Abstract
BACKGROUND: Permanent pacing in pediatric patients is complicated by small body size, vascular access limitations, and the need for durable long-term management. Leadless pacemakers offer an emerging alternative that minimizes infection and lead-related complications, yet vascular access remains a key challenge in young patients. CASE PRESENTATION: We report a 14-year-old male with congenital central hypoventilation syndrome (CCHS; PHOX2B polyalanine repeat mutation 20/26) who presented with recurrent nocturnal oxygen desaturation secondary to prolonged sinus pauses despite tracheostomy-assisted mechanical ventilation. Continuous implantable loop recorder monitoring confirmed pauses of up to 10 s, temporally associated with desaturation events. Given the small caliber of the femoral veins and the need for long-term pacing, a leadless pacemaker was implanted via the right internal jugular vein. Device positioning was optimized under fluoroscopic guidance to achieve stable septal fixation with excellent electrical parameters (sensing: 9 mV; threshold 0.5 V at 0.24 ms; impedance: 530 Ω). The procedure and recovery were uneventful. FOLLOW-UP: At 1-year follow-up, pacing burden was 1% with stable sensing and threshold parameters, and no recurrent desaturation episodes were observed. CONCLUSION: This case highlights the complex interplay between arrhythmia and oxygen desaturation in CCHS, illustrating how permanent pacing can restore both rhythm and ventilatory stability. Transjugular leadless pacemaker implantation represents a safe and effective option for adolescents when femoral access is limited. In younger patients, careful anatomical assessment, awareness of long-term device management, and individualized selection between retrievable and established systems are essential to optimize outcomes.