Permanent Junctional Reciprocating Tachycardia Research Articles

Permanent Junctional Reciprocating Tachycardia in Infants from Myocarditis-like Presentation to Simple Tachycardia: A Case Series

Permanent Junctional Reciprocating Tachycardia (PJRT) is a rare type of supraventricular tachycardia. It can be misdiagnosed and hence under-treated in infants and children, leading to significant cardiac morbidities and mortalities. We describe four cases of young infants who presented with a wide range of clinical scenarios from neonatal tachycardia to overtly decompensated heart failure, all caused by PJRT. All cases showed complete recovery with appropriate medical treatment. In this case series, we aim to discuss the diversity of clinical presentations, and the challenges surrounding the diagnosis, management, and prognosis. To achieve this aim, we have also performed a detailed literature review and reflected on our cases presented to our center between 2016 to 2020. PJRT is rare; it is often missed or diagnosed late in the presentation. Hence, the key is to keep it in mind in infants presenting with acute cardiac problems from simple tachycardia to a myocarditis-like presentation. Anti-arrhythmic medications are safe and effective as first-line therapy for PJRT in neonates and infants. They can prevent or delay the need for invasive cardiac ablation, which is more challenging in these age groups and should be considered in cases refractory to medical treatment.

An interesting sinus beat initiation of incessant permanent junctional reciprocating tachycardia (PJRT)

We describe a rare surface electrocardiogram (EKG) recorded sinus beat initiation of permanent junctional reciprocating tachycardia in a 40-year old female with structurally normal heart presenting with incessant tachycardia over last 48 hours refractory to intravenous amiodarone infusion. Our case is an interesting illustration of sinus beat initiation of PJRT which has been rarely reported.

Radiofrequency catheter ablation of patients with permanent junctional reciprocating tachycardia and long-term follow-up results.

Permanent junctional reciprocating tachycardia (PJRT) is an infrequent form of atrioventricular re-entrant tachycardia. We report the clinical and electrophysiological properties of PJRT and outcomes of radiofrequency catheter ablation (RCA) in a large group of patients. We included 62 patients with the diagnosis of PJRT. Radiofrequency catheter ablation was performed in all. Location of accessory pathway was right posteroseptal in 37 (59,7%) cases, right midseptal in 3 (4,8%), left posterior in 7 (11,3%), left lateral in 5 (8,1%), left posterolateral in 3 (4,8%), left anterolateral in 2 (3,2%), left posteroseptal in 2 (3,2%), middle cardiac vein in 2 (3,2%), and left coronary cusp in 1 (1,6%). Single procedure success rate was 90.3%. None of patients had recurrence during follow-up after repeat ablations. Overall long-term success rate was 98.4%. Left ventricular systolic function recovered in all patients with tachycardia-induced cardiomyopathy (TIC). Retrograde decremental accessory pathways are mainly located in posteroseptal region. Radiofrequency catheter ablation is a safe and effective approach in patients with PJRT.

Outcomes of sustained fetal tachyarrhythmias after transplacental treatment.

