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Erschienen in:
05.09.2023 | Case Report
Twin JET Engines: Multiple JET Foci in Patient With Heterotaxy
Erschienen in: Pediatric Cardiology | Ausgabe 8/2023
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This is a 6-week-old F with heterotaxy, right-atrial isomerism, common atrium, double-outlet right ventricle, large inlet VSD, and infradiaphragmatic total anomalous pulmonary venous return (TAPVR). Pre-operatively she demonstrated the evidence of twin AV nodes (Fig. 1). She underwent an uncomplicated TAPVR repair and PA band placement and developed episodes of both narrow and wide complex tachycardia, leading to a code event. Telemetry (Fig. 2) showed narrow complex junctional ectopic tachycardia (JET) with change to a wide complex pattern prior to the code. Both QRS morphologies on telemetry were consistent with her twin AV nodes and VA dissociation during both narrow and wide complex tachycardia made JET the most likely diagnosis. She was loaded with amiodarone and remained in sinus rhythm with no evidence of ventricular arrhythmias. After 72 h post-operatively, she was weaned off of amiodarone without recurrence though she did continue to have intermittent conduction through her twin AV nodes with minimal decrease in hemodynamics when conducting through the anterior AV node. This represents the first reported case of twin junctional ectopic tachycardias.
Fig. 1
A The initial baseline 12-lead ECG demonstrates sinus rhythm (PR 120 ms) with a narrow QRS complex (QRS duration 80 ms) due to conduction through the posterior AV node, left axis deviation (−88), and abnormal R-wave progression in the precordial leads. B An additional pre-operative 12-lead ECG demonstrates sinus rhythm with similar P-wave axis (PR 112 ms) with a wider-QRS morphology (QRS duration 102 ms) due to conduction through the anterior AV node, northwest QRS axis (+243), and abnormal R-wave progression in the precordial leads. Based on the diagnosis of heterotaxy with right-atrial isomerism, these studies confirmed the presence of twin AV nodes
Fig. 2
A The initial strip shows evidence of narrow complex tachycardia with VA dissociation consistent with junctional ectopic tachycardia. Though JET was likely diagnosis in this scenario, differential does include both AVNRT and twin-AVNRT. B Narrow complex JET continues and there is a change to a wide complex tachycardia with VA dissociation. The arterial line tracing demonstrates the loss of waveform with resulting code event requiring CPR. The patient was loaded with amiodarone which led to appropriate rhythm with conversion to sinus. C Telemetry during a period of sinus rhythm later in the course demonstrates the evidence of sinus rhythm with a wide QRS complex consistent with twin AV node physiology. The ECG leads displayed are lead III and they show consistent morphology of both twin AV nodes based on baseline ECGs and telemetry though additional ventricular tachycardia focus cannot be completely ruled out as a cause of the wide QRS tachycardia
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