Early and midterm results of frozen elephant trunk operation with Evita open stent-graft in patients with Marfan syndrome: results of a multicentre study

Abstract

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 with 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 Patients enrolled in the International E-vita Open Registry (IEOR) who underwent FET procedure between January 2001 and February 2020 meeting Ghent criteria for MFS were included in the study. Early and midterm results were retrospectively analyzed. Preoperative, postoperative and follow-up computed tomography angiography scans were analysed. 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 history of aortic surgical interventions. The 30-day and in-hospital mortality amounted to 8 and 14% respectively. False lumen exclusion was present in 73% in stented segment in last postoperative CT. 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.

E-vita OPEN NEO in the treatment of acute or chronic aortic pathologies: first interim results of the NEOS study

“…Other systematic reviews and meta-analyses calculated values for spinal cord injury after FET procedures as 1.9% [ 14 ], up to 6.5% [ 7 ]. Smaller studies reported values for paraparesis ranging from 2.0% to 2.7% [ 15 , 16 ], although including values reported for transient paraparesis. Hence, the NEOS study paraparesis and paraplegia rates are in line with published data in literature.…”

Challenges in Prompt Identification and Surgical Correction of Marfan Syndrome Aortic Disease in a middle-income Country: A Case Series Study.

“…It has the potential to address issues related to endoleaks and the migration of endoprostheses by securing the stent-graft more proximally. This approach aims to reduce the occurrence of Type I and Type III endoleaks or the displacement of the stent-graft and provide an instrument for incoming surgeries if needed (20,21). The method involves accessing the aortic arch through a sternotomy and directly suturing the stent-graft to facilitate a more secure xation within the aortic arch repair (23).…”

“…The method involves accessing the aortic arch through a sternotomy and directly suturing the stent-graft to facilitate a more secure xation within the aortic arch repair (23). The study published by Widenka et al supported this argument, as 37 patients with MFS who underwent a FET were analyzed and at 5 years-follow up no aortic arch reinterventions were required (21).…”

“…Open surgical approaches might be preferred to patients with MFS mainly because of the complex and extensive involvement of the aorta (with the chance of also requiring intervention of the aortic valve), the increased risk for endoleaks and endoprosthesis migration, and the young age of the patients that may face a lifetime risk of aortic complications (15). However, endovascular and hybrid approaches have been reported in the literature (13,14,(20)(21)(22). In our case series, 88% of the procedures were performed with open approaches.…”

Genetic Basis, New Diagnostic Approaches, and Updated Therapeutic Strategies of the Syndromic Aortic Diseases: Marfan, Loeys–Dietz, and Vascular Ehlers–Danlos Syndrome

“…Considering the genetic etiology and the high reintervention rate these patients experience, the use of a hybrid technique such as FET allows for easier second-stage operations, providing a platform for surgical and endovascular reinterventions [ 51 ]. Furthermore, it has been shown that even in the aortic dissection of MFS patients, FET determines a positive remodeling of the distal aorta, ensuring an increase in survival and freedom from reoperation in the long term [ 52 ].…”