Surgical Outcomes of Patients with Marfan Syndrome Complicated by Type A Aortic Dissection_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LIU Xintian , WU Mingxiang , LIU Chengwei , FU Jun , ZHANG Zhiping , YANG Yuchun , XU Zhiyong.

Department of Cardiac Intensive Care Unit， Wuhan Asia Heart Hospital， Wuhan 430022， P. R. China;

Corresponding author：

Keywords：

Marfan syndrome; Type A aortic dissection; Survival analysis; COX regression; Time-varying covariate

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Abstract： Objective To evaluate surgical outcomes of patients with Marfan syndrome （MFS） complicated by type A aortic dissection （AAD） during follow-up. Methods We retrospectively reviewed clinical data of 44 patients with MFS complicated by AAD who were admitted to Wuhan Asia Heart Hospital from January 2006 to January 2012. There were 31 male patients and 13 female patients with their age of 12-54 （33.0±9.8） years. Twenty-three patients underwent Bentall procedure at different time after the onset of AAD， while the other 21 patients received conservative treatment in stead of surgery because of economical or other reasons. COX regression with time-varying covariates was performed to analyze related factors， using primary end point， primary end point＋secondary end point as the outcome variables respectively， to compare postoperative outcomes and quality of life between the surgical treatment patients and conservative treatment patients. Results Postoperatively 1 patient died of multiple organ failure， and the other 22 patients survived the surgery. All the 43 patients were followed up from 1 to 75 months. The 3-year survival rate of the 22 surgical treatment patients was 95.7%， and they all had a good quality of life during follow-up. The survival condition and quality of life of the 21　conservative treatment patients was poor， and 13 patients （61.9%） died with the 3-year survival rate of only 31.7%. The main causes of their death included acute cardiac tamponade， aortic dissection rupture， acute myocardial infarction andcardiogenic shock. COX regression with time-varying covariates showed that the treatment outcomes of the surgical treatment patients were statistically different from those of the conservative treatment patients after modifying the influence caused by different operating time （OR of T_COV_ =0.088， P=0.028） . The risk of death of surgical treatment was only 8.8% of that of conservative treatment. Conclusion The prognosis of patients with MFS complicated by AAD is very poor. Therefore， all these patient， both in acute stage and chronic stage， should undergo surgical treatment as early as possible. The short-term and follow-up outcomes of surgical treatment are satisfactory.

Citation： LIU Xintian,WU Mingxiang,LIU Chengwei,FU Jun,ZHANG Zhiping,YANG Yuchun,XU Zhiyong.. Surgical Outcomes of Patients with Marfan Syndrome Complicated by Type A Aortic Dissection. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2013, 20(1): 49-53. doi: Copy

1.	Kim EK， Choi ER， Ko SM， et al. Comparison of aortic dissection in korean patients with versus without the marfan syndrome. Am J Cardiol， 2012， 109 （3）：423-427.
2.	Linggen G， Lin Z， Xiaohan F， et al. Comparison of aortic dissection　 in Chinese patients with and without Marfan syndrome. Postgrad Med J， 2011， 87 （1027）：325-330.
3.	Tsai TT， Evangelista A， Nienaber CA， et al. Long-term survival in patients presenting with type A acute aortic dissection. Insights from the international registry of acute aortic dissection （IRAD）.　 Circulation， 2006， 114 （1 Suppl）：I350-I356.
4.	陈灏珠，王乐民，戍卫海，等. 主译. BRUANWALD心脏病学. 第7版. 北京：人民卫生出版社， 2007. 1776-1777.
5.	Gilon D， Mehta RH， Oh JK， et al. Characteristics and in-hospital outcomes of patients with cardiac tamponade complicating type A acute aortic dissection. Am J Cardiol， 2009， 103 （7）：1029-1031.
6.	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.
7.	Tagusari O， Ogino H， Kobayashi J， et al. Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome ? J Thorac Cardiovasc Surg， 2004， 127 （5）：1373-1380.
8.	Bachet J， Larrazet F， Goudot B， et al. When should the aortic arch be replaced in Marfan patients ? Ann Thorac Surg， 2007，83 （2）：S774-S790.
9.	郑斯宏，孙衍庆，孟旭，等. 马凡综合征主动脉根部瘤手术治疗84例经验. 中华医学杂志， 2005， 85 （32）：2279-2282.
10.	Galicia-Tornell MM， Marín-Solís B， Fuentes-Orozco C， et al. Bentall　 procedure in ascending aortic aneurysm：hospital mortality. Cir Cir， 2010， 78 （1）：45-51.
11.	Guilmet D， Bonnet N， Saal JP， et al. Long term survival with the Bentall button operation in 150 patients. Arch Mal Coeur Vaiss， 2004， 97 （2）：83-91.
12.	徐志云，邹良建，宋智钢，等．升主动脉根部瘤的外科治疗．中国胸心血管外科临床杂志， 2006， 13 （6）：374-377．.
13.	Koshiyama H， Nakajima M， Amenomori S， et al. A refined flanged Bentall technique using valsalva tube graft for proximal reinforcement. Eur J Cardiothorac Surg， 2011， 40 （6）：1537-1539.
14.	Chen LW， Dai XF， Wu XJ. A modified composite valve dacron graft for prevention of postoperative bleeding from the proximal anastomosis after Bentall procedure. Ann