Endovascular Repair of Postdissection Thoracoabdominal Aortic Aneurysm in Patients With Vascular Ehlers-Danlos Syndrome

PURPOSE: To report endovascular repair of postdissection thoracoabdominal aortic aneurysm (TAAA) in 2 patients with vascular Ehlers-Danlos syndrome (vEDS).

CASE REPORTS: Case 1. A 56-year-old vEDS male patient with a 50-mm type III TAAA [history of aortic root repair, hemiarch replacement, and thoracic endovascular aortic repair (TEVAR) for acute type A aortic dissection (TAAD) 7 years ago] was treated by a 2-stage procedure; first, cervical debranching of the left subclavian artery and second TEVAR and t-branch. The postoperative course was uneventful. Follow-up computed tomography angiography (CTA) 3.5 years postoperatively demonstrated aortic remodeling with patency of targeted visceral vessels and no endoleak. Case 2. A 47-year-old vEDS male patient presented with a TAAA (diameter of 67 mm). The patient had a history of aortic valve and arch replacement with elephant trunk for acute TAAD, and consequently a TEVAR and candy-plug procedure after a ruptured false lumen (FL) aneurysm of the descending thoracic aorta. He also had a surgical repair by an aorto-bi-iliac graft. Two years later, CTA demonstrated aneurysmal FL dilatation distally to the candy-plug and he was treated with fenestrated EVAR (F-EVAR).

CONCLUSION: Endovascular repair of postdissection TAAA was feasible and safe with good short-term outcome in 2 patients with vEDS.