Author's Accepted Manuscript

Permanent junctional reciprocating tachycardia in

MD, Christina Y. Miyake MD, MS, Andrew D.

PII: S1547-5271(14)00461-5
DOI: http://dx.doi.org/10.1016/j.hrthm.2014.04.033
Reference: HRTHM5754

To appear in: Heart Rhythm

Cite this article as: Kristopher T. Kang MD, James E. Potts PhD, Andrew E. Radbill MD,
Martin J. La Page MD, FHRS, John Papagiannis MD, FHRS, Jason M. Garnreiter MD,
Petr Kubus MD, Michal J. Kantoch MD, FRCP, Nicholas H. Von Bergen MD, Anne
Fournier MD, Jean-Marc Côté MD, Thomas Paul MD, Charles C. Anderson MD, Bryan
C. Cannon MD, Christina Y. Miyake MD, MS, Andrew D. Blaufox MD, FHRS, Susan
P. Etheridge MD, FHRS, Shubhayan Sanatani MD, FHRS, Permanent junctional
reciprocating tachycardia in children: A multi-center experience, Heart Rhythm, http:
//dx.doi.org/10.1016/j.hrthm.2014.04.033

This is a PDF file of an unedited manuscript that has been accepted for publication. As a
service to our customers we are providing this early version of the manuscript. The
manuscript will undergo copyediting, typesetting, and review of the resulting galley proof
before it is published in its final citable form. Please note that during the production process
errors may be discovered which could affect the content, and all legal disclaimers that apply
to the journal pertain.
Permanent junctional reciprocating tachycardia in children: A multi-center experience

Short Title: Permanent junctional reciprocating tachycardia

Kristopher T. Kang, MD*, James E. Potts, PhD*, Andrew E. Radbill, MD†, Martin J. La Page,

MD, FHRS‡, John Papagiannis, MD, FHRS‡‡, Jason M. Garnreiter, MD§, Petr Kubus, MD|,

Michal J. Kantoch, MD, FRCP¶, Nicholas H. Von Bergen, MD#, Anne Fournier, MD**, Jean-

Marc Côté, MD††, Thomas Paul, MD§§, Charles C. Anderson, MD||, Bryan C. Cannon, MD¶¶,

Christina Y. Miyake, MD, MS##, Andrew D. Blaufox, MD, FHRS***, Susan P. Etheridge, MD,

FHRS§, and Shubhayan Sanatani, MD, FHRS*

Affiliations: From the Departments of Pediatrics, Divisions of Cardiology, *British Columbia

Children's Hospital, Vancouver, BC, Canada, †Vanderbilt University Medical Center, Nashville,

TN, ‡University of Michigan, Ann Arbor, MI, ‡‡Mitera Children’s Hospital, Athens, Greece,

§Primary Children’s Hospital, Salt Lake City, UT, |Children’s Heart Centre, University Hospital

Motol, Prague, Czech Republic, ¶Stollery Children's Hospital, Edmonton, AB, Canada, #

University of Iowa Children's Hospital, Iowa City, IA, **CHU Mère-Enfant Sainte-Justine,

University of Montreal, Montreal, QC, Canada, ††Centre Mère-Enfant, CHUQ, Quebec, QC,

Canada, §§University Children's Hospital, Georg-August University, Göttingen, Germany,

||Providence Sacred Heart Children's Hospital, Spokane, WA, ¶¶Mayo Clinic, Rochester, MN,

##Lucile Packard Children’s Hospital, Palo Alto, CA, ***Steven and Alexandra Cohen

Children's Medical Center of New York, Lake Success, NY.

1
Funding Sources: This work was funded by a grant from the Rare Disease Foundation,

Vancouver, Canada. Dr. Kubus was supported by MH CZ – DRO, University Hospital Motol,

Prague, Czech Republic.

Conflicts of interest: There are no potential conflicts of interest.

