La tunisie Medicale - 2019 ; Vol 97 ( n°03 ) : 500-503
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Summary


 Summary :
 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

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Article

Introduction
Persistent junctional reciprocating tachycardia (PJRT) is a rare variant of persistent reentry tachycardia leading to dilated cardiomyopathy and requires agressive therapeutic management. Typically the tachycardia is incessant. It is an orthodromic reciprocating tachycardia that uses a slow-conducting accessory pathway commonly located in the right posterior septum near the ostium of the coronary sinus (1) .Although some reports exist(2) on fetuses diagnosed with supraventricular tachycardia that proved to be PJRT postnatally, little is known about the clinical and outcome characteristics of prenatally diagnosed PJRT.
Case report
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 (Figure1) with short atrioventricular (AV) interval and a long ventriculo-atrial (VA) interval, suggesting a slow conducting accessory pathway (figure1) .
Upon initial presentation no signs of heart failure were noted and treament was initiated with amiodarone with the doses of 600 mg/day during 5 days. This resulted in only a minimal decrease in heart rate to 180 bpm.  Digoxin was added at 0,125 mg twice a day to 400mg of amiodarone during 5 days. Fetus heart rate slowed at 175 bpm. A regularly follow up every 2 weeks was required during treatment period.  Fœtal echocardiography during follow up have never demonstrate signs of heart failure ,hydrops or valvular regurgitation.The fetus was delivered by caesarean section ( for obstetrical cause) at a gestational age of 37weeks. A healthy boy of 3340 gram was born with Apgar scores of 9/9 without clinical signs of heart failure, and he was transferred to our department for management. Postnatal electrocardiogram showed a tachycardia with heart rate at 190 bpm, inverted Pwaves in the inferior leads and a P-R interval shorter than the R-P interval confirming the diagnosis of PJRT (figure 2 ). Echocardiography did not reveal any abnormalities.
Electrocardiogram record confirmed the incessant character of this arrhythmia that begun after a sinus tachycardia, and which was characterized by alternance of episodes of fast tachycardia and of short periods of remission (Figure3 A)
New born was put on  amiodarone (500mg/m²) once a day and proparonal 3 mg/kg (twice a day ). Sinus rhythm at 144 bpm was rapidly achieved 9 days later
Patient was put on amiodarone (250 mg/m²) daily and proponolol 3 mg/kg/day in order to maintain sinus rhythm.
Evolution was favorable , with absence of signs or symptoms of recurring tachycardia, and rythm monitoring showed absence of supraventricular tachychardia or accessory path-way, the main heart rate over 24h in electrocardiogram record was 120 bpm (figure 3B) and echocardiography was normal. The patient doing well at 10 months of age with maintain of sinus rhythm.
Discussion
PJRT is a rare form of re-entrant SVT characterized by an often incessant tachycardia at a rate of 120-250 beats per minute with inverted P waves in the inferior leads with an accessory retrograde pathway with slow conduction. This leads to a characteristic long RP interval exceeding the PR interval. There is a typical 1:1 conduction (3). The literature reports that the tachycardia can be diagnosed in adults, children and usually can be recognized in early childhood (3,4), but PJRT diagnosed antenatally have been described in few reports(2,5) and also reported in our case.
M-mode echocardiography is the most commonly used method of diagnosis in fetal arrhythmias through measuring the atrioventricular (AV) and ventriculoatrial  (VA) interval dividing these intervals results in a VA/AV ratio, which is a measure of the conduction properties through eventual retrograde accessory pathways (5).
Fetal echocardiography in our case showed a short Atrioventricular (AV) interval and a long ventriculo-atrial (VA) interval, that could be found in atrial ectopic tachycardia, but the incessant nature and the recurrence of the tachycardia after a few sinus beats relieve the confusion (3).
PJRT is commonly incessant from birth or infancy (3). Its persistence over a long period of time may lead to a tachycardia-induced cardiomyopathy (6) which has described with all types of SVT including PJRT, but is mostly associated with a fetal heart rate above 220 bpm.
Althougth tachycardia was persistant in fetal period and post natal period, we don’t objectived in our case any signs of  cardiomyopathy this can be explained by the relative slow initial rhythm rate comparing with what’s reported ( 2 ) and because of the effect of antiarrhythmic drugs on heart rate. To prevent cardiomyopathy from chronic tachycardia, a treatment of PJRT should be initiated in fœtus. Transplacental therapy can be effective, but uncertainty remains regarding the best médication for rhythm conversion (7). Digoxin is often used as first‐line therapy due to its low maternal risk profile and experience of use.(7). However, more recent publications have questioned the efficacy of transplacental digoxin, particularly in fetuses with hydrops fetalis, and suggest that flecainide or sotalol may be better first‐line options (8). In our case, since there were no signs of hydrops ,we did not prescribe flecainide and we used association of amiodarone and digoxin .
Altough posnatal diagnosis is easy, treatment is difficult in neonatate and its objectif is to slow the rhythm since the effective therapy was radiofrequency ablation which was not feasable in fetus and in new born (  9 ).
EHRA and AEPC-Arrhythmia Working Group (10) conclude that combination of antiarrythmic drugs Ic or III with beta-blokers and digoxine had shown the most effective treatment. Our attitude was similar to what recommended (10) since we had used amiodarone and proponalol.
One of the main finding in our case is the relatively rapidity resolution of PJRT. In contrast to what has been observed in smaller series of pediatrics patients reported so far, spontaneous resolution of PJRT was not so uncommon (10) and such an evolution happened sometimes many years after diagnosis of tachycardia  but in our case a sinus rhythm was obtained rapidly after 9 days of birth.
Another interesting finding of our report is the remarkable efficiency of antiarrhythmic drugs, In fact sinus rythm was obtained rapidly and maintained by using medical bitherapy alone up to 10 moths .Usual efficiency of these drugs allowed delay of radiofrequency ablation until patients have reached an adequate growth, wheras many reports (9) described PJRT us a arrhythmia considered to be usually refractory to drug treatment (3,4) and requiring the use radiofrequency ablation which is a technique that should be reserved for older patients because complications when realized in infancy are more frequent (9 )

