Outcomes of endovascular management of the aorta in connective tissue disorders

Background Endovascular management of type B aortic dissection has been well established and accepted in the community. However, due to the lack of information on long-term outcome, endovascular management remains controversial in the setting of connective tissue disorders such as Marfan syndrome. Although surgical repair is recommended in type B aortic dissection with connective tissue disorders, recent studies demonstrated both feasibility and safety of endovascular interventions in those patients at least short term. Purpose Single centre experience and documentation of long-term outcomes after endovascular management of aortic conditions in patients with connective tissue disorders. Methods All patients with genetic confirmation of connective tissue disorders who underwent endovascular repair of the thoracic aorta were identified and evaluated retrospectively. Perioperative, procedure-specific and follow-up data were extracted from medical records of a structured surveillance program. Results Between 2002 and 2020, 18 patients were identified having undergone TEVAR procedures and protocol follow-up. The median age was 35.5 (IQL 30.0 – 42.7), and 12 (66.7%) were males; median ACEF II score was 0.6 (IQL 0.5 – 0.925). This cohort comprised 15 (83.3%) patients with genetically confirmed Marfan syndrome, two (11.1%) with Loeys-Dietz, and one (5.6%) with Ehlers-Danlos syndrome. Of these, 13 (72.2%) patients had undergone aortic root or aortic root/ascending aorta replacement independently in the past. The indication for a TEVAR procedure was seen in one case for degenerative aneurysmal disease (5.6%), in one case for elephant trunk stenosis (5.6%), in five cases for residual aortic dissection (27.8%), and in 11 cases for new type B dissection (61.1%). The most frequently used stent-graft was Valiant™ (Medtronic) in 10 cases (55.6%), followed by TAG® (Gore®) in four cases (22.2%), Zenith® (COOK®) in two cases (11.1%), and for Relay® (Bolton) and Sinus-XL® (Optimed) in one case each (5.6%). The average total covered length was 192.2±40.2 mm. Median follow-up duration was 77.5 months (IQR 35.8 - 131); three (16.7%) cases revealed failure of TEVAR and led to conversion to open surgery. Two patients (11.1%) had died including one patient in the perioperative phase. At the last follow-up, complete thrombosis of the false lumen was observed in 14 cases (82.3%), and 15 cases (88.2%) showed no aortic expansion or progression during the follow-up period resulting in an overall endovascular success rate of 72.2%. Conclusion Thoracic endovascular aortic repair can be performed for selected patients with connective tissue disorders at relatively low long-term mortality and morbidity. With improved devices and technical skills, mid- and long-term surveillance revealed promising outcomes and a low rate of aneurysmal degeneration. Structured surveillance remains crucial to detect emerging late complications and to accumulate more long-term data. FUNDunding Acknowledgement Type of funding sources: None.