Address for correspondence:

Shubhayan Sanatani, MD

British Columbia Children’s Hospital

4480 Oak Street, Vancouver, BC

V6H 3V4

Phone: 604-875-3619 Fax: 604-875-3463

E-Mail: [email protected]

Word count: 4,384

ABSTRACT

Background: Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of

supraventricular tachycardia in children. Treatment of this arrhythmia has been considered

difficult due to a high medication failure rate and risk of cardiomyopathy. Outcomes in the

current era of interventional treatment with catheter ablation have not been published.

Objective: To describe the presentation and clinical course of PJRT in children.

2
Methods: This is a retrospective review of 194 pediatric patients with PJRT managed at 11

institutions between January 2000 and December 2010.

Results: Median age at diagnosis was 3.2 months, including 110 infants (57%) (age <1 year).

PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant

compared to non-incessant 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 patients.

Conclusion: 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.

Key words: permanent junctional reciprocating tachycardia; antiarrhythmic agents; catheter

ablation; pediatrics

ABBREVIATIONS

AV = atrioventricular

3
ECG = electrocardiogram

EF = ejection fraction

IQR = interquartile range

PJRT = permanent junctional reciprocating tachycardia

RFA = radiofrequency catheter ablation

SF = shortening fraction

SVT = supraventricular tachycardia

TIC = tachycardia-induced cardiomyopathy

INTRODUCTION

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of supraventricular

tachycardia (SVT) that occurs predominantly in infants and children.1 The arrhythmia substrate

is an accessory pathway with slow, decremental retrograde conduction 2 that is commonly

located in the posteroseptal region of the atrioventricular (AV) junction.3 In the majority of

affected patients, PJRT is incessant and may lead to tachycardia-induced cardiomyopathy (TIC),

which is reversible with a sustained period of rate or rhythm control.

There are several studies on the course and treatment of PJRT in children,4-9 but they do not

reflect management and outcomes in the current era. In previous case series, PJRT was

controlled with antiarrhythmic medications in up to 25% of pediatric patients, and spontaneous

resolution was uncommon.5, 8, 9 Disappointing outcomes from medical management have resulted

in the use of catheter ablation as primary therapy and for PJRT refractory to antiarrhythmic

medications.4-8 Pediatric catheter ablation has evolved rapidly over the past decade and modern

technologies such as cryoablation and electroanatomic mapping have improved outcomes and

4
decreased the risk of adverse events, the latter primarily reducing the need for fluoroscopy use

during the procedure.10

The objective of this study is to describe the clinical course and outcomes of treatment of PJRT

in children in the modern era to reflect these therapeutic advances in catheter ablation

technology.

METHODS

This is a retrospective cohort study of PJRT patients from 11 pediatric centers. Participating

centers were solicited through the Pediatric and Congenital Electrophysiology Society. Local

ethics approval was obtained at each center.

Inclusion criteria: Patients were included if their diagnosis occurred from age 0 to 18 years with

at least one follow-up visit between January 2000 and December 2011. The PJRT diagnosis was

based on electrocardiographic (ECG) criteria previously described:2 (1) narrow QRS tachycardia,

(2) negative P waves in inferior leads, (3) PR to RP ratio <1, (4) 1:1 AV relation during

tachycardia with no evidence of functional AV block, and (5) absent delta waves in the PR

segment during sinus rhythm. Other evidence to support the diagnosis included termination with

vagal maneuvers, adenosine or cardioversion; spontaneous termination with an atrial event; and

absence of a warm-up or cool-down.11 For patients who underwent electrophysiology testing,

confirmation of the diagnosis of PJRT and identification of an accessory pathway were

required.12

Data Collection: Data was collected from existing medical records using a standardized data

5
collection form. Study data were collected and managed using REDCap electronic data capture

tools hosted by the Child and Family Research Institute at British Columbia Children's

Hospital.13

Definitions. PJRT was considered incessant if present for >90% of monitored time by 24-hour

Holter or telemetry, sustained if present 50-90%, episodic if present 10-50%, and sporadic if

present <10% of the time. Cardiomyopathy was defined by a left ventricular ejection fraction

