Thoracic endovascular repair of a rare case of leaking aortic arch intramural hematoma secondary to Giant cell arteritis

Abstract

Background: Traditionally thoracic aortic aneurysms (TAA) secondary to Giant Cell Arteritis (GCA) were treated with resection and open repair. However no prior studies have reported an aortic intramural hematoma (IMH) as a presentation of GCA or outcome of thoracic endovascular aortic repair (TEVAR) in TAA or IMH secondary to GCA. Case presentation: A 59 year old female, nonsmoker, non-hypertensive, non-diabetic with a known history of GCA, temporal arteritis on prednisone presented with shortness of breath & chest pain. Chest CT revealed aortic arch IMH and large left hemothorax. CTA confirmed distal aortic arch focal dilation, a focal intimal irregularity in the distal aortic arch and extensive IMH without any active extravasation or signs of aortitis. Patient underwent an urgent TEVAR without oversizing the aortic landing zones. Post TEVAR aortogram showed exclusion of the site of IMH origin and dilated aortic arch segment by the stent and absence of active extravasation. One month post-TEVAR CTA showed patent stent graft with resolution of IMH and hemothorax. One year after TEVAR, patient remained asymptomatic. Conclusion: GCA can present as an IMH secondary to underlying chronic vasculitis. When endovascular repair is considered, great care should be taken not to grossly oversize aortic landing zones.