center retrospective study. SETTING: PICU at the university hospital. PATIENTS: Of 561 pediatric patients who underwent open-heart surgery between 2006 and 2017, 10 patients developed sustained junctional ectopic tachycardia and were selected for landiolol treatment. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Landiolol decreased mean heart rate significantly from 206.1 ± 14.5 to 158.0 ± 8.6 beats/min within 2 hours after administration (p < 0.01). Mean time to achieve 20% heart rate reduction was 2.1 ± 0.5 hours. Systolic blood pressure between pre and post landiolol administration did not change significantly (72.6 ± 5.9 to 79.7 ± 6.2 mm Hg). Once junctional heart rate was sufficiently suppressed, atrioventricular sequential pacing was introduced to stabilize hemodynamics. Nine of 10 cases (90%) had atrioventricular sequential pacing to maintain appropriate heart rate and restore atrioventricular synchronicity under suppressed junctional heart rate. Subsequently, eight of 10 cases (80%) were converted to regular sinus rhythm within 24 hours after starting landiolol administration. The average time to achieve sinus rhythm conversion was 7.9 ± 3.4 hours. CONCLUSIONS: Landiolol rapidly suppresses junctional heart rate in junctional ectopic tachycardia after pediatric heart surgery without significant blood pressure compromises. Subsequent atrioventricular sequential pacing was effective at restoring atrioventricular synchronicity and stabilizing hemodynamics. Combining junctional rate control with landiolol and atrioventricular sequential pacing is therefore suggested as a promising option for prompt management of postoperative junctional ectopic 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.

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.

Adjunctive ivabradine in combination with amiodarone: A novel therapy for pediatric congenital junctional ectopic tachycardia.

BACKGROUND: Treatment of congenital junctional ectopic tachycardia (JET) is often challenging. In the majority of patients affected, a combination of ≥2 antiarrhythmic drugs is required for JET control. OBJECTIVE: The purpose of this study was to assess the efficacy and safety of adjunctive ivabradine therapy for pediatric congenital JET. METHODS: Since January 2015, 5 consecutive patients aged 10 days to 3.5 years (median 8 weeks) were treated with adjunctive ivabradine for congenital JET. All patients had previously undergone antiarrhythmic therapy with unsatisfactory control of JET. Ivabradine was administered orally at an initial dosage of 0.05-0.1 mg/kg/d divided into 2 single doses and was increased up to 0.28 mg/kg/d if necessary. RESULTS: In all 5 patients, ivabradine proved to be successful in controlling JET. Complete suppression of JET and conversion into sinus rhythm were achieved in 4 of 5 patients. The remaining patient had effective heart rate control with persistent slow JET. Mean heart rate was reduced by 31% compared to pre-ivabradine (P = .03) as assessed by 24-hour Holter monitoring. Echocardiography revealed improvement of left ventricular function in all 3 patients with previously impaired left ventricular function. No significant side effects of ivabradine were encountered during median follow-up of 135 days (range 37-203 days). CONCLUSION: In our group of patients with congenital JET, adjunctive treatment with ivabradine resulted in effective and safe rhythm/heart rate control and therefore may be recommended early in the course of this rare inborn tachyarrhythmia.