BackgroundFetal tachyarrhythmia is a condition that may lead to cardiac dysfunction, hydrops, and death. Despite a transplacental treatment, failure to obtain or maintain sinus rhythm may occur.ObjectiveWe aimed to analyze the perinatal outcomes of sustained fetal tachyarrhythmias after in utero treatment.MethodsWe performed a retrospective evaluation of 69 cases with sustained fetal tachyarrhythmia. We compared the perinatal and long-term outcomes of prenatally converted and drug-resistant fetuses. Tachyarrhythmia subtypes were also evaluated.ResultsConversion to sinus rhythm was obtained in 74% of cases; 26% of cases were drug-resistant and delivered arrhythmic. Three perinatal deaths occurred in both groups (6.7% vs 17%, P = .34). Neonates delivered arrhythmic were more frequently admitted to neonatal intensive care units (75% vs 31%, P < .01), and their hospital stay was longer (20.9 vs 6.64 days, P < .001). Multiple neonatal recurrences (81% vs 11%, P < .001), temporary hemodynamic dysfunction or heart failure (50% vs 6.7%, P < .001), and postnatal use of a combination treatment (44% vs 13%, P = .028) were also more frequent in this population. Beyond the neonatal period, rates of recurrences within the first 16 months were higher in drug-resistant fetuses (HR = 16.14, CI 95% [4.485; 193.8], P < .001). In this population, postnatal electrocardiogram revealed an overrepresentation of rare mechanisms, especially permanent junctional reciprocating tachycardia (PJRT) (31%).ConclusionPrenatal conversion to stable sinus rhythm is a major determinant of perinatal and long-term outcomes in fetal tachyarrhythmias. The underlying electrophysiological mechanisms have a major role in predicting these differential outcomes with an overrepresentation of PJRT in the drug-resistant population.

Electrophysiologic approach to diagnosis and ablation of patients with permanent junctional reciprocating tachycardia associated with complex anatomy and/or physiology.

Permanent junctional reciprocating tachycardia (PJRT) is a rare supraventricular tachycardia (SVT), typically involving a single decremental posteroseptal accessory pathway (AP). Four patients with long RP SVT underwent electrophysiology (EP) study and ablation. The cases were reviewed. Case 1 recurred despite 3 prior ablations at the site of earliest retrograde atrial activation during orthodromic reciprocating tachycardia (ORT). Mapping during a repeat EP study demonstrated a prepotential in the coronary sinus (CS). Ablation over the earliest atrial activation in the CS resulted in dissociation of the potential from the atrium during sinus rhythm. The potential was traced back to the CS os and ablated. Case 2 underwent successful ablation at 6 o'clock on the mitral annulus (MA). ORT recurred and successful ablation was performed at 1 o'clock on the MA. Case 3 had tachycardia with variation in both V-A and A-H intervals which precluded the use of usual maneuvers so we used simultaneous atrial and ventricular pacing and introduced a premature atrial contraction with a closely coupled premature ventricular contraction. Case 4 had had two prior atrial fibrillation ablations with continued SVT over a decremental atrioventricular bypass tract that was successfully ablated at 5 o'clock on the tricuspid annulus. A second SVT consistent with a concealed nodoventricular pathway was successfully ablated at the right inferior extension of the AV nodal slow pathway. We describe challenging cases of PJRT by virtue of complex anatomy, diagnostic features, and multiple arrhythmia mechanisms.

TACH-ING AWAY: PERMANENT JUNCTIONAL RECIPROCATING TACHYCARDIA

Permanent junctional reciprocating tachycardia (PJRT) is a rare variation of reentrant supraventricular tachycardia that can lead to tachycardia-induced cardiomyopathy if left untreated. A 78-year-old woman presented with symptomatic atrial fibrillation for scheduled cardioversion after failed AV

A RARE CASE OF PERMANENT JUNCTIONAL RECIPROCATING TACHYCARDIA IN AN ADULT WHO PRESENTS IN CARDIOGENIC SHOCK

Permanent junctional reciprocating tachycardia (PJRT) in adults is rare and may often go unidentified. It commonly presents with cardiomyopathy (CM), and management can be difficult. A 31-year-old male with history of methamphetamine use presented with dyspnea. Left ventricular ejection fraction (

Long-term outcome of neonates and infants with permanent junctional reciprocating tachycardia. When cardiac ablation changes natural history

