Early and Midterm results of Frozen Elephant Trunk Operation with Evita Open Stent-Graft in Patients with Marfan Syndrome: Results of a Multicentre Study

Background: Endovascular treatment of patients with Marfan syndrome (MFS) is not recommended. Hybrid procedures such as frozen elephant trunk (FET), which combines stent-graft deployment and an integrated non-stented fabric graft for proximal grafting and suturing have not been previously evaluated. The aim of this study was to assess the safety and feasibility of FET operation in patients with MFS. Methods: Between January 2005 and January 2017 out of 1049 patients 1020 who underwent the FET procedure were enrolled in the International E-vita Open Registry (IEOR). Included were patients, who met Ghent criteria for MFS. Early and midterm results were retrospectively analyzed. Postoperative and follow-up computed tomography angiography scans were analyzed. Results: We analyzed 37 patients [mean age 38±11 years, 65% men]. Acute or chronic aortic dissection was present in 35 (95%) patients (14 and 21 patients respectively). Two (5%) patients had an aneurysm without dissection. Malperfusion syndrome was present in 4 patients. Twenty-nine (78%) patients had previous aortic surgical interventions. The 30-day and in-hospital mortality was 8 and 14% respectively. False lumen exclusion was presented in 73% in stented segment. The overall 5-year survival was 71% and freedom from reintervention downstream was 58% at 5 years. Of the nine patients who required reintervention for distal aortic disease one patient died. Conclusions: FET operation for patients with MFS can be performed with acceptable mortality and morbidity. In long-term follow up no reinterventions on the aortic arch were required. FET allows for easier second stage operations providing platform for surgical and endovascular reinterventions.