Treatment strategy selection of Marfan syndrome combined with thoracoabdominal aortic diseases_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

SHI Juan , MA Mingjia ,  WEI Xiang

Department of Cardiovascular Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 453300, P.R.China;

Corresponding author：

WEI Xiang, Email: xiangwei@tjh.tjmu.edu.cn

Keywords：

Marfan syndrome; Stanford B aortic dissection; total thoracoabdominal aortic replacement; thoracic endovascular aortic repair; treatment

DOI：

10.7507/1007-4848.201910036

Video：

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Objective To summarize the surgical treatment plan and experience of patients with Marfan syndrome complicated with Stanford type B aortic dissection, and to explore the treatment strategy selection.Methods A retrospective analysis was conducted on 27 patients with Marfan syndrome complicated with thoracoabdominal aortic diseases who were treated in the department of cardiovascular surgery of our hospital from January 2013 to June 2019, including 13 males and 14 females, with an average age of 32.2±8.6 years. According to the patients' conditions, 19 of them received single pump-assisted blood transfusion combined with total thoracoabdominal aortic replacement (TAAAR), and 8 received thoracic endovascular aortic repair (TEVAR) in critically ill and pregnant patients. The patients were followed up in the outpatient clinic, and the thoracoabdominal aortic CT angiography was reexamined at 3 months, 6 months, 12 months and annually. The outcome of surgery, the incidence of intermediate cardiovascular adverse events, defined as the reoperation due to aortic or cardiac diseases, and intermediate survival rate were studied.Results All 27 patients successfully completed the operation, the operation time was 60-852 (395.10±222.60) min, the spinal cord ischemia time was 14-26 (19.33±3.44) min, and the abdominal viscera ischemia time was 16-23 (19.83±1.94) min. Eight patients of TEVAR were all operated in acute phase and 19 patients of TAAAR in chronic phase. Two early postoperative deaths occurred in TEVAR patients. One died of puerperal infection and multiple organ dysfunction after cesarean section at the same time. After TEVAR, type A dissection re-ocurred in one patient. The family member gave up the treatment, and the patient died of the dissection ruptured after cesarean section. During the average follow-up of 47.6±36.7 months, 1 patient died of cerebrovascular accident and 9 patients were reoperated for adverse cardiovascular events, including 4 in TEVAR and 5 in TAAAR.Conclusion TAAAR is the first choice for the treatment of Marfan syndrome combined with thoracoabdominal aortic diseases. TEVAR is easy to operate, with a low incidence of early mortality and complications, but has the risk of internal leakage and avulsion, and a high reoperation rate in the middle stage, so it can be used for high-risk elderly patients not suitable for open surgery, or as a bridge therapy for emergency patients before open surgery.

Citation： SHI Juan, MA Mingjia, WEI Xiang. Treatment strategy selection of Marfan syndrome combined with thoracoabdominal aortic diseases. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(7): 742-748. doi: 10.7507/1007-4848.201910036 Copy

1.	Stheneur C, Laffond C, Rioux S, et al. Recent progress in Marfan syndrome. Arch Pediatr, 2012, 19(5): 551-555.
2.	Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax, 1968, 23(4): 338-339.
3.	Coselli JS, LeMaire SA, Preventza O, et al. Outcomes of 3309 thoracoabdominal aortic aneurysm repairs. J Thorac Cardiovasc Surg, 2016, 151(5): 1323-1337.
4.	宫汉东, 汪曾炜, 张仁福, 等. 升主动脉瘤台并主动脉瓣关闭不全的外科治疗. 中华心胸血管外科杂志, 2001, 17(3): 13.
5.	Finkbohner R, Johnston D, Crawford ES, et al. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation, 1995, 91(3): 728-733.
6.	Ince H, Rehders TC, Petzsch M, et al. Stent-grafts in patients with Marfan syndrome. J Endovasc Ther, 2005, 12(1): 82-88.
7.	Nordon IM, Hinchliffe RJ, Holt PJ, et al. Endovascular management of chronic aortic dissection in patients with Marfan syndrome. J Vasc Surg, 2009, 50(5): 987-991.
8.	Dong ZH, Fu WG, Wang YQ, et a1. Retrograde type A aortic dissection after endovascular stent graft placement for treatment of type B dissection. Circulation, 2009, 119(5): 735-741.
9.	Waterman AL, Feezor RJ, Lee WA, et al. Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg, 2012, 55(5): 1234-1240.
10.	Eid-Lidt G, Gaspar J, Meléndez-Ramírez G, et al. Endovascular treatment of type B dissection in patients with Marfan syndrome: mid-term outcomes and aortic remodeling. Catheter Cardiovasc Interv, 2013, 82(7): E898-E905.
11.	Cooper DG, Walsh SR, Sadat U, et al. Treating the thoracic aorta in Marfan syndrome: surgery or TEVAR? J Endovasc Ther, 2009, 16(1): 60-70.
12.	赵纪春, 黄斌, 袁丁, 等. 外科联合腔内杂交手术治疗马凡综合征所致复杂胸腹主动脉夹层动脉瘤一例. 中华普通外科杂志, 2014, 29(6): 483-484.
13.	唐骁, 符伟国, 郭大乔, 等. 马凡氏综合征心血管病变的外科分期治疗 1 例报道. 临床急诊杂志, 2013, 14(3): 108-110.
14.	Lipscomb KJ, Smith JC, Clarke B, et al. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol, 1997, 104(2): 201-206.
15.	Eilen B, Kaiser IH, Becker RM, et al. Aortic valve replacement in the third trimester of pregnancy: case report and review of the literature. Obstet Gynecol, 1981, 57(1): 119-121.
16.	舒畅, 方坤, 黎明, 等. 晚期妊娠和产褥期主动脉夹层的腔内修复治疗. 中国普通外科杂志, 2013, 22(12): 1541-1547.
17.	杨璞玉, 张军, 李燕娜, 等. 合并主动脉夹层的马凡综合征孕妇的妊娠结局分析. 中华妇产科杂志, 2015, 50(5): 334-340.
18.	Grabenwöger M, Alfonso F, Bachet J, et al. Thoracic endovascular aortic repair (TEVAR) for the treatment of aortic diseases: a position statement from the European Association for Cardio-Thoracic Surgery (EACTS) and the European Society of Cardiology (ESC), in collaboration with the European Association of Percutaneous Cardiovascular Interventions (EAPCI). Eur J Cardiothorac Surg, 2012, 42(1): 17-24.
19.	Preventza O, Price MD, Amarasekara HS, et al. Chronic type Ⅰ and type Ⅲ aortic dissections: a propensity analysis of outcomes after open distal repair. Eur J Cardiothorac Surg, 2018, 54(3): 510-516.
20.	Kalkat MS, Rahman I, Kotidis K, et al. Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm. Eur J Cardiothorac Surg, 2007, 32(2): 250-254.
21.	LeMaire SA, Carter SA, Volguina IV, et al. Spectrum of aortic operations in 300 patients with confirmed or suspected Marfan syndrome. Ann Thorac Surg, 2006, 81(6): 2063-2078.
22.	张良, 于存涛, 常谦, 等. 常温非体外循环胸腹主动脉置换术治疗广泛主动脉瘤. 中华外科杂志, 2016, 54(2): 119-124.
23.	孙立忠, 程力剑, 朱俊明, 等. 常温非体外循环下全胸腹主动脉替换术. 中华胸心血管外科杂志, 2011, 27(12): 705-708.
24.	Juthier F, Rousse N, Banfi C, et al. Endovascular exclusion of patch aneurysms of intercostal arteries after thoracoabdominal aortic aneurysm repair. Ann Thorac Surg, 2013, 95(2): 720-722.

1. Stheneur C, Laffond C, Rioux S, et al. Recent progress in Marfan syndrome. Arch Pediatr, 2012, 19(5): 551-555.
2. Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax, 1968, 23(4): 338-339.
3. Coselli JS, LeMaire SA, Preventza O, et al. Outcomes of 3309 thoracoabdominal aortic aneurysm repairs. J Thorac Cardiovasc Surg, 2016, 151(5): 1323-1337.
4. 宫汉东, 汪曾炜, 张仁福, 等. 升主动脉瘤台并主动脉瓣关闭不全的外科治疗. 中华心胸血管外科杂志, 2001, 17(3): 13.
5. Finkbohner R, Johnston D, Crawford ES, et al. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation, 1995, 91(3): 728-733.
6. Ince H, Rehders TC, Petzsch M, et al. Stent-grafts in patients with Marfan syndrome. J Endovasc Ther, 2005, 12(1): 82-88.
7. Nordon IM, Hinchliffe RJ, Holt PJ, et al. Endovascular management of chronic aortic dissection in patients with Marfan syndrome. J Vasc Surg, 2009, 50(5): 987-991.
8. Dong ZH, Fu WG, Wang YQ, et a1. Retrograde type A aortic dissection after endovascular stent graft placement for treatment of type B dissection. Circulation, 2009, 119(5): 735-741.
9. Waterman AL, Feezor RJ, Lee WA, et al. Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg, 2012, 55(5): 1234-1240.
10. Eid-Lidt G, Gaspar J, Meléndez-Ramírez G, et al. Endovascular treatment of type B dissection in patients with Marfan syndrome: mid-term outcomes and aortic remodeling. Catheter Cardiovasc Interv, 2013, 82(7): E898-E905.
11. Cooper DG, Walsh SR, Sadat U, et al. Treating the thoracic aorta in Marfan syndrome: surgery or TEVAR? J Endovasc Ther, 2009, 16(1): 60-70.
12. 赵纪春, 黄斌, 袁丁, 等. 外科联合腔内杂交手术治疗马凡综合征所致复杂胸腹主动脉夹层动脉瘤一例. 中华普通外科杂志, 2014, 29(6): 483-484.
13. 唐骁, 符伟国, 郭大乔, 等. 马凡氏综合征心血管病变的外科分期治疗 1 例报道. 临床急诊杂志, 2013, 14(3): 108-110.
14. Lipscomb KJ, Smith JC, Clarke B, et al. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol, 1997, 104(2): 201-206.
15. Eilen B, Kaiser IH, Becker RM, et al. Aortic valve replacement in the third trimester of pregnancy: case report and review of the literature. Obstet Gynecol, 1981, 57(1): 119-121.
16. 舒畅, 方坤, 黎明, 等. 晚期妊娠和产褥期主动脉夹层的腔内修复治疗. 中国普通外科杂志, 2013, 22(12): 1541-1547.
17. 杨璞玉, 张军, 李燕娜, 等. 合并主动脉夹层的马凡综合征孕妇的妊娠结局分析. 中华妇产科杂志, 2015, 50(5): 334-340.
18. Grabenwöger M, Alfonso F, Bachet J, et al. Thoracic endovascular aortic repair (TEVAR) for the treatment of aortic diseases: a position statement from the European Association for Cardio-Thoracic Surgery (EACTS) and the European Society of Cardiology (ESC), in collaboration with the European Association of Percutaneous Cardiovascular Interventions (EAPCI). Eur J Cardiothorac Surg, 2012, 42(1): 17-24.
19. Preventza O, Price MD, Amarasekara HS, et al. Chronic type Ⅰ and type Ⅲ aortic dissections: a propensity analysis of outcomes after open distal repair. Eur J Cardiothorac Surg, 2018, 54(3): 510-516.
20. Kalkat MS, Rahman I, Kotidis K, et al. Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm. Eur J Cardiothorac Surg, 2007, 32(2): 250-254.
21. LeMaire SA, Carter SA, Volguina IV, et al. Spectrum of aortic operations in 300 patients with confirmed or suspected Marfan syndrome. Ann Thorac Surg, 2006, 81(6): 2063-2078.
22. 张良, 于存涛, 常谦, 等. 常温非体外循环胸腹主动脉置换术治疗广泛主动脉瘤. 中华外科杂志, 2016, 54(2): 119-124.
23. 孙立忠, 程力剑, 朱俊明, 等. 常温非体外循环下全胸腹主动脉替换术. 中华胸心血管外科杂志, 2011, 27(12): 705-708.
24. Juthier F, Rousse N, Banfi C, et al. Endovascular exclusion of patch aneurysms of intercostal arteries after thoracoabdominal aortic aneurysm repair. Ann Thorac Surg, 2013, 95(2): 720-722.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Treatment strategy selection of Marfan syndrome combined with thoracoabdominal aortic diseases

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