Supraventricular tachycardias (SVT) are the most common arrhythmias in the perinatal period. Permanent junctional reciprocating tachycardia (PJRT) is a rare form of SVT, often incessant and refractory to pharmacological treatments. Our goal was to analyze the clinical features and treatment of PJRT in patients younger than 2 months and to describe their long-term outcomes. MethodsRetrospective descriptive observational study of patients diagnosed between 2000 and 2015 in the NICU of a referral center for the treatment of pediatric arrhythmias. History of pregnancy, neonatal period, pharmacological treatment, electrophysiological study and long-term follow-up were reviewed. Results129 of the 10.198 (1.26%) patients admitted to the NICU had SVT, sixteen of them (12.3%) being diagnosed as PJRT. Ten cases had a prenatal diagnosis. For those six patients postnatally diagnosed, the tachycardia was detected either during a routine check-up or because of acute hemodynamic instability. The majority of patients required combinations of drugs, despite that the tachycardia was poorly controlled. Fifteen patients underwent cardiac ablation, nine patients (60%) in the neonatal period and six during childhood. The procedure was completely effective in all cases. One patient had a transient complete AV block that resolved spontaneously 24 hours after the procedure. No other complications were seen. After a mean follow-up of 10.9 years, no patient has presented recurrence, cardiac dysfunction, signs of ischemia or EKG abnormalities, they all have a normal life. ConclusionsWhen PJRT is refractory to multiple drugs, cardiac ablation should be taken into account at early stages even in very young patients.

Permanent junctional reciprocating tachycardia in infants and Children.

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of supraventricular tachycardia (SVT). It generally presents in infants but can be difficult to diagnose. The characteristic EKG findings, response to Adenosine and persistence or frequent recurrences are helpful in making the diagnosis. It is usually difficult to manage with the initial and single medications used in SVT. Many patients are misdiagnosed and not treated effectively and end up having end stage cardiomyopathy and are diagnosed in patients referred for transplant. Hence all patients referred for a cardiac transplant with dilated cardiomyopathy need to be evaluated for this arrhythmia. If appropriate treatment is started early in the course, the failure can be ameliorated, and the cardiomyopathy can be resolved.

Abstract 13142: Characteristics of Decremental Accessory Pathways in Children

Introduction: While decremental retrograde accessory pathways (DAPs) classically present clinically as permanent junctional reciprocating tachycardia (PJRT), they may be diagnosed unexpectedly during electrophysiology study (EPS). Although DAPs associated with PJRT have been studied, information about other variants of DAPs in children is limited. Methods: We retrospectively studied patients 0 - 21 years of age with retrograde DAPs who underwent EPS between 2005 and 2014. Decrement was defined as rate-dependent prolongation of the local ventriculo-atrial (VA) time by &gt; 30 ms during ventricular extrastimulus or rapid ventricular pacing. Clinical presentation, electrophysiologic properties and ablation outcomes of patients with DAPs were compared to an age-matched cohort of patients with non-decremental accessory pathways. In addition, a sub-analysis examined patients with DAPs depending on whether clinical PJRT was diagnosed prior to the EPS. Results: A total of 29 patients with DAPs and 83 controls were included [mean age at time of EPS 10.3 ± 5.7 years and 10.7 ± 5.2 years, respectively (p = NS)]. Compared to controls, DAPs were more likely to be associated with syncope [6/29 (21%) vs 3/83 (4%), p = 0.01], ventricular dysfunction [6/29 (21%) vs 4/83 (5%), p = 0.04], and treatment with antiarrhythmic medications [20/29 (69%) vs 39/83 (47%) p = 0.04]. In addition, DAPs were more likely to be right-sided [20/29 (69%) vs. 24/83 (29%), p &lt; 0.01] and septal [19/29 (66%) vs 17/83 (21%), p &lt; 0.01]. DAPs and their age-matched controls had similar rates of acute ablation success [26/29 (90%) vs 77/83 (93%), p = NS] and recurrence [2/29 (7%) vs 4/83 (5%), p = NS]. There were no major procedural complications in either group. Of the patients with DAPs, only 12 (41%) had clinical PJRT, and these patients had more syncope [6/12 (50%) vs 0/17 (0%), p &lt; 0.01], slower ORT (mean cycle length 383 ± 89 ms vs 323 ± 60 ms, p = 0.04), and longer maximum VA times (mean 283 ± 116 ms vs 208 ± 42 ms, p = 0.01) compared to those with DAPs without clinical PJRT. Conclusions: The majority of pediatric patients with DAPs do not present with clinical PJRT. DAPs in general are typically associated with more severe symptoms, but ablation outcomes are similar to age-matched controls with non-decremental pathways.

