1023: Permanent Junctional Reciprocating Tachycardia (Pjrt): A Rare Case of Refractory Neonatal SVT

Introduction: Supraventricular Tachycardia (SVT) can be dangerous especially in neonates. Permanent junctional reciprocating tachycardia (PJRT) is a subvariant of atrioventricular re-entry tachycardia. It is estimated to make up 1% of SVTs in pediatrics. PJRT poses significant diagnostic and therapeutic challenges as it is resistant to typical pharmacological treatments. Description: A 13-day-old previously healthy male was admitted from the emergency department with a one-week history of tachycardia concerning for SVTs. On admission, the patient was tachycardic (230 beats per minute) but otherwise vitally stable. Echocardiography (EKG) showed narrow complex tachycardia with a long RP interval. Electrolyte and blood counts were normal. Two doses of adenosine were given without leading to conversion. This raised concerns of refractory SVT. Synchronized cardioversion was done and failed. Amiodarone bolus followed by infusion was initiated but failed to convert. Echocardiogram showed 49% of cardiac function. Due to amiodarone failure, a third dose of adenosine was given but failed. Following this setback, the patient was transferred to a tertiary care center for advanced electrophysiological evaluation and management. There, he underwent six attempts of transcutaneous pacing and was started on a combination of esmolol, flecainide, and amiodarone. Once sinus rhythm was restored, esmolol was gradually weaned off. The patient was eventually discharged home in stable condition on flecainide and amiodarone. Discussion: This case illustrates the hallmark features of PJRT- a rare but serious subtype of SVTs that are resistant to pharmacological therapy. In one retrospective chart review, pharmacotherapy only led to complete cessation in 23% of PJRT cases. In comparison, ablation had a 90% success rate. Failure to successfully treat PJRT has dire consequences – leading to dilated cardiomyopathy, heart failure, and death. SVTs resistant to typical pharmacology should raise concerns for PJRT, a rare but potentially lethal cause of SVT typically treated with ablation. However, in neonates this procedure is very rarely performed. Given its resistance to pharmacologic agents, PJRT demands early recognition and a targeted approach to management in pediatric and neonatal populations.