Thoracic Aortic Stent Grafting in Patients with Connective Tissue Disorders: A Word of Caution

Abstract

: Use of thoracic Stent-graft in patients with connective tissue disorders (CTD) remains limited. We herein report 3 patients with CTD who underwent stent grafting. : Case 1; A male Marfan patient was operated for thoraco-abdominal aneurysm. On computed tomography (CT), large false aneurysm at the proximal anastomosis was documented which was excluded with a 30 mm Talent stent-graft with 10-15% oversize. Case 2; A female with Ehlers-Danlos syndrome had undergone resection of descending aortic thoracic aneurysm presented with an enlarging aneurysm distal to the graft. Three Talent stent-grafts (15% oversize) were deployed with balloon dilatation to exclude the aneurysm. The immediate postoperative period was complicated by an extensive intramural hematoma of the descending aorta with hemothorax, managed conservatively. Case 3; A female Marfan patient had undergone Bentall procedure and mitral repair followed with resection of the proximal descending aorta. Three months later a false aneurysm at the distal anastomosis was treated with a 24 mm Valiant stent-graft (30% oversize). Aortic dissection distal to stent was documented on the early postoperative CT. The dissected aneurysm enlarged significantly with a type I distal endoleak during follow-up. Concomitantly, the patient presented a class III dyspnea owing to a severe mitral regurgitation. The patient underwent a successful MVR and stent-graft explantation with replacement of the descending aorta. : Significant complications supervened when stent-grafts were deployed in native aorta. We thus recommend that deploying a stent-graft in a CTD diseased aorta should be considered a relative contraindication. In cases with prohibitive or high risk surgery, use of a stent-graft with minimal radial force and minimal oversizing without balloon dilatation should be considered.