(EF) <40% or shortening fraction (SF) <28%. Rhythm control was defined as normal sinus

rhythm on 24-hour Holter; rate control was defined as an age-appropriate normal heart rate and

variability on 24-hour Holter. Acute success with catheter ablation was defined as normal sinus

rhythm and noninducibility of the PJRT at the completion of the procedure. Recurrence was

defined as documented PJRT following successful catheter ablation. Resolution was defined as

normal sinus rhythm on 24-hour Holter with no recurrence of symptoms after discontinuing

antiarrhythmic therapy for at least one month. Spontaneous resolution was defined as resolution

not requiring ongoing antiarrhythmic medications or ablation therapy. Clinical benefit was

defined as any improvement in PJRT arrhythmia burden, including rate control, less incessant

PJRT, or complete suppression. Determination of these was left to the discretion of the attending

pediatric electrophysiologist.

Statistical Analysis. Frequency tables were generated for all categorical data. Differences

between groups were analyzed using either a chi-square or Fisher's exact test. Continuous data

were analyzed using a univariate procedure. Data are presented as median (interquartile range,

IQR). Differences between the infant (<1 year at the time of diagnosis) and older patient (1

6
year) cohorts and those diagnosed with PJRT before and after 2006 were analyzed using a

Wilcoxon Rank Sum Test. All tests were 2-sided and a p-value <0.05 was considered

statistically significant. All statistical analyses were completed using SAS Statistical Software

Version 9.3 (SAS Institute; Cary, NC).

RESULTS

Clinical presentation and PJRT at diagnosis. There were 194 patients (98 male) included in

the study. The median age at diagnosis was 3.2 (IQR, 0.1 to 116.1) months. PJRT cases were

observed in a unimodal distribution peaking in infancy (Figure 1). There were 110 patients

(57%) diagnosed with PJRT in infancy (age <1 year) including 53 patients (27%) presenting

with fetal tachycardia. PJRT presented with symptoms in 134 of 194 patients (69%), while in

the remaining 31% of patients it was an incidental finding on physical examination or ECG.

The most common presenting symptoms were palpitations in 44 of 194 patients (23%) and

symptoms of heart failure in 36 of 194 patients (19%). Fetal tachycardia was present with and

without hydrops in 7% and 21% of patients, respectively. Most patients (67%) were

hospitalized at the time of diagnosis.

Based on ECG data, the median cycle length of PJRT at diagnosis was 300 ms (IQR, 268 to

375 ms) with an RP interval of 210 ms (IQR, 176 to 250 ms). Like other incessant forms of

SVT, PJRT is defined, in part, as a form of tachycardia with a long RP interval, meaning the

time in ms from the R to the P wave onset of the following beat exceeds the PR interval. PJRT

was incessant in 83 of 177 patients (47%). The median PJRT cycle length was similar in

incessant compared to non-incessant cases (303 vs. 306 ms, p=0.22). The ratio of the RP

7
interval to the cycle length was higher for incessant compared to non-incessant cases (0.71 vs.

0.63, p=0.002); thus, incessant PJRT had a proportionally longer RP interval. Patients with

incessant PJRT were younger at diagnosis (1.0 vs. 16.0 years, p=0.005) and were more likely

to have TIC (28 vs. 12%, p=0.007) compared to patients with non-incessant PJRT. Infants

were more likely to have incessant PJRT than patients in the older group (55 vs. 36%, p=0.01).

An echocardiogram was performed at the time of diagnosis in 178 of 194 patients (92%). The

SF and EF were similar in the infant cohort compared to the older cohort, at the time of

diagnosis and at last follow-up (Figure 2). The electrophysiologic and echocardiographic data

are presented in Table 1.

