A Case of Lown-Ganong-Levine Syndrome: Due to an Accessory Pathway of James Fibers or Enhanced Atrioventricular Nodal Conduction (EAVNC)?
Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease
Juanita Hunter, Emmanouil Tsounias, John Cogan, Ming-Lon Young
Department of Pediatrics, University of Miami, Miami, FL, USA
Am J Case Rep 2018; 19:309-313
Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia. The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). Similar features are seen in enhanced atrioventricular nodal conduction (EAVNC), with the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria.
CASE REPORT: A 17-year-old man presented with a history of recurrent narrow-complex and wide-complex tachycardia on electrocardiogram (ECG). An electrophysiologic study showed an unusually short atrial to His (AH) conduction interval and a normal His to ventricle (HV) interval, without a delta wave. Two stable AH intervals coexisted in the same atrial pacing cycle length. In the recovery curve study, this pathway had a flat conduction curve without an AH increase until the last 60 ms, before reaching the effective refractory period. These ECG changes did not respond to an adenosine challenge. When this pathway became intermittent, there was a paradoxical response to adenosine challenge with conduction via a short AH interval, but without conduction block. Catheter ablation of the AV nodal region resulted in a normalized AH interval, decremental conduction properties, and resulted in a positive response to an adenosine challenge.
CONCLUSIONS: In this case of Lown-Ganong-Levine syndrome, electrophysiologic studies supported the role of the accessory pathway of James fibers.
Keywords: Accessory Atrioventricular Bundle, Catheter Ablation, Lown-Ganong-Levine Syndrome