Surgical treatment of aortic root aneurysm in adolescent patients with Marfan syndrome_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

XIA Yu , LI Chengnan , GE Yipeng , YANG Yi , CHEN Lei ,  ZHU Junming , LIU Yongmin , SUN Lizhong

Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, 100029, P.R.China;

Corresponding author：

ZHU Junming, Email: anzhenzjm@163.com

Keywords：

Marfan syndrome; adolescent/teenage; aortic root aneurysm; Bentall procedure

DOI：

10.7507/1007-4848.201709069

Video：

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Objective To study the mid-term and long-term postoperative results of Bentall procedure in patients with Marfan syndrome. Methods From February 2009 to July 2016, ten adolescent patients (mean age of 12.30±2.31 years ranged 9-16 years, mean height of 172.50±12.55 mm, mean weight of 48.60±17.08 kg) underwent Bentall procedure in our hospital. All these teenage patients were with Marfan syndrome, 6 boys and 4 girls. Five of them underwent mitral valve replacement procedure at the same time while 2 of them with tricuspid valvuloplasty. Results No mortality was found in hospitalization. One patient suffered respiratory failure after surgery. No renal failure, mediastinal infection or re-operation caused by bleeding were observed. All were well followed up for 62 months. One died of malignant arrhythmia, the other were in good condition with the latest interview. Conclusion Teenage patients with Marfan syndrome who underwent Bentall procedure have favorable outcomes in mid-term and long-term follow-up, they probably will not suffer anti-coagulation complications and restriction of growth.

Citation： XIA Yu, LI Chengnan, GE Yipeng, YANG Yi, CHEN Lei, ZHU Junming, LIU Yongmin, SUN Lizhong. Surgical treatment of aortic root aneurysm in adolescent patients with Marfan syndrome. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(9): 777-780. doi: 10.7507/1007-4848.201709069 Copy

1.	Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation, 2009, 119(6): 880-890.
2.	Patel ND, Arnaoutakis GJ, George TJ, et al. Valve-sparing aortic root replacement in children: intermediate-term results. Interact Cardiovasc Thorac Surg, 2011, 12(3): 415-419.
3.	Roubertie F, Ben Ali W, Raisky O, et al. Aortic root replacement in children: a word of caution about valve-sparing procedures. Eur J Cardiothorac Surg, 2009, 35(1): 136-140.
4.	Vricella LA, Williams JA, Ravekes WJ, et al. Early experience with valve-sparing aortic root replacement in children. Ann Thorac Surg, 2005, 80(5): 1622-1626.
5.	Cattaneo SM, Bethea BT, Alejo DE, et al. Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients. Ann Thorac Surg, 2004, 77(1): 168-176.
6.	Carrel T, Berdat P, Pavlovic M, et al. Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur J Cardiothorac Surg, 2003, 24(2): 249-254.
7.	Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax, 1968, 23(4): 338-339.
8.	Gott VL, Greene PS, Alejo DE, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med, 1999, 340(17): 1307-1313.
9.	Gott VL, Cameron DE, Alejo DE, et al. Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg, 2002, 73(2): 438-443.
10.	Cameron DE, Alejo DE, Patel ND, et al. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann Thorac Surg, 2009, 87(5): 1344-1349.
11.	Anttila V, Piaszczynski M, Mora B, et al. Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms. Eur J Cardiothorac Surg, 2005, 27(3): 420-424.
12.	Rakhra SS, Brizard CP, d’Udekem Y, et al. Valve-sparing aortic root replacement in children. J Thorac Cardiovasc Surg, 2012, 144(4): 980-981.
13.	David TE, David CM, Manlhiot C, et al. Outcomes of aortic valve-sparing operations in Marfan syndrome. J Am Coll Cardiol, 2015, 66(13): 1445-1453.
14.	Kari FA, Beyersdorf F, Rylski B, et al. David Ⅰ reimplantation procedure for aortic root replacement in Marfan patients: medium-term outcome. Interact Cardiovasc Thorac Surg, 2014, 19(5): 743-748.
15.	Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J, 2014, 35(41): 2873-2926.
16.	Campens L, Demulier L, De Groote K, et al. Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories. Am J Cardiol, 2014, 114(6): 914-920.

1. Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation, 2009, 119(6): 880-890.
2. Patel ND, Arnaoutakis GJ, George TJ, et al. Valve-sparing aortic root replacement in children: intermediate-term results. Interact Cardiovasc Thorac Surg, 2011, 12(3): 415-419.
3. Roubertie F, Ben Ali W, Raisky O, et al. Aortic root replacement in children: a word of caution about valve-sparing procedures. Eur J Cardiothorac Surg, 2009, 35(1): 136-140.
4. Vricella LA, Williams JA, Ravekes WJ, et al. Early experience with valve-sparing aortic root replacement in children. Ann Thorac Surg, 2005, 80(5): 1622-1626.
5. Cattaneo SM, Bethea BT, Alejo DE, et al. Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients. Ann Thorac Surg, 2004, 77(1): 168-176.
6. Carrel T, Berdat P, Pavlovic M, et al. Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur J Cardiothorac Surg, 2003, 24(2): 249-254.
7. Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax, 1968, 23(4): 338-339.
8. Gott VL, Greene PS, Alejo DE, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med, 1999, 340(17): 1307-1313.
9. Gott VL, Cameron DE, Alejo DE, et al. Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg, 2002, 73(2): 438-443.
10. Cameron DE, Alejo DE, Patel ND, et al. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann Thorac Surg, 2009, 87(5): 1344-1349.
11. Anttila V, Piaszczynski M, Mora B, et al. Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms. Eur J Cardiothorac Surg, 2005, 27(3): 420-424.
12. Rakhra SS, Brizard CP, d’Udekem Y, et al. Valve-sparing aortic root replacement in children. J Thorac Cardiovasc Surg, 2012, 144(4): 980-981.
13. David TE, David CM, Manlhiot C, et al. Outcomes of aortic valve-sparing operations in Marfan syndrome. J Am Coll Cardiol, 2015, 66(13): 1445-1453.
14. Kari FA, Beyersdorf F, Rylski B, et al. David Ⅰ reimplantation procedure for aortic root replacement in Marfan patients: medium-term outcome. Interact Cardiovasc Thorac Surg, 2014, 19(5): 743-748.
15. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J, 2014, 35(41): 2873-2926.
16. Campens L, Demulier L, De Groote K, et al. Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories. Am J Cardiol, 2014, 114(6): 914-920.

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