Tachycardia-induced cardiomyopathy. TIC was observed in 34 of 194 patients (18%),

including 20 infants. Medical therapy for TIC was used in 28 of 34 patients (82%), including

diuretics in 14 patients, intravenous inotropes in 11 patients, angiotensin converting enzyme

inhibitors in 9 patients, digoxin in 6 patients, and anticoagulants in 1 patient. One patient (2.0

months, female) with incessant PJRT at a rate of 170 beats per minute presented with

symptoms of heart failure and an EF of 12% and required extracorporeal life support. TIC

resolved in all patients by the end of the study period, including 6 of 34 patients (18%) who

did not require catheter ablation. The time from documented TIC to normal heart function by

echocardiogram was 2.8 months (IQR, 0.7 to 9.2 months) and was similar in infants and the

older cohort (3.9 vs. 1.1 months, p=0.74).

Initial management. Antiarrhythmic medications were used for initial therapy in 148 of 194

patients (76%). Catheter ablation was used as primary therapy in 19 of 194 patients (10%) and

8
21 of 194 patients (11%) had monitoring only with neither medical therapy nor catheter

ablation. Data concerning initial management were unavailable for 3% of patients. Patients

who initially received antiarrhythmic medications were younger than patients who had a

catheter ablation (0.1 vs. 13.7 years, p<0.0001) and had a shorter PJRT cycle length (300 vs.

400 ms, p<0.0001). There was no difference in the prevalence of incessant PJRT or TIC

among patients receiving initial therapy with medication versus ablation. Overall, initial

medical therapy was used for 2.3 (IQR, 0.1 to 17.9) months. When grouped by age, the

duration of initial medical therapy was shorter in infants compared with the older cohort (0.6

vs. 8.1 months, p=0.02). There was no difference in the initial length of therapy in patients

treated medically or with catheter ablation (0.2 vs. 3.0 months, p=0.07).

There were 21 patients who had monitoring only as the initial management strategy. The age at

presentation in this group was 11.3 (IQR, 4.6 to 14.2) years, including 3 patients who presented

as infants and 5 who presented with incessant PJRT. The median PJRT rate was 154 (IQR, 120 to

200) bpm with EF and SF of 65 (IQR, 58 to 69) % and 33 (IQR, 30 to 35) %, respectively.

Nineteen patients went on to catheter ablation. Family choice was the primary reason given for

abandoning the monitoring only strategy. One patient (16.3 years, male) who presented with

episodic PJRT had a successful catheter ablation procedure after developing cardiac dysfunction

during 14 months of monitoring. Of the two patients who did not have an ablation procedure, one

(8.0 years, male) resolved spontaneously without catheter ablation. The other patient (13.2 years,

male) remained in PJRT at the time of last follow-up and elective catheter ablation was planned.

Neither patient received a trial of medical therapy.

9
Antiarrhythmic medications. Overall, antiarrhythmic medications were used as part of PJRT

management in 158 of 194 patients (82%). The median number of medication regimens per

patient was 2 (IQR, 1 to 5), however, 65/158 patients (41%) received only 1 medication. Beta-

blockers were the most common choice, however, nearly 40 different medication

combinations were used (Figure 3). There was clinical benefit for 64% of all regimens,

including complete suppression of PJRT in 23%. Among medication regimens, a combination

of amiodarone and a beta-blocker achieved clinical benefit in 16 of 16 cases (100%). Used

individually, amiodarone and beta-blockers achieved clinical benefit in 24 of 32 (75%) and 31

of 56 (55%) cases, respectively. Monotherapy with flecainide or propafenone and combination

therapy with digoxin and propafenone were each beneficial in >70% of cases. Clinical

deterioration was observed with 2% of all regimens.

Medication use was more common in infants with 99% receiving medications compared to

60% of older patients (p<0.0001). The median number of medications prescribed to infants

was also greater (2.5 vs. 1.0, p<0.0001). The most common medications used were beta-

blockers (18% vs. 6%, p<0.0001), digoxin (16% vs. 5%, p<0.0001), amiodarone (12% vs. 2%,

p<0.0001), sotalol (9% vs 3%, p=0.0002), propafenone (9% vs. 3%, p<0.0001), and flecainide

(9% vs. 1%, p<0.0001).

