Superior vena cava obstruction induced by pacemaker leads resulting in downhill esophageal varices

A 33-year-old male was introduced to our department to investigate the cause of downhill esophagus varices (Panel A) diagnosed 2 years ago during general medical checkup. He was implanted a pacemaker via left subclavian vein at the age of 16 due to congenital complete atrioventricular block. Contrast enhanced computed tomography showed occlusion from the innominate vein to the superior vena cava (SVC) and dilatation of the azygos vein, with varices around the esophagus (Panel B). Angiogram from above the SVC-right atrium (RA) junction showed a stenosis at the junction (Panel C), and SVC-RA pullback pressure examination showed a pressure gradient of 13 mmHg (Panel D) to the RA pressure at the stenosis sight. Downhill esophagus varices are a rare form mainly due to SVC obstruction, which in the current case was supposed to be induced by the pacemaker leads. As the SVC was totally occluded from the innominate vein, solely lead extraction without SVC reconstruction will not resolve the SVC obstruction. Resolving the stenosis at the SVC-RA has a possibility to reduce the elevated pressure. As the current varices staging were mild, the patient desired to perform close endoscopy follow-up and consider the heart operation when worsening of the varices.