Conclusion:
Our case report illustrates the fact that PJRT is a diagnostic and a therapeutic challenge for physicians.  The characteristics of our prenatal PJRT cases included its good clinical tolerance, its absence of evolution towards cardiomyopathy despite its incessant and permanent character and its rapid and unusual response to antiarrhythmics  postnatally, allowed us to delay radiofrequency ablation at a higher age.

Conflict of interest : No conflict of interest


Figure 1: The fetal M-mode echocardiography showed a 1/1 atrio ventricular ratio with short atrioventricular (AV) interval and a long ventriculo-atrial (VA)

Figure 2: Postnatal electrocardiogram showed a tachycardia with heart rate at 190 bpm, inverted P waves in the inferior leads and a P-R interval shorter than the R-P interval confirming the diagnosis of PJRT

Figure 3: Electrocardiogram record confirmed the incessant character of this arrhythmia that begun after a sinus tachycardia, and which was characterized by alternation of episodes of fast tachycardia and of short periods of remission

Figure 4: Rhythm monitoring showed absence of supraventricular tachychardia or accessory path-way

References
1.    Scaglione M, Caponi D,  Riccardi R, et al. Accessory pathway potential recording in a case of permanent junctional reciprocating tachycardia with decremental conduction localized on the atrial site. Ital Heart J 2001; 2:147–151.
2.    Simpson JM. Fetal arrhythmias. Ultrasound Obstet  Gynecol .2006;27: 599 – 606.
3.    Vaksmann G, ’Hoinne C, Lucet V et al. Permanent junctional reciprocating tachycardia in children: a multicentrestudy on clinical profile and outcome. Heart 2006; 92:101 – 104
4.    Kang KT, Potts JE, Radbill AE, et al. Permanent junctional reciprocating tachycardia in children: A multicenter experience. Heart Rhythm 2014;11:1426-32.
5.    Jaeggi ET,  Nii M.  Fetal brady and  tachyarrhythmias: new and accepted diagnostic and treatment methods. Semin Fetal Neonatal Med 2005;10 :504 – 514.
6.    Cornette J, Ten Harkel A, Steegers P . Fetal dilated cardiomyopathy caused by persistent junctional reciprocating tachycardia J. Ultrasound Obstet Gynecol .2009; 33: 595–598
7.    Hill GD, Kovach JR, Saudek DE et al. Transplacental treatment of fetal tachycardia:  A systematic review and meta-analysis. Prenat Diagn. 2017 Nov; 37(11):1076-1083.
8.    Ekiz A, Kaya B, Bornaun H, et al. Flecainide as first line treatment for fetal supraventricular tachycardia. J Matern Fetal Neonatal Med 2017;14:1
9.    Femenia, F., Sarquella-Brugada, G, Brugada J et al. Single-catheter radiofrequency ablation of a permanent junctional reciprocating tachycardia in a premature neonate.  Cardiol Young. 2011
10.    Brugada J, Blom N, Georgia Sarquella-B. Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement. Europace 2013; 15, 1337–82.

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