Cor triatriatum dextrum is an extremely rare congenital heart abnormality in which the right atrium is separated into two chambers by a persistent fibrous membrane. A transvenous approach to place a dual-chamber pacemaker in such patients is technically challenging. We report the first case of a transvenous permanent pacemaker placement in a patient with cor triatriatum dextrum. An 87-year-old woman was diagnosed with paroxysmal atrial fibrillation. She was accidentally found to have cor triatriatum dextrum during the transesophageal echocardiography (TEE) prior to cardioversion. Later during her hospital stay, it was indicated to place a permanant pacemaker due to high grade atrioventricular block. After thorough reviewing TEE imagings, a transvenous catheter-based approach was decided feasible. Patient successfully received a dual chamber pacemaker through left subclavian venous approach. Furthermore in our case, using specially designed pacemaker leads and cautious intra-procedural maneuvering under fluoroscopic guidance ensured procedural success. In summary, a thorough pre-operative evaluation with transesophageal echocardiography is critical for the planning and eventual success of the transvenous placement of right-sided leads.