COEXISTENCE of PERMANENT JUNCTIONAL RECIPROCATING TACHYCARDIA with RHEUMATIC VALVULAR DISEASE; A CASE SUCESSFULLY TREATED with RADIOFREQUENCY ABLATION

A seventeen years old female had presented with palpitation. On the electrocardiography (ECG), long RP, narrow QRS tachycardia, inverted P waves in leads D2, D3 and aVF were noticed. On transthoracic echocardiography, rheumatic mitral valvular disease (RMVD) with normal ventricular systolic function was observed. Eccentric mitral regurgitation was also present. Electrophysiology study (EPS) was performed at our institution. According to EPS and surface ECG findings our diagnosis was permanent junctional reciprocating tachycardia (PJRT). Atrial activation appeared earliest and nearly ostium of the coronary sinus. The accessory pathway was detected near the coronary sinus ostium. RF ablation was performed, successfully. Tachycardia was terminated in three seconds. Permanent junctional reciprocating tachycardia can rarely coexist with RMVD. Here, we are reporting a case of PJRT and RMVD coexistence that underwent successful radiofrequency (RF) catheter ablation. This case report confirms that RF catheter ablation should be considered as the treatment of choice in adult patients with PJRT for the following reasons: catheter ablation is highly successful, the complication rate is low, and PJRT may lead to tachycardia induced cardiomyopathy, which is reversible.

Permanent junctional reciprocating tachycardia in children: a multicenter experience.

Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published. To describe the presentation and clinical course of PJRT in children. This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010. The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients. PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.

Permanent junctional reciprocating tachycardia in a dog

A 5-year-old male English Bulldog was presented with a 1-year history of paroxysmal supraventricular tachycardia (SVT) partially responsive to amiodarone. At admission the surface ECG showed sustained runs of a narrow QRS complex tachycardia, with a ventricular cycle length (R–R interval) of 260 ms, alternating with periods of sinus rhythm. Endocardial mapping identified the electrogenic mechanism of the SVT as a circus movement tachycardia with retrograde and decremental conduction along a concealed postero-septal atrioventricular pathway (AP) and anterograde conduction along the atrioventricular node. These characteristics were indicative of a permanent junctional reciprocating tachycardia (PJRT). Radiofrequency catheter ablation of the AP successfully terminated the PJRT, with no recurrence of tachycardia on Holter monitoring at 12 months follow-up.

Pseudo-PJRT in an 8 year-old boy with severe decompensated heart failure and recurrent episodes of ventricular fibrillation

We present a case of 8 year-old boy with several episodes of ventricular fibrillation in the course of tachycardia-mediated cardiomyopathy and severe decompensated heart failure. The cardiomyopathy was caused by incessant long-RP tachycardia that was resistant to pharmacotherapy. Despite initial suspition that the arrhythmia was permanent junctional reciprocating tachycardia (PJRT) electrophysiology study revealed atypical atrioventricular nodal reentrant tachycardia. Due to clinical and electrocardiographical presentation mimicking PJRT such arrhythmia merits the name 'pseudo PJRT'.

Incessant SVT in children: Ectopic atrial tachycardia and permanent junctional reciprocating tachycardia

Ectopic atrial tachycardia (EAT) and permanent junctional reciprocating tachycardia (PJRT) can cause incessant and medically refractory SVT in children, leading to heart failure and shock. In this review, we discuss the diagnosis, medical treatment, and indications for ablation for these two very different but equally difficult to treat arrhythmias.