Medication side effects requiring cessation of therapy were observed with 4% of all

medication regimens. Serious side effects included QRS prolongation in 4 patients and thyroid

toxicity in 3 patients. One patient had refractory hypotension and bradycardic arrest, and one

each had transient second-degree AV block, respiratory depression, and hepatic toxicity.

10
Catheter ablation. Catheter ablation was performed in 140 of 194 patients (72%), including

108 who had ablation after an initial trial of primary medical therapy. There were 175

procedures in total: 138 with radiofrequency ablation, 27 with cryoablation, and 10 with both.

The median age at the time of first ablation was 7.9 (IQR, 3.7 to 14.3) years. There were 46 of

140 patients (17%) who had ablation when they were younger than 5 years and 10 of 140

patients (7%) who were younger than 1 year. Elective catheter ablation was the most common

indication for catheter ablation, accounting for 45% of ablation cases, and was more common

in patients in the older cohort (60% vs. 25%, p<0.0001). Ablation was performed for PJRT

refractory to medical therapy in 41% of patients and was more common in infants (64% vs.

21%, p<0.0001). Ablation for TIC was performed in 7% of patients. Patients undergoing

radiofrequency catheter ablation (RFA) and cryoablation procedures were of similar age (8.9

vs. 5.1 years, p=0.28). The proportion of patients undergoing RFA (78 vs. 81%, p=0.69) and

cryoablation procedures (16 vs. 14%, p=0.79) were similar in the infants and older patients.

Catheter ablation was successful in 90% of all procedures, including 90% with RFA, 93% with

cryoablation, and 90% for the procedures where both techniques were used. Recurrence of the

arrhythmia occurred in 18 of 134 initial ablation procedures (13%) and 5 of 26 second ablation

procedures (19%). Resolution of PJRT without catheter ablation was observed in 12% of

patients.

Three-dimensional electroanatomic mapping was used in 55 of 172 procedures (32%) and was

associated with similar rates of acute success in 91 vs. 89% of cases performed without

11
advanced mapping strategies. The locations of the accessory pathways targeted for catheter

ablation are shown in (Figure 4). Location of the accessory pathways in the right posteroseptal

region or at the coronary sinus were associated with a higher rate of acute success regardless

of ablation modality (96 vs. 81%, p=0.002). There was significantly less fluoroscopy

exposure in the recent era (after 2006) compared to the earlier era (up to and including 2006)

(11 vs. 18 minutes, p=0.002). Procedure times have remained similar between eras (168.5 vs.

165 minutes, p=0.93).

Ultimately, catheter ablation was performed in 63 of 110 of those presenting as infants (58%)

at a median age of 47 (27 to 77) months. In this cohort, 82 procedures were performed,

including 61 using RFA, 15 using cryoablation and 6 using both techniques. A lower

proportion of infants had an ablation procedure when compared to the older patient cohort

(58% vs. 93%, p<0.0001). Computerized electroanatomic mapping was used in 20% of

ablation procedures in infants. Acute success was achieved in all. The median fluoroscopy and

procedure times were similar in the infant and older patient cohorts.

Complications associated with catheter ablation occurred in 9% of all procedures, including

7% with RFA and 12% with cryoablation. The most common complication was transient third-

degree AV block in 5 cases, including 3 using RFA, 1 using cryoablation, and 1 using both

techniques. Minor complications including transient AV block were noted in 4 of 10 patients

(40%) in the infant cohort and 8 of 130 patients (6.2%) in the older cohort who received

catheter ablation.

12
Outcomes and follow-up. The median duration of follow-up was 3.8 years (0.1 to 23.7 years).

One patient died during the follow-up period, unrelated to the arrhythmia or its therapy. At the

time of last follow-up, 174 of 194 patients (90%) were in sinus rhythm. Seventy-five of 110

infants (68%) and 72 of 84 patients in the older cohort (86%) had complete resolution of PJRT.

