Absent ventriculo-atrial conduction during right ventricular apical pacing but nodal response during para-Hisian pacing - What is the mechanism?

A 13-year-old-girl presented with one episode of pre-syncope while standing in a train. Her ECG was suggestive of preexcitation. Echocardiography revealed structurally normal heart without any ventricular hypertrophy. During electrophysiology study, her ventriculo-atrial (VA) conduction was absent even on isoprenaline. However, a para-Hisian pacing maneuver (PHP) revealed consistent VA conduction with a nodal response. This finding indicated that the VA dissociation at baseline was at infra-Hisian (VH) level and conduction at HA level was intact. In addition, this finding is coherent with a speculation of a fasciculo-ventricular pathway (FVP) resulting in such an ECG pattern in her. Pacing from various atrial sites (right atrium, coronary sinus) exhibited nearly fixed preexcitation and short non-varying HV interval confirmatory of FVP. Testing for a PRKAG mutation was advised for her.