The infant with supraventricular tachycardia: Diagnosis and management

Ectopic atrial tachycardia (EAT) and permanent junctional reciprocating tachycardia (PJRT) can cause incessant and medically refractory SVT in children, leading to heart failure and shock. In this review, we discuss the diagnosis, medical treatment, and indications for ablation for these two very different but equally difficult to treat arrhythmias.

A Rare Case of Permanent Junctional Reciprocating Tachycardia Ablated on the Roof of the Left Atrium

Left-sided anteroseptal accessory pathways that course through the aortomitral fibrous continuity are some of the rarest types of accessory pathways. At this region the atrium and ventricle are separated by their greatest distance because of the intervening aortic valve. These pathways often have a long circuitous course that may involve the root and cusps of the aortic valve. Prior reports have demonstrated the feasibility of ablating these pathways along the anteroseptal mitral annulus, the left ventricular outflow tract, or in the left or noncoronary cusps of the aortic valve. We describe a case of a concealed decremental anteroseptal accessory pathway that was ablated on the roof of the left atrium remote from the mitral or aortic valve annuli. This report indicates that when an appropriate site for ablation of a left-sided anteroseptal accessory pathway is not found close to a valve annulus, these pathways may be amenable to ablation by targeting their atrial insertion site.

Peculiar Electrocardiographic Feature Accurately Localizing an Unusual Case of Permanent Junctional Reciprocating Tachycardia

Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia due to an accessory pathway with slow retrograde conduction that is mostly localized in the posteroseptal area. The standard ECG shows long RP tachycardia with negative P waves in the inferior leads and positive P waves in leads I and aVL. We report a case of PJRT in a 31-year-old lady with incessant long RP tachycardia displaying negative P waves not only in the inferior leads but also in leads I and aVL. The accessory pathway was localized in the left posteroseptal region and managed successfully with radiofrequency catheter ablation.

Post-partum cardiogenic shock in a patient with permanent junctional re-entry tachycardia

Permanent junctional reciprocating tachycardia (PJRT) is a rareform of supraventricular arrhythmia, most often encountered inchildrenandadolescents,characterizedbyantegradeconductionoverthe atrio-ventricular node and retrograde conduction over a decre-mental accessory pathway [1]. In the majority of the patients theclinical scenario is dominated by frequent episodes of palpitations,often triggered by exercise. In about 20–30% of cases PJRT maybecome incessant and lead to advanced left ventricular systolic dys-function, that is generally reversible after restoration of stable sinusrhythm [1–3]. However, cardiogenic shock caused by PJRT has neverbeen reported.We describe the case of a young woman with a history of PJRTdating from late childhood; a totally benign clinical scenario character-ized by periodic recurrences of well-tolerated arrhythmias rapidlyturnedintoadramaticadvancedheartfailuresettingsoonafterpartum.This case underlines the importance of an accurate evaluation andmanagement of cardiac arrhythmias during pregnancy.A young woman was chronically treated with atenolol (50 mg) tomanage recurrences of PJRT episodes. On treatment she suffered rareand self-limiting, mildly symptomatic episodes. At the age of 32 shebecame pregnant and beta-blocker therapy was withdrawn to avoidgrowth restriction to the fetus. A 12-lead ECG performed in the firstweeks of pregnancy showed normal sinus rhythm, and an echocar-

Persistent Long R–P Tachycardia

A case of persistent, recalcitrant long R-P tachycardia with decremental retrograde conduction consistent with permanent junctional reciprocating tachycardia (PJRT) is presented, refractory to multiple prior attempts at catheter ablation and antiarrhythmic drug therapy trials. During repeat study at their center, the authors demonstrated the conduction properties and oblique course of the accessory pathway (AP) within the coronary sinus (CS) during PJRT. The authors describe their successful approach to catheter ablation, targeting first the AP-atrium interface at a site distal to the AP recording within the CS, then the AP potential itself at the os. The current case highlights the complexity of the AV connection and the importance of careful mapping of the CS in patients with PJRT.