马方综合征主动脉根部手术后远端主动脉病变再次外科治疗

Reoperation for distal aortic disease after root surgery in Marfan syndrome patients

目的总结马方综合征主动脉根部手术后远端主动脉病变的再次外科治疗结果，探讨相关治疗策略。方法2000年1月至2010年1月，28例马方综合征主动脉根部手术后远端主动脉病变患者进行再次手术治疗。其中男20例，女8例；年龄23～52岁，平均（38．5±8．7）岁。首次手术包括Ben-tall手术24例，David手术4例。StanfordA型夹层8例，主动脉根部瘤20例。再次手术包括：胸腹主动脉置换术10例，全主动脉弓置换及支架象鼻术7例，胸降主动脉置换术6例，全主动脉置换术2例，全主动脉弓置换术2例，部分主动脉弓置换术1例。两次手术间隔1～12年，平均（6．43±3．07）年。结果术后发生神经系统并发症4例（17％），包括脑卒中1例，截瘫1例，单侧下肢一过性运动障碍2例。二次开胸止血3例，急性肾功能衰竭接受血滤治疗1例。3例因术后呼吸机辅助时间延迟接受气管切开术。术后全部随访，随访时间10～118个月，平均（40．8±29．5）个月。住院死亡2例（7．1％），术后1年、5年实际生存率分别为（94．5±1．3）％、（90．6±1．4）％。结论马方综合征行主动脉根部手术后因远端主动脉病变再次外科治疗临床结果满意。对于患主动脉A型夹层的马方综合征，首次手术即采用积极的主动脉全弓置换及象鼻手术更好。

Objective Analyze the results of distal aortic reoperation in Marfan syndrome patients after proximal aortic surgery. Methods Between January 2000 and January 2010, 28 Marfan patients underwent surgical repair of distal aortic disease after aortic root surgery at our institution. There were 20 males and 8 females. Age ranged from 23 to 52 years [ mean （ 38.5 ± 8.7 ） years ]. First time operations were Bentall procedure in 24, David procedure in 4. There were 8 cases of Stanford A dissection and 20 cases of aortic root aneurysm. The second time operations included 1 partial aortic arch replacement, 2 total arch replacements, 7 total arch replacements combined with stent elephant trunk, 6 descending thoracic aorta replacements, 10 thoracoabdominal aorta replacements and 2 total aorta replacements. The interval between the 1st and 2nd operation was 1 to 12 years [ mean （6.43 ± 3.07 ） years]. The surgical technique used for distal procedures was dependent on the pathology of the aorta. Median repeat sternotomy was used for surgical exposure in 10 patients, who required an arch/proximal descending aortic procedure. Cardiopulmonary bypass （CPB） was established through the right axillary perfusion and right femoral venous drainage in all of these patients. 16 patients were operated on through a left-sided incision, namely, a posterolateral thoracotomy, or a thoracoabdominal incision, depending on the distal extent of aortic replacement. For such patients, the left femoral vessels were cannulated for CPB in 14 patients and the rest 2 patients were operated on without CPB. Two patients with total aorta replacement were operated on through a median sternotomy combined with thoracoabdominal incision. 22 patients underwent deep hypothermic circulatory arrest because of the replacement of aortic arch or the extensive aneurismal arch. Reconstruction of intercostal arteries （ t8 - L1 ） was performed in 16 patients for the protection of spinal cord. Results Two patients （7.1% ） died postoperatively. Neurological morbidity included 1 patient with stroke, 1 paraplegia and 2 temporary paraparesis Three patients required temporary tracheotomy for prolonged weaning form the respirator. All patients were followed up for 10 - 118 months [ mean （40.8 ± 29.5 ） months 1. Two patients died during follow-up. The survival rate was （94.5 ±1.3 ） %, （90.6 ± 1.4） % at 1- and 5- year, respectively. Conclusion Distal aortic reoperation in Marian patients can be performed with good short- and long-term results. Complete aortic arch replacement with elephant trunk technique, if safely performed, should be considered for Marian patients presenting with type A dissection.