Fate of distal aorta after frozen elephant trunk and total arch replacement for type A aortic dissection in Marfan syndrome.

The use of the frozen elephant trunk technique for type A aortic dissection in Marfan syndrome is limited by the lack of imaging evidence for long-term aortic remodeling. We seek to evaluate the changes of the distal aorta and late outcomes after frozen elephant trunk and total arch replacement for type A aortic dissection in patients with Marfan syndrome. Between 2003 and 2015, we performed frozen elephant trunk + total arch replacement for 172 patients with Marfan syndrome suffering from type A aortic dissection (94 acute; 78 chronic). Mean age was 34.6 ± 9.3 years, and 121 were male (70.3%). Early mortality was 8.1% (14/172), and follow-up was complete in 98.7% (156/158) at a mean of 6.2 ± 3.3 years. Aortic dilatation was defined as a maximal diameter of greater than 50 mm or an average growth rate of greater than 5 mm/year at any segment detected by computed tomographic angiography. Temporal changes in the false and true lumens and maximal aortic size were analyzed with linear mixed modeling. After surgery, false lumen obliteration occurred in 86%, 39%, 26%, and 21% at the frozen elephant trunk, unstented descending aorta, diaphragm, and renal artery, respectively. The true lumen expanded significantly over time at all segments (P < .001), whereas the false lumen shrank at the frozen elephant trunk (P < .001) and was stable at distal levels (P > .05). Maximal aortic size was stable at the frozen elephant trunk and renal artery (P > .05), but grew at the descending aorta (P = .001) and diaphragm (P < .001). Respective maximal aortic sizes before discharge were 40.2 mm, 32.1 mm, 31.6 mm, and 26.9 mm, and growth rate was 0.4 mm/year, 2.8 mm/year, 3.6 mm/year, and 2.6 mm/year. By the latest follow-up, distal maximal aortic size was stable in 63.5% (99/156), and complete remodeling down to the mid-descending aorta occurred in 28.8% (45/156). There were 22 late deaths and 23 distal reoperations. Eight-year incidence of death was 15%, reoperation rate was 20%, and event-free survival was 65%. Preoperative distal maximal aortic size (mm) predicted dilatation (hazard ratio, 1.11; P < .001) and reoperation (hazard ratio, 1.07; P < .001). A patent false lumen in the descending aorta predicted dilatation (hazard ratio, 3.88; P < .001), reoperation (hazard ratio, 3.36; P = .014), and late death (hazard ratio, 3.31; P = .045). The frozen elephant trunk technique can expand the true lumen across the aorta, decrease or stabilize the false lumen, and stabilize the distal aorta in patients with Marfan syndrome with type A aortic dissection, thereby inducing favorable remodeling in the distal aorta. This study adds long-term clinical and radiologic evidence supporting the use of the frozen elephant trunk technique for type A dissection in Marfan syndrome.

Chen Y ，Ma WG ，Zhi AH ，Lu L ，Zheng J ，Zhang W ，Liu YM ，Zhu JM ，Elefteriades JA ，Sun LZ ... -  《-》

Total arch replacement and frozen elephant trunk for type A aortic dissection after Bentall procedure in Marfan syndrome.

Chen Y ，Ma WG ，Zheng J ，Liu YM ，Zhu JM ，Sun LZ ... -  《-》

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.

Chen Y ，Ma WG ，Li JR ，Zheng J ，Li Q ，Liu YM ，Zhu JM ，Sun LZ ... -  《-》

Is the frozen elephant trunk technique justified for chronic type A aortic dissection in Marfan syndrome?

Chronic type A aortic dissection (cTAAD) in Marfan syndrome (MFS) is rare. Surgical experience is limited and the role of frozen elephant trunk (FET) technique remains undefined. We seek to evaluate the safety and efficacy of the total arch replacement (TAR) and FET technique for cTAAD in MFS. The clinical data of sixty-eight patients with MFS undergoing FET and TAR for cTAAD were analyzed. Mean age was 35.8±9.7 years and thirty-nine were male (57.4%). Operative mortality was 10.3% (7/68). Stroke occurred in one (1.5%), re-exploration for bleeding in five (7.3%), low cardiac output in four (5.9%), and acute renal failure in two (2.9%). Follow-up was complete in 100% (61/61) at mean 7.3±4.0 years. The false lumen was obliterated in 73.5% across FET and 50.0% in unstented descending aorta (DAo). Distal dilation occurred in twenty patients, six of whom underwent thoracoabdominal aortic replacement, one abdominal aortic replacement and one thoracic endovascular aortic repair (TEVAR). Late death occurred in five. At ten years, 59.8% were free from distal aortic dilation, and the incidences were 23.2% for death, 14.4% for distal reoperation, and 62.4% for reoperation-free survival. Predictors for operative mortality were extra-anatomic bypass [odds ratio (OR), 229.592; P=0.036], preoperative maximal size (DMax) of aortic sinuses (mm) (OR, 1.134; P=0.032) and cardiopulmonary bypass (CPB) time (minute) (OR, 1.061; P=0.041). Risk factors for aortic dilatation included patent false lumen at diaphragmatic hiatus [hazard ratio (HR), 5.374; P=0.008], preoperative DMax (mm) of proximal DAo (HR, 1.068; P=0.001) and renal arteries (HR, 1.102; P=0.005) which also predicted distal reoperation (HR, 1.149; P=0.001). The time from onset to operation (day) (HR, 1.002; P=0.004) and CPB time (minute) (HR, 1.032; P=0.036) predicted late death. This study shows that the TAR and FET technique is a safe and durable approach to cTAAD in patients with MFS. The operation should be performed as early as possible to optimize clinical outcomes.

Chen Y ，Ma WG ，Li JR ，Zheng J ，Liu YM ，Zhu JM ，Sun LZ ... -  《-》

Aortic remodeling, reintervention, and survival after zone 0 arch repair with frozen elephant trunks for acute type A aortic dissection: Midterm results.

To investigate the midterm results after zone 0 arch repair with frozen elephant trunks for acute type A aortic dissection. Between October 2014 and April 2021, 196 patients underwent zone 0 arch repair with frozen elephant trunks for acute type A aortic dissection. The true lumen area, aortic lumen area, and false lumen status were assessed at four aortic levels, the proximal and distal descending thoracic aorta (level A and level B, respectively), celiac artery branching (level C), and terminal aorta (level D). Aortic remodeling (postoperative area as a percentage of the preoperative area) was classified into 3 groups, positive (true lumen area ≥120% with aortic lumen <120% or true lumen area ≥80% with aortic lumen <80%), minimal (80% ≤ true lumen area and aortic lumen area <120%), and negative remodeling (all other changes). In-hospital mortality was 13 (6.6%) patients. The overall survival rate was 85.1% at 5 years. The freedom from distal aortic reintervention was 89.9% at 5 years. The prevalence of completely thrombosed or obliterated false lumen at 2 years was 96.8% at level A, 88.4% at level B, 47.2% at level C, and 27.6% at level D. The prevalence of positive aortic remodeling at 2 years was 84.7% at level A, 75.0% at level B, 29.2% at level C, and 16.7% at level D. Zone 0 arch repair with frozen elephant trunks for acute type A aortic dissection can avoid invasive aortic arch resection and facilitate aortic remodeling of the descending thoracic aorta. The FET effect on aortic remodeling is limited at the aortic level below the FET stent end.

Wada T ，Yamamoto H ，Takagi D ，Kadohama T ，Yamaura G ，Kiryu K ，Igarashi I ... -  《-》