EARLY AND LONG-TERM OUTCOMES OF FROZEN ELEPHANT TRUNK IN MARFAN SYNDROME: THE BOLOGNA EXPERIENCE.

Introduction: Endovascular procedures in patients with Marfan syndrome are controversial. This study evaluates the early and long-term outcomes of the frozen elephant trunk procedure in these patients. Materials and Methods: From 2007 to 2022, 27 patients with Marfan syndrome underwent FET at our institution. Outcomes were analyzed retrospectively. Results: Mean age was 42.8 ± 13.5 years; 74.1% were male. Most procedures were reoperations (81.5%), with 74.1% previously treated for proximal aortic disease. Indications included residual type A aortic dissection (48.1%), chronic type B dissection (22.2%), degenerative aneurysm (11.1%), and acute type A and type B dissections (3.7% and 11.1%). Combined procedures were performed in 48.1% of case, with a total of 37% root replacements, such as Bentall or David procedure. Permanent and transient neurological deficits affected 3 (11.1%) and 2 (7.4%) patients, respectively; paraplegia occurred in 1 patient (3.7%). In- hospital mortality was 11.1%, comprising patients who underwent emergency surgery. Long- term survival rates at 1 and 5 years were 85.4% and 74.6%. During follow up, stent graft–induced new entry (SINE) was developed in 14.8%. TEVAR extension was required in 33.3% and open surgery in 14.8%. Freedom from TEVAR at 1 and 5 years was respectively 66.7% and 61.1%, while from open surgery was 90.5% and 77.6%. Conclusions: Frozen elephant trunk can be considered a promising option in patients with Marfan syndrome requiring arch replacement. During follow-up a straight surveillance is necessary to early detect distal aortic pathologies.