Leadless AV Synchronous Pacing in a Patient With Ebstein's Anomaly and Prior Glenn Shunt

Leadless pacing offers an important alternative to transvenous systems in patients with complex congenital heart disease and limited venous access.

We report the case of a 29-year-old woman with Ebstein anomaly, hypoplastic right ventricle, prior bidirectional Glenn shunt, multiple septal defect repairs, and transcatheter pulmonic valve replacement who presented with symptomatic paroxysmal atrioventricular block. Given preserved biventricular function and complex right-sided anatomy, a leadless pacing strategy was selected. Using transesophageal echocardiography, 3-dimensional electroanatomic mapping, and fluoroscopy guidance, a Micra AV leadless pacemaker was successfully implanted in the anteroseptal aspect of the right ventricle without interfering with the apically displaced septal tricuspid leaflet.

This case highlights the importance of individualized procedural planning and multimodality imaging to enable safe leadless pacemaker implantation in patients with complex congenital heart disease.

Leadless AV synchronous pacing is feasible in complex congenital heart disease with careful procedural planning and imaging guidance.