Complete resolution was more common for patients in the older cohort (68 vs. 86%, p=0.005).

Spontaneous resolution of PJRT without catheter ablation or medications was observed in 12%

of patients. Twenty-five infants (13%) and 2 patients in the older cohort (2%) had suppression of

their PJRT and were in normal sinus rhythm with ongoing antiarrhythmic therapy. Thirty-three of

194 patients (17%) continued on medication(s).

Three infants (3%) and 4 patients in the older cohort (5%) had ongoing PJRT. Among these

patients, 4 had incessant and 3 had intermittent tachycardia. Two other patients in the older

cohort had ongoing tachycardia other than PJRT. Three patients had catheter ablation, all with

acute success, although 1 patient had recurrent PJRT. Six patients remain on medical therapy; 1

was not receiving treatment but was planned for ablation therapy. Follow-up data was

incomplete for 7 infants and 4 patients in the older cohort.

DISCUSSION

This study, the largest to date, describes the clinical course and outcomes of PJRT detailing

treatment strategies in the current era. Similar to prior studies,5, 8 these data show that the

incidence is highest in infancy, that there is a low likelihood of spontaneous resolution, and

that the risk of TIC is significant. One of the important findings of this study is that, despite

the 90% success rate of catheter ablation and low risk of serious complications, it is the initial

13
treatment for only 10% of patients. Overall, outcomes in infants with PJRT are excellent.

The clinical course of PJRT is not always benign. Many patients undergo multiple trials of

antiarrhythmic medications and ultimately require catheter procedures for tachycardia control.

The rationale for aggressive management is to prevent TIC, an important complication

associated with PJRT. In our study, TIC was observed in 18% of patients, consistent with

previous reports.5, 8 Most patients with TIC had medical therapy to support heart function,

however, catheter ablation for definitive management was effective and safe in this group. The

distinction between reversible ventricular function and a cardiomyopathy has not been made

in the literature, but clearly, in some cases, the extent of dysfunction allows for rapid

reversibility.

TIC was more common among patients with incessant PJRT, which was associated with a

longer RP interval and longer RP:cycle length. This is consistent with slower conduction

through the accessory pathway and a wider excitable gap.14 Several patients with TIC

developed progressively incessant PJRT and reduced heart function after initial treatment,

highlighting the importance of ongoing monitoring even in patients without symptoms at

presentation.

Based on the two largest studies to-date, there is disagreement regarding the best therapeutic

strategy. Typically, the use of medical versus ablation therapy reflects the observed

effectiveness of antiarrhythmic medications and rate of spontaneous resolution. In a series of

85 PJRT patients diagnosed between 1966 and 1998 from 7 centers in France, spontaneous

14
resolution was reported in 22%.8 Medical therapy using regimens that included amiodarone

and verapamil was successful in >80% of cases. Ablation therapy was used in only 18 patients.

In contrast, in a single center study of 21 patients diagnosed between 1989 and 1995 in the

United States, no patient had spontaneous resolution. Medical therapy was ineffective in all

patients except one, and that patient had to subsequently stop medications due to side effects. 5

Ablation was performed in all cases with 5 of 21 patients (24%) experiencing a recurrence

after the first ablation. Repeat ablation was successful in 4 of 5 patients.

The current study confirms that resolution of PJRT in the absence of ablation therapy is

uncommon, occurring in only 12% of cases. Overall, antiarrhythmic medications were

beneficial in approximately two-thirds of patients, with complete suppression in 25% and a

partial effect in an additional 43% of patients. However, most patients ultimately proceeded to

catheter ablation despite any achieved degree of medical control. Medical therapy was

complicated by side effects in 9%. In contrast, catheter ablation using either RFA or

cryoablation was effective in 90% of patients with no major complications. Given this finding,

it is surprising that catheter ablation was the initial treatment attempted in only 10% of cases.

Notably, the majority of patients were diagnosed in infancy when catheter ablation presents

additional risk and medication is the preferred treatment. Medication is often not effective for

complete control of this arrhythmia and catheter ablation should be the primary treatment for

most older patients, especially in the era of cryoablation.

