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

Loeys-Dietz syndrome is an autosomal-dominant connective tissue disorder characterized by aggressive vascular manifestations, often necessitating early surgical intervention. Informed consent has been obtained from the patient for publication of the case report and accompanying images-videos. Patient gave written informed consent for the anonymous collection of the data for the study in the consent form provided by our institution and approved by Institutional Review Board. We report on a 14-year-old girl with Loeys-Dietz syndrome type 2, with previous open surgical repair of the aortic root and arch, who presented with a symptomatic diverticulum of the ductus arteriosus and critical right upper extremity ischemia, owing to newly diagnosed occlusion of a subclavian artery (SA) aneurysm. Urgent surgical management included the exclusion of the right SA aneurysm using an 8-mm reinforced Dacron graft. In a second procedure 2 days later, left common carotid artery-SA bypass and thoracic endovascular aortic repair landing in zone 2 followed. Postoperative scans revealed distal progression of the disease, resulting in distal extension with thoracic endovascular aortic repair and eventually in an open thoracoabdominal aortic repair.