Application of reimplantation technique in treating Marfan syndrome and giant aortic root aneurysm during mid-pregnancy: A case report_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

NIU Hao ¹ , QIAN Hong ¹ , SONG Haibo ² , DU Lei ² , YU Hai ² , ZHANG Eryong ¹ ,  XIAO Zhenghua ¹

1. Department of Cardiovascular Surgery, West China Hospital, Sichuan University, Chengdu, 610041, P. R. China;
2. Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, 610041, P. R. China;

Corresponding author：

XIAO Zhenghua, Email: xiaozhenghua@wchscu.cn

Keywords：

Marfan syndrome; pregnancy; valve-sparing root replacement; aortic aneurysm; case report

DOI：

10.7507/1007-4848.202304017

Video：

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Pregnancy complicated by aortic root aneurysm in patients with Marfan syndrome is one of the main causes of termination of pregnancy or even death in pregnant women. A very small number of pregnant women require cardiac surgery to preserve pregnancy under extracorporeal circulation, and all surgeries use aortic root replacement. We reported a 30-year-old patient with severe aortic regurgitation combined with giant aortic root aneurysm and Marfan syndrome in mid-pregnancy. Valve-sparing root replacement using reimplantation technology was performed via a multidisciplinary cooperation model. This not only achieved the patient’s desire to continue pregnancy but also avoided the anticoagulation and bleeding complications brought by mechanical valve replacement, reduced pregnancy risks and improved long-term quality of life. Postoperative echocardiography showed a small amount of aortic valve regurgitation, aortic valve coaptation height of 0.6 cm, effective height of 1.1 cm, maximum aortic flow velocity of 1.4 m/s, mean transvalvular pressure gradient of 4.4 mm Hg, and satisfactory clinical results.

Citation： NIU Hao, QIAN Hong, SONG Haibo, DU Lei, YU Hai, ZHANG Eryong, XIAO Zhenghua. Application of reimplantation technique in treating Marfan syndrome and giant aortic root aneurysm during mid-pregnancy: A case report. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2025, 32(3): 416-420. doi: 10.7507/1007-4848.202304017 Copy

1.	Cox DA, Ginde S, Kuhlmann RS, et al. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Arch Gynecol Obstet, 2014, 290(4): 797-802.
2.	Steinberg ZL, Dominguez-Islas CP, Otto CM, et al. Maternal and fetal outcomes of anticoagulation in pregnant women with mechanical heart valves. J Am Coll Cardiol, 2017, 69(22): 2681-2691.
3.	Keane MG, Pyeritz RE. Medical management of Marfan syndrome. Circulation, 2008, 117(21): 2802-2813.
4.	Goland S, Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome. Circulation, 2009, 119(4): 619-623.
5.	European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC guidelines on the management of cardiovascular diseases during pregnancy: The Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Euro Heart J, 2011, 32(24): 3147-3197.
6.	董秀华, 程卫平, 卢家凯, 等. 保留妊娠孕妇心脏手术的麻醉处理. 心肺血管病杂志, 2017, 36(2): 123-124.Dong XH, Cheng WP, Lu JK, et al. Anesthesia for cardiac surgery in pregnant patients. J Cardiovasc Pulm Dis, 2017, 36(2): 123-124.
7.	Bazylev VV, Rosseykin EV, Evdokimov ME, et al. Valve sparing aortic root replacement (David procedure) in 36 year-old woman with the Marfan syndrome at the 27th week of pregnancy. Khirurgiia (Mosk), 2018, 8: 75-77.
8.	Yuan SM. Indications for cardiopulmonary bypass during pregnancy and impact on fetal outcomes. Geburtshilfe Frauenheilkd, 2014, 74(1): 55-62.
9.	吴淑燕, 韩凤珍. 妊娠合并主动脉夹层12例妊娠结局分析. 中国实用妇科与产科杂志, 2022, 38(4): 455-458.Wu SY, Han FZ. Analysis of pregnancy outcomes in 12 cases of pregnancy complicated with aortic dissection. Chin J Pract Gynecol Obstet, 2022, 38(4): 455-458.
10.	陈劲进, 肖颖彬, 王咏, 等. 妊娠合并心脏病患者的外科手术策略. 局解手术学杂志, 2019, 28(5): 361-364.Chen JJ, Xiao YB, WangY, et al. Surgical strategies for patients with pregnancy complicated by heart disease. J Reg Anat Oper Surg, 2019, 28(5): 361-364.
11.	van Hagen IM, Roos-Hesselink JW, Ruys TP, et al. Pregnancy in women with a mechanical heart valve: Data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC). Circulation, 2015, 132(2): 132-142.
12.	Vranes M, Velinovic M, Kovacevic-Kostic N, et al. Pregnancy-related aortic aneurysm and dissection in patients with Marfan's syndrome: Medical and surgical management during pregnancy and after delivery. Medicina, 2011, 47(11): 87.
13.	Kunihara T, Ichihara N, Miyata H, et al. Valve-sparing root replacement and composite valve graft replacement in patients with aortic regurgitation: From the Japan Cardiovascular Surgery Database. J Thorac Cardiovasc Surg, 2019, 158(6): 1501-1511.
14.	Tang GH, Malekan R, Lansman SL, et al. Aortic valve-sparing reimplantation and mitral repair in a pregnant, second trimester Marfan patient: surgical decision. Ann Thorac Surg, 2013, 95(2): 701-703.
15.	Vallabhajosyula P, Szeto WY, Habertheuer A, et al. Bicuspid aortic insufficiency with aortic root aneurysm: Root reimplantation versus bentall root replacement. Ann Thorac Surg, 2016, 102(4): 1221-1228.
16.	Yates MT, Soppa G, Smelt J, et al. Perioperative management and outcomes of aortic surgery during pregnancy. J Thorac Cardiovasc Surg, 2015, 149(2): 607-610.