Our research does not provide specific age, weight, or other clinical parameters to guide the

use of catheter therapy, however, we show that ablation can be effective and safe in children as

15
young as 1.2 months even in the setting of TIC. The role of medical therapy is primarily for

young or small children, where trials of antiarrhythmic medications may still be beneficial as a

temporary measure to prevent incessant PJRT and TIC prior to ablation or to allow for the rare

patient with spontaneous resolution.

Our study is limited by the retrospective data collection method, which for completeness

depends on the availability and accuracy of medical records. Not all information was available

for every patient in the series. Where information was unavailable or incomplete, this was

reflected in the analysis and results. As for all case series, this study may also be limited by

selection bias for patients with particular interventions (i.e. ablation therapy) or better

outcomes. Patients who were not managed by a pediatric electrophysiologist, likely those in

whom PJRT was not incessant or associated with cardiac dysfunction, were not captured in

this study. These may represent additional cases in which observation may suffice.

CONCLUSION

This international multicenter review of PJRT provides a comprehensive assessment of

medical treatment options, suggesting that PJRT may be amenable to medical control, but is

unlikely to resolve spontaneously. The success of catheter ablation as a treatment for PJRT

exceeds that provided by medication. Compared to previous studies of catheter ablation for

this arrhythmia, the current study shows the risk of recurrence or major complications is

lower. Catheter ablation for PJRT should be considered more often as the initial treatment for

children and adolescents with PJRT, with medication as the primary therapy for infants and

small children. There may be a role for observation in selected patients who do not have

16
incessant PJRT, ventricular dysfunction, or a long RP interval potentially associated with TIC.

17
REFERENCES

1. Coumel P, Cabrol C, Fabiato A, Gourgon R, Slama R. Tachycardie permanente par

rythme reciproque. Preuves du diagnostic par stimulation auriculaire et ventriculaire.

Arch Mal Coeur Vaiss 1967; 60:1830-1864.

2. Critelli G, Gallagher JJ, Monda V, Coltorti F, Scherillo M, Rossi L. Anatomic and

electrophysiologic substrate of the permanent form of junctional reciprocating

tachycardia. J Am Coll Cardiol 1984; 4:601-610.

3. Ticho BS, Saul JP, Hulse JE, De W, Lulu J, Walsh EP. Variable location of accessory

pathways associated with the permanent form of junctional reciprocating tachycardia and

confirmation with radiofrequency ablation. Am J Cardiol 1992; 70:1559-1564.

4. Aguinaga L, Primo J, Anguera I, Mont L, Valentino M, Brugada P, Brugada J. Long-term

follow-up in patients with the permanent form of junctional reciprocating tachycardia

treated with radiofrequency ablation. Pacing Clin Electrophysiol 1998; 21:2073-2078.

5. Dorostkar PC, Silka MJ, Morady F, Dick M, 2nd. Clinical course of persistent junctional

reciprocating tachycardia. J Am Coll Cardiol 1999; 33:366-375.

6. Gaita F, Montefusco A, Riccardi R, Giustetto C, Grossi S, Caruzzo E, Bianchi F, Vivalda

L, Gabbarini F, Calabro R. Cryoenergy catheter ablation: A new technique for treatment

of permanent junctional reciprocating tachycardia in children. J Cardiovasc

Electrophysiol 2004; 15:263-268.

7. Smith RT, Jr., Gillette PC, Massumi A, McVey P, Garson A, Jr. Transcatheter ablative

techniques for treatment of the permanent form of junctional reciprocating tachycardia in

young patients. J Am Coll Cardiol 1986; 8:385-390.

8. Vaksmann G, D'Hoinne C, Lucet V, Guillaumont S, Lupoglazoff JM, Chantepie A,

18
 Denjoy I, Villain E, Marcon F. Permanent junctional reciprocating tachycardia in

children: A multicentre study on clinical profile and outcome. Heart 2006; 92:101-104.

