Abstract Background There is a lack of data regarding the use of hybrid stent graft prostheses in patients with hereditary thoracic aortic disease (HTAD) involving the aortic arch and proximal descending aorta. This retrospective analysis aimed to evaluate the short- and mid-term outcomes of hybrid stent-graft prostheses in Frozen Elephant Trunk (FET) procedures for patients with HTAD, with a particular focus on its safety and feasibility. Methods A total 280 patients who underwent FET procedures between October 2010 and November 2024 were retrospectively analysed in compliance with the 2024 EACTS/ STS recommendations for shared decision-making within the multidisciplinary aortic team. Among them, 51 patients had genetically confirmed HTAD (Marfan syndrome (FBN1), Loeys-Dietz syndrome (TGFBR1, TGFBR2, SMAD3, TGFB2), vascular Ehlers-Danlos syndrome (COLSA1), and non-syndromic HTAD (ACTA2, MYH11, MYLK)). The Thoraflex™ prosthesis was implanted in 50 of the 51 patients. Short- and mid-term outcomes were assessed descriptively. Survival and subsequent thoracic aortic intervention rates were analysed using the Kaplan-Meier method. Results The overall 30-day mortality was 2.0% ( n = 1). Perioperatively, permanent neurological deficit was 3.9% ( n = 2), with minor disability on the modified Rankin Scale (mRS 1 and 2). There were no instances of paraplegia. The median follow-up was 4.0 years. The 1-, 3- and 5-year overall survival rate was 93.9%, 90.6%, and 90.6%, respectively. Freedom from subsequent aortic interventions was at 1, 3, 5 years 55.8%, 45.6%, and 33.1%. Early device-related complications occurred in 7 patients (13.7%), including intraluminal FET thrombosis in 4 patients (12.5%) and distal stent graft-induced new entry in 3 patients (9.4%). Mid-term device-related complications occurred 2 patients (4.3%). Conclusions Hybrid stent graft prostheses can be safely implanted with the FET technique in elective and acute HTAD patients with arch and proximal descending aortic disease. Our single-center short- and mid-term outcomes are encouraging, but long-term durability and efficacy are not yet established. This warrants multi-center studies with extended follow-up.
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