Thoracoabdominal aortic aneurysm in connective tissue disorder patients

Abstract

Connective tissue disorders (CTDs) are a group of genetically triggered diseases in which the primary defect involves collagen and elastin protein assembly with potential vascular degenerations such as thoracoabdominal aortic aneurysm (TAAA) and dissection. These most commonly include Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and familial thoracic aortic aneurysm and dissection. Open surgical repair represents the standard approach in this specific group of patients. Extensive aortic replacements are generally performed in order to reduce long-term complications caused by the progressive dilatation of the remnant aortic segments. In the last decades, endovascular interventions have emerged as a valid alternative in patients affected by degenerative TAAA. However, in patients with CTD, this approach presents higher rates of reinterventions and postoperative complications with a disputable long-term durability, and it is nowadays performed for very selective indications such as severe comorbidities and urgent/emergent settings. Despite a deeper knowledge of the pathophysiological mechanisms involved in CTD, improvements in medical therapy, and a multidisciplinary approach fully involved in the management of these usually frailer patients, this specific group still represents a challenge. Further dedicated studies addressing mid-term and long-term outcomes in this selected population are needed.

Keywords: Aortic dissection; Connective tissue disorder; Ehlers-Danlos syndrome; Loeys-Dietz syndrome; Marfan syndrome; Thoracoabdominal aortic aneurysm.

Fig. 1

Clinical presentation of a patient with MFS. (A) The patient is very tall with long arms and legs, (B) with hindfoot deformity, (C) hand arachnodactyly, and (D) pectus carinatum deformity (MFS, Marfan syndrome)

Fig. 2

(A) Using a MPR, the curved path of the dilated dissected thoracic aorta is well reproduced and the true lumen and the false lumen are properly visualized. (B) 3D volume rendering plays a role in understanding the conformation of the entire dissection and the relative position of the visceral and renal vessels, in order to plan a tailored reimplantation strategy

Fig. 3

Preoperative evaluation with angio-CT is important to assess the involvement of the iliac arteries by the dissection and the origins of the visceral vessels. The blue line on the 3D volume rendering underlines the partially compressed true lumen; on the axial scans, it is possible to observe the origins of the visceral and renal vessels from the true and false lumen

Fig. 4

Proximal anastomosis. (A) The proximal aortic clamp is placed between LCCA and LSA (blue arrow) after the left subclavian artery is selectively clamped (green arrow). The thoracic aorta is completely transected and the false (single asterisk) and the true (double asterisks) lumen are visualized. (B) The proximal end of the graft is sutured to the descending thoracic aorta with a 3/0 polypropylene running suture reinforced with a Teflon felt (blue arrows). LCCA, left common carotid artery; LSA, left subclavian artery

Fig. 5

Final reconstruction after open surgical repair of patients with CTD. (A) Visceral and renal vessels are selectively reattached using a multibranched graft. A selective bypass I was also performed to reattach a couple of critical intercostal arteries. (B) Selective reattachment of two couples of intercostal arteries using bypass. CTD, connective tissue disorder

Fig. 6

(A) Preoperative angio-CT. Dissecting TAAA in a Marfan patient with previous ascending aorta and aortic valve replacement and TEVAR. The patient developed a distal progression of disease with type IB endoleak. (B) Surgical repair with multibranched graft and selective reattachment of visceral and renal vessels. In this case, the proximal end of the graft is sutured to the distal end of the previous stent graft using a 2/0 monofilament polypropylene running suture. (C) Postoperative angio-CT-3D volume rendering. CT, computerized tomography; TAAA, thoracoabdominal aortic aneurysm; TEVAR, thoracic endovascular aortic repair