Transvenous lead extraction in a patient with polysplenia and inferior vena cava defect

A 28-year-old woman with polysplenia was referred to our hospital for atrial lead failure. She had undergone an intracardiac repair (ICR) for incomplete atrioventricular septal defect and the implantation of epicardial pacing leads due to complete atrioventricular block at the age of 1 year. When she was 13 years old, an endocardial dual-chamber pacemaker was implanted via the right subclavian vein because of epicardial lead failure. The contrast-enhanced computed tomography scan revealed an inferior vena cava defect with an azygos vein connection to the superior vena cava, occlusion of the right brachiocephalic vein, a defect of the left brachiocephalic vein, and a persistent left superior vena cava ligated at the ICR. Therefore, lead exchange was indicated. During the operation, the temporary pacing lead and the guidewire for emergent deployment of the Bridge Occlusion Balloon® were advanced through the azygos vein and placed at the right ventricle and the hepatic vein, respectively. Both 11-Fr and 13-Fr mechanical rotational dilator sheaths were needed for the lead extraction owing to dense calcification and tight adhesions. The atrial lead was successfully extracted without any complications despite extremely restricted venous access. A new atrial lead was inserted through the space created by the 13-Fr sheath. <Learning objective: Transvenous lead extraction in patients with polysplenia is technically challenging. These patients often undergo pacemaker implantation in childhood, which results in tight adhesions and dense calcifications on the leads, and venous access is extremely restricted. It may be impossible to use a snare and deploy the endovascular balloon to prevent a catastrophic complication from the right femoral vein to the superior vena cava in cases of the inferior vena cava defect.>.