报道1例62岁右心室畸形合并病态窦房结综合征女患者，因长期服用糖皮质激素为起搏器感染高危人群，在全身麻醉及经食管超声心动图引导下经颈内静脉入路植入主动固定式无导线起搏器。手术顺利，术后患者反复心悸、头晕症状消失，心电图示起搏心率60次/min，起搏与感知功能正常。本例提示经颈内静脉入路植入主动固定式无导线起搏器对右心室畸形合并病态窦房结综合征患者安全可行，术前多模态影像评估及术中精细操作是成功关键。

One case of a 62-year-old female patient with right ventricular malformation complicated by sick sinus syndrome was reported. Due to the high risk of pacemaker infection associated with long-term glucocorticoid use, an active-fixation leadless pacemaker was implanted via the internal jugular vein approach under general anesthesia with transesophageal echocardiography guidance. The procedure was successfully completed. Postoperatively, the patient's recurrent palpitations and dizziness resolved completely. Electrocardiography revealed a pacing rate of 60 bpm with normal pacing and sensing functions. This case indicates that implantation of an active-fixation leadless pacemaker via the internal jugular vein approach is safe and feasible for patients with right ventricular malformation, complicated by sick sinus syndrome. Preoperative multimodal imaging assessment and meticulous intraoperative manipulation are the key factors for successful outcomes.