Long-Term Outcomes of Frozen Elephant Trunk Technique for Acute Type A Aortic Dissection in Marfan Syndrome

Objective We seek to evaluate the long-term outcomes of the frozen elephant trunk (FET) technique for acute type A dissection (ATAAD) in patients with Marfan syndrome (MFS). Methods Data were analyzed for 113 patients with MFS undergoing FET for ATAAD from September 2003 to August 2016. In a matched cohort of 103 pairs, the early and late outcomes of FET were compared between MFS and non-MFS patients. Results Mean age was 34.1 ± 9.2 years and 78 were men (69%). Malperfusion was seen in 9.7%; moderate or severe aortic regurgitation in 86.5%. Bentall was done in 98 patients (86.7%) and ascending aortic repair in 15 (13.3%). Rate of operative mortality was 8.0% (9/113), stroke 5.3%, and paraplegia 2.7%. Follow-up were 100% (104/104) for a mean duration of 10.1 ± 3.8 years (40.4% > 10 years). False lumen was obliterated in 40% and 26% at mid-descending aorta (DAo) and diaphragm at mean 8.5 ± 4.0 years, respectively. Distal aortic dilation occurred in 36 patients at 4.8 ± 3.3 years. At median 3.2 years, 19 patients underwent 20 reoperations, including thoracoabdominal aortic aneurysm repair in 14 and thoracic endovascular aortic repair in 3. At 7.0 ± 4.4 years, late death occurred in 16 patients (10 due to aortic rupture). In competing risks analysis, the rate of all-cause mortality was 19.5%, reoperation 18.4%, and reoperation-free survival 62.1%. At 12 years, 60% were free from distal aortic dilatation, and 79.5% were free from reoperation at 15 years. Comparison in the matched cohort did not show significant difference in operative mortality (8.7% vs 4.9%, P = 0.268), and survival (75.5% vs 73.9%, P = 0.983) and freedom from reoperation (90.1% vs 79.5%, P = 0.114) at 15 years from patients without MFS. DAo dilation (>5 cm) predicted late death (hazard ratio [HR] 5.91, P = 0.006); while preoperative proximal and mid-DAo diameters (mm) predicted distal aortic dilation and reoperation (HR 1.09; P = 0.009). Conclusions In this series, the FET technique achieved equally favorable long-term outcomes to patients without MFS and may be a reasonable approach to ATAAD in MFS. This study highlights the importance of rigorous postoperative follow-up, particularly for patients with a larger descending aorta before surgery.