9. van Stuijvenberg M, Beaufort-Krol GC, Haaksma J, Bink-Boelkens MT. Pharmacological

treatment of young children with permanent junctional reciprocating tachycardia. Cardiol

Young 2003; 13:408-412.

10. Wan G, Shannon KM, Moore JP. Factors associated with fluoroscopy exposure during

pediatric catheter ablation utilizing electroanatomical mapping. J Interv Card

Electrophysiol 2012; 35:235-242.

11. Gillette PC, Garson A, Jr. Electrophysiologic and pharmacologic characteristics of

automatic ectopic atrial tachycardia. Circulation 1977; 56:571-575.

12. Dick M, O'Connor BK, Serwer GA, LeRoy S, Armstrong B. Use of radiofrequency

current to ablate accessory connections in children. Circulation 1991; 84:2318-2324.

13. Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data

capture (redcap)--a metadata-driven methodology and workflow process for providing

translational research informatics support. J Biomed Inform 2009; 42:377-381.

14. Gaztanaga L, Marchlinski FE, Betensky BP. Mechanisms of cardiac arrhythmias. Rev

Esp Cardiol 2012; 65:174-185.

15. Kugler JD, Danford DA, Houston K, Felix G, and Members of the Pediatric EP Society

RCAR. Radiofrequency catheter ablation for paroxysmal supraventricular tachycardia in

children and adolescents without structural heart disease. Am J Cardiol 1997; 80:1438-

1443.

19
FIGURE LEGENDS

Figure 1. Age at diagnosis for permanent junctional reciprocating tachycardia patients.

The median age at diagnosis was 3.2 months.

Figure 2. Echocardiographic shortening and ejection fractions at the time of diagnosis and

at last follow-up. The median values, IQR (error bars) and 5th and 95th percent outliers (x) are

indicated on each plot. EF = ejection fraction; IQR = interquartile range; SF = shortening

fraction.

Figure 3. Common antiarrhythmic medications used for treatment of permanent junctional

reciprocating tachycardia. The proportion of regimens with clinical improvement is marked in

black. Clinical improvement included patients who achieved rate control, less incessant

arrhythmia, or complete suppression. Amio = amiodarone; BB = beta-blocker; Dig = digoxin;

Sot = sotalol; Prop = propafenone; Flec = flecainide.

Figure 4. Location of accessory pathways in permanent junctional reciprocating

tachycardia. Schematic illustrating left anterior oblique view demonstrating the location of

accessory pathways determined during catheter ablation. Adapted from Kugler et al.15 AV Node

= atrioventricular node; LIS = left intermediate septal; LL = left lateral; LP = left posterior; LPS

= left posteroseptal; RAS = right anteroseptal; RIS = right intermediate septal; RP = right

posterior; RPS = right posteroseptal.

20
Table 1

Table 1. Electrophysiologic and echocardiographic data for the infant and older cohorts.

The median (IQR) or the number (%) are reported.

Infant Cohort Older Cohort P

Heart Rate (bpm) 210 (187-242) 164 (135-200) <0.0001

Cycle Length (ms) 285 (248-321) 365 (300-444) <0.0001

RP Interval (ms) 188 (160-220) 240 (200-294) <0.0001

RP Interval/Cycle Length 0.67 (0.58-0.77) 0.70 (0.59-0.77) 0.51

SF at Diagnosis (%) 32 (22-39) 32.5 (28-37) 0.85

EF at Diagnosis (%) 52.5 (38-66) 58 (42-69) 0.53

SF at Follow-Up (%) 37 (35-41) 36 (33-39) 0.07

EF at Follow-Up (%) 66 (62-71) 65 (60-69) 0.10

TIC 20/110 (18%) 14/84 (17%) 0.76

EF = ejection fraction; IQR = interquartile range; SF = shortening fraction; TIC = tachycardia-

induced cardiomyopathy.
Figure 1
Figure 2
Figure 3
Figure 4