Can Frozen Elephant Trunk Cure Type I Dissection Confined to Thoracic Aorta in Marfan Syndrome?

This study sought to evaluate the long-term impact of frozen elephant trunk (FET) on the distal aorta of patients with Marfan syndrome (MFS) who had type I dissection confined to the thoracic aorta (above the diaphragmatic hiatus).

Between 2003 and 2016, 42 patients with MFS (Ghent or revised Ghent criteria) (age 33.3 ± 8.9 years; 27 men; 64.3%) sustaining type I dissection above the diaphragmatic hiatus involving the aortic arch (22 acute; 52.4%) underwent total arch replacement and FET. Dissection extended distally to the mid-descending aorta in 32 (76%) and to above the diaphragmatic hiatus in 10 (24%) patients. Operative mortality was 4.8% (2 of 42). Follow-up was 100% at 6.3 ± 3.0 years.

Maximal aortic sizes at the mid-descending aorta, diaphragmatic hiatus, renal arteries, and largest segment of abdominal aorta were 22.8, 21.1, 19.1, and 19.9 mm preoperatively and 23.1, 22.0, 19.8, and 22.4 mm on the latest computed tomographic angiography. Dilation and complete remodeling of the distal aorta occurred in 10.0% (4 of 40) and 90% (36 of 40) of patients, respectively. One late death occurred, and 3 distal reoperations were performed. Preoperative abdominal aortic maximal aortic size was predictive of distal dilatation (mm) (hazard ratio, 1.78; P = .021) and reoperation (≥25 mm vs <25 mm) (hazard ratio, 12.88; P = .037). At 10 years, freedom from dilation, reoperation, and death were 69.8%, 78.1%, and 90.0%, respectively. At 8 years, the rates of death, reoperation, and reoperation-free survival were 10%, 11%, and 79%, respectively.

The FET technique has a positive remodeling impact on type I dissection confined to the thoracic aorta in patients with MFS. This study adds evidence supporting the safety and durability of this extended approach for type I aortic dissection in MFS.