Endovascular repair of a common iliac artery aneurysm with an iliac branch device in a patient with vascular Ehlers-Danlos syndrome due to a null COL3A1 variant

Endovascular repair is avoided in patients with connective tissues disorders due to concerns for stent graft migration and endoleaks. We describe a successful endovascular repair of a common iliac artery aneurysm with a bifurcated aortoiliac stent graft and iliac branch endoprosthesis in a patient with Vascular Ehlers-Danlos syndrome (VEDS) due to a null COL3A1 variant. This case demonstrates that the VEDS genotype is associated with tissue integrity, specifically, individuals with VEDS due to null/haploinsufficiency variants, and adds to our understanding of endovascular repair in this population

Open repair of abdominal aortic aneurysms in patients with vascular Ehlers-Danlos syndrome

Vascular Ehlers-Danlos syndrome (VEDS) is rare, affecting an estimated 1 per 50,000 individuals, and is associated with abdominal aortic aneurysms (AAAs), among other arteriopathies. We present three patients with genetically confirmed VEDS who underwent successful open AAA surgical repair and demonstrate that elective open AAA repair with careful tissue manipulation is safe and feasible for patients with VEDS. These cases also demonstrate that the VEDS genotype is associated with the aortic tissue quality (genotype–surgical phenotype correlation), with the most friable tissue encountered in the patient with a large amino acid substitution and the least friable tissue in the patient with a null (haploinsufficiency) variant

Operative repair of right intrathoracic subclavian artery aneurysms in patients with genetic arteriopathy

True intrathoracic subclavian artery aneurysms (SCAAs) are rare and have various etiologies. Right intrathoracic SCAAs pose specific anatomic challenges to repair. We present three different operative approaches, open, endovascular, and hybrid repair, for the repair of a right intrathoracic SCAA in three patients with genetic arteriopathy: Marfan syndrome, vascular Ehlers-Danlos syndrome, and unspecified Ehlers-Danlos syndrome, respectively. These cases demonstrate an individualized operative approach based on the genetic diagnosis for each patient presenting with a right intrathoracic SCAA

A multidisciplinary multistage complete mega aorta replacement and utilization of extracorporeal membrane oxygenation in thoracoabdominal aneurysm repair

A 49-year-old woman underwent a 11-month multistage complete replacement of a mega aorta. Replacement stages included ascending aorta and arch replacement in conjunction with a frozen elephant trunk thoracic endovascular aortic repair, extension of thoracic endovascular aortic repair to zone 5, and open repair of the thoracoabdominal aneurysm with the use of venoarterial extracorporeal membrane oxygenation for circulatory support. This case illustrates the complexity of repairing a mega aorta, the multidisciplinary care and staging needed for repair, and the use of peripheral venoarterial extracorporeal membrane oxygenation for circulatory perfusion during thoracoabdominal aneurysm repair