Systemic ventricular implantation of a leadless pacemaker in a patient with a univentricular heart and atrioventricular node calcification

Key Teaching Points•Patients with complex congenital cardiac lesions require special consideration when deciding on the optimal pacemaker system. Implantation of a leadless pacemaker with good thresholds is feasible in patients with univentricular physiology.•The use of multiple imaging modalities helps to guide safe pacemaker implantation and optimization of thresholds.•Anticoagulation prophylaxis is essential in patients with endovascular pacemaker systems and an obligatory right-to-left shunt, although many of these patients may be treated prior to implantation. •Patients with complex congenital cardiac lesions require special consideration when deciding on the optimal pacemaker system. Implantation of a leadless pacemaker with good thresholds is feasible in patients with univentricular physiology.•The use of multiple imaging modalities helps to guide safe pacemaker implantation and optimization of thresholds.•Anticoagulation prophylaxis is essential in patients with endovascular pacemaker systems and an obligatory right-to-left shunt, although many of these patients may be treated prior to implantation.