Case Report of a 4-Year-Old Girl Presenting Cardiomyopathy Due to a Permanent Junctional Reciprocating Tachycardia

Background: Permanent junctional reciprocating tachycardia (PJRT) represents an infrequent supraventricular tachycardia, first described by Coumel et al in 1967. It occurs mainly in infants and children and is caused by a reentry mechanism with a slow accessory pathway commonly located in the posteroseptal region of the atrioventricular (AV) junction. Due to its decremental retrograde conduction properties, the ECG hallmarks include RP interval > PR with inverted P waves in leads II, III, aVF, and V3–6. It is often incessant which can lead to severe cardiomyopathy.

Method: We present the case of a 4-year-old girl who was referred to our hospital for treatment of dilatative cardiomyopathy. She had a tachycardia up to 200 beats/minute and was in a poor clinical condition. An inotropic therapy with dobutamine and levosimendan was started immediately. The 12-lead ECG demonstrated negative P waves in II, III, aVF, and lateral precordial leads. R-P interval was more extended than the P-R interval. The ECG hallmarks led to the diagnosis of PJRT. The tachycardia was temporarily terminated with adenosine but then it reconverted to PJRT. CMR imaging depicted a severely restricted cardiac function (LV-EF: 13%) with a dilated left ventricle. NT-ProBNP was significantly increased (31,022 ng/L). Under continuous infusion of amiodarone, the heart rate could be reduced to 140 to 160 beats/minute. We began an antiarrhythmic therapy with propranolol and digoxin, under which the cardiac function improved significantly (LV-EF: 39–44% after 2 weeks). After 1 week, the patient developed amiodarone-induced hyperthyroidism, so amiodarone had to be discontinued. The Holter ECG showed persisting episodes of PJRT, so that we began a therapy additionally with Sotalol. The patient was dismissed after 17 days in stable clinical conditions (antiarrhythmic medication at discharge: digoxin, propranolol, and sotalol).

Conclusion: PJRT in children is frequently incessant and could be associated with severe tachycardia-induced cardiomyopathy. Rapid diagnosis and initiation of antiarrhythmic therapy are of great importance. In the long term, radiofrequency catheter ablation represents a safe and effective therapeutic strategy.

Publication History

Article published online:
12 February 2022