Permanent junctional reciprocating tachycardia in infants and Children

Abstract

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.

Treatment of Permanent Junctional Reciprocating Tachycardia in a Preterm Neonate: Case Report

“…However, if the tachycardia does not resolve with medical treatment, catheter ablation procedures may be required as an alternative option. 2,4,6,13,14 In this specific case, although tachycardia was resolved completely on the sixth day of life without further need of the catheter ablation procedure in the patient, he then died due to septic shock. Intravenous adenosine is recommended as the first choice of treatment to terminate hemodynamically stable acute episodes of re-entrant SVT involving AV node.…”

“…2 12 Although earlier studies have stated that using a combination of amiodarone, digoxin, and verapamil is the most effective regimen, currently, a combination of flecainide and amiodarone has been reported to be slightly more successful in the treatment of PJRT and reversing cardiomyopathy. 2 In a study by Kang et al 3 antiarrhythmic medications were used for initial management in 76% of patients, while catheter ablation was used initially in only 10% of patients. They also reported that medications achieved clinical benefit in 64% of patients and complete resolution in 23% of patients, and 72% of all patients underwent catheter ablation procedures with a success rate of 90%.…”

“…Digoxin, amiodarone, flecainide, or β-blockers are the most commonly used drugs in the medical therapy of PJRT; however, rare side effects from using these medications can be observed. [2][3][4]6,7 Term delivery is recommended for fetuses with SVT in the absence of significant clinical compromise to avoid complications of preterm birth. 8 If PJRT is detected in the fetal period, transplacental treatment with digoxin, sotalol, or flecainide should be initiated to prevent heart failure.…”

“…It was noted that electrocardiograms (ECG) show inverted P waves in inferior leads II, III, and augmented vector foot (AVF). 2 If the persistent tachycardia is not resolved, it may lead to congestive heart failure and tachycardia-induced cardiomyopathy. 3 4 Tachycardia may infrequently occur in the fetal period that may lead to heart failure and hydrops fetalis.…”

Taquiarritmias supraventriculares neonatales, aproximación y tratamiento desde la fisiopatología

“…De manera característica es incesante y las características electrocardiográficas son las de una taquicardia con complejos QRS estrechos, regulares, con un intervalo RP muy largo y uno PR normal, pero con un eje de la onda P anormal, a diferencia del sinusal. Por lo general es difícil de tratar con los fármacos iniciales; las bajas frecuencias cardíacas de la taquicardia rara vez producen síntomas, pero su carácter incesante puede desencadenar miocardiopatía arrítmica 46 .…”