Chen Yu, Ma Wei-Guo, Zheng Jun, Liu Yong-Min, Zhu Jun-Ming, Sun Li-Zhong
Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing Engineering Research Center of Vascular Prostheses, Beijing 100029, China.
J Thorac Dis. 2018 Apr;10(4):2377-2387. doi: 10.21037/jtd.2018.03.79.
We seek to report the long-term outcomes of the total arch replacement and frozen elephant trunk (TAR + FET) technique for type A aortic dissection (TAAD) following prior Bentall procedure in patients with Marfan syndrome (MFS).
Between 2003 and 2015, we performed TAR + FET for 26 patients with MFS who developed TAAD following a prior Bentall procedure. Mean age at FET 36.9±9.7 years and 24 were males. TAAD was acute in 8 (30.8%, all new dissections from precious root aneurysm) and chronic in 18 (69.2%, 15 residual and 3 new). The interval from Bentall procedure to FET averaged 6.4±5.8 years, which was significantly longer in the acute group (10.3±6.3 4.6±4.9, P=0.021). The early and long-term outcomes were compared between two groups and risk factors identified for late adverse events.
Operative mortality was 11.5% (3/26). Stroke, lower limb ischemia and reexploration for bleeding occurred in 1 patient each (3.8%). Follow-up was complete in 100% (23/23) at mean 5.1±2.3 years (range, 0.9-11.2 years). The maximal diameter (DMax) of distal aorta in the chronic group was significantly greater at the unstented descending aorta [DA, (56.4±15.5 35.6±12.2 mm, P=0.006)] compared to acute patients. The false lumen was obliterated in 95.7% across the FET and 56.5% in the unstented DA. Distal aortic dilation occurred in 13 patients (11 chronic, 68.8%). Of those 11 patients, 4 underwent an open thoracoabdominal aortic repair and 3 died of distal aortic rupture. Late death occurred in 7 patients at mean 3.9±2.5 years. At 6 years, the incidence was 18% for death, 11% for distal aortic reoperation, and 71% for reoperation-free survival. Survival did not differ between two groups (75.0% 71.3%, P=0.851), while acute patients had significantly higher freedom from late rupture and reoperation at 6 years (100% . 61.9%, P=0.046). Hypertension was the sole risk factor for distal aortic dilatation [hazard ratio (HR) =7.271; 95% confidence interval (CI), 1.814-29.143; P=0.005]. Risk factors for late adverse events were hypertension (HR =6.712; 95% CI, 1.201-37.503; P=0.030) and age <35 years (HR =6.760; 95% CI, 1.154-39.587; P=0.034).
The TAR and FET technique was feasible and efficacious for TAAD following previous Bentall procedure in patients with MFS. Early and late survival did not differ with acute and chronic dissections, while freedom from late rupture and reoperation is significantly higher in patients with acute TAAD. Patients with hypertension and aged <35 years are at higher risk for late distal aortic dilation, reoperation and death.
我们旨在报告马方综合征（MFS）患者在先前进行Bentall手术后，采用全弓置换加象鼻支架植入术（TAR + FET）治疗A型主动脉夹层（TAAD）的长期疗效。
2003年至2015年间，我们对26例MFS患者进行了TAR + FET手术，这些患者在先前的Bentall手术后发生了TAAD。象鼻支架植入时的平均年龄为36.9±9.7岁，男性24例。TAAD为急性的有8例（30.8%，均为源于既往根部动脉瘤的新发夹层），慢性的有18例（69.2%，15例为残留夹层，3例为新发夹层）。从Bentall手术到象鼻支架植入的间隔平均为6.4±5.8年，急性组明显更长（10.3±6.3对4.6±4.9，P = 0.021）。比较两组的早期和长期疗效，并确定晚期不良事件的危险因素。
手术死亡率为11.5%（3/26）。中风、下肢缺血和因出血再次手术各发生1例（3.8%）。100%（23/23）的患者完成了随访，平均随访时间为5.1±2.3年（范围0.9 - 11.2年）。与急性患者相比，慢性组降主动脉未植入支架处的远端主动脉最大直径（DMax）显著更大[降主动脉（DA），（56.4±15.5对35.6±12.2 mm，P = 0.006）]。象鼻支架植入处假腔闭合率为95.7%，降主动脉未植入支架处为56.5%。13例患者（11例慢性患者，占68.8%）发生远端主动脉扩张。在这11例患者中，4例接受了开胸胸腹主动脉修复术，3例死于远端主动脉破裂。7例患者发生晚期死亡，平均时间为3.9±2.5年。6年时，死亡率为18%，远端主动脉再次手术率为11%，无再次手术生存率为71%。两组生存率无差异（75.0%对71.3%，P = 0.851），而急性TAAD患者6年时免于晚期破裂和再次手术的比例显著更高（100%对61.9%，P = 0.046）。高血压是远端主动脉扩张的唯一危险因素[风险比（HR）= 7.271；95%置信区间（CI），1.814 - 29.143；P = 0.005]。晚期不良事件的危险因素为高血压（HR = 6.712；95% CI，1.201 - 37.503；P = 0.030）和年龄<35岁（HR = 6.760；95% CI，1.154 - 39.587；P = 0.034）。
对于MFS患者先前Bentall手术后发生的TAAD，TAR和FET技术是可行且有效的。急性和慢性夹层患者的早期和晚期生存率无差异，而急性TAAD患者免于晚期破裂和再次手术的比例显著更高。高血压患者和年龄<35岁的患者发生晚期远端主动脉扩张、再次手术和死亡的风险更高。
