Multistaged aortic repair for a 14-year-old girl with Loeys-Dietz syndrome

<h2>Abstract</h2> Loeys-Dietz Syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by aggressive vascular manifestations, often necessitating early surgical intervention. We report on a 14-year-old female with LDS Type 2, with a previous open surgical repair (OSR) of aortic root and arch, who presented with a symptomatic diverticulum of the ductus arteriosus and critical right upper extremity ischemia, due to newly diagnosed occlusion of a subclavian artery (SA) aneurysm. Urgent surgical management included the exclusion of the right SA aneurysm using a 8mm reinforced Dacron graft. In a second procedure two days later, left common carotid artery (LCCA) - SA bypass and thoracic endovascular aortic repair (TEVAR) landing in zone 2 followed. Postoperative scans revealed distal progression of the disease, resulting in distal extension with TEVAR and eventually in an open thoracoabdominal aortic repair.