1. Cox DA, Ginde S, Kuhlmann RS, et al. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Arch Gynecol Obstet, 2014, 290(4): 797-802.
2. Steinberg ZL, Dominguez-Islas CP, Otto CM, et al. Maternal and fetal outcomes of anticoagulation in pregnant women with mechanical heart valves. J Am Coll Cardiol, 2017, 69(22): 2681-2691.
3. Keane MG, Pyeritz RE. Medical management of Marfan syndrome. Circulation, 2008, 117(21): 2802-2813.
4. Goland S, Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome. Circulation, 2009, 119(4): 619-623.
5. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC guidelines on the management of cardiovascular diseases during pregnancy: The Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Euro Heart J, 2011, 32(24): 3147-3197.
6. 董秀华, 程卫平, 卢家凯, 等. 保留妊娠孕妇心脏手术的麻醉处理. 心肺血管病杂志, 2017, 36(2): 123-124.Dong XH, Cheng WP, Lu JK, et al. Anesthesia for cardiac surgery in pregnant patients. J Cardiovasc Pulm Dis, 2017, 36(2): 123-124.
7. Bazylev VV, Rosseykin EV, Evdokimov ME, et al. Valve sparing aortic root replacement (David procedure) in 36 year-old woman with the Marfan syndrome at the 27th week of pregnancy. Khirurgiia (Mosk), 2018, 8: 75-77.
8. Yuan SM. Indications for cardiopulmonary bypass during pregnancy and impact on fetal outcomes. Geburtshilfe Frauenheilkd, 2014, 74(1): 55-62.
9. 吴淑燕, 韩凤珍. 妊娠合并主动脉夹层12例妊娠结局分析. 中国实用妇科与产科杂志, 2022, 38(4): 455-458.Wu SY, Han FZ. Analysis of pregnancy outcomes in 12 cases of pregnancy complicated with aortic dissection. Chin J Pract Gynecol Obstet, 2022, 38(4): 455-458.
10. 陈劲进, 肖颖彬, 王咏, 等. 妊娠合并心脏病患者的外科手术策略. 局解手术学杂志, 2019, 28(5): 361-364.Chen JJ, Xiao YB, WangY, et al. Surgical strategies for patients with pregnancy complicated by heart disease. J Reg Anat Oper Surg, 2019, 28(5): 361-364.
11. van Hagen IM, Roos-Hesselink JW, Ruys TP, et al. Pregnancy in women with a mechanical heart valve: Data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC). Circulation, 2015, 132(2): 132-142.
12. Vranes M, Velinovic M, Kovacevic-Kostic N, et al. Pregnancy-related aortic aneurysm and dissection in patients with Marfan's syndrome: Medical and surgical management during pregnancy and after delivery. Medicina, 2011, 47(11): 87.
13. Kunihara T, Ichihara N, Miyata H, et al. Valve-sparing root replacement and composite valve graft replacement in patients with aortic regurgitation: From the Japan Cardiovascular Surgery Database. J Thorac Cardiovasc Surg, 2019, 158(6): 1501-1511.
14. Tang GH, Malekan R, Lansman SL, et al. Aortic valve-sparing reimplantation and mitral repair in a pregnant, second trimester Marfan patient: surgical decision. Ann Thorac Surg, 2013, 95(2): 701-703.
15. Vallabhajosyula P, Szeto WY, Habertheuer A, et al. Bicuspid aortic insufficiency with aortic root aneurysm: Root reimplantation versus bentall root replacement. Ann Thorac Surg, 2016, 102(4): 1221-1228.
16. Yates MT, Soppa G, Smelt J, et al. Perioperative management and outcomes of aortic surgery during pregnancy. J Thorac Cardiovasc Surg, 2015, 149(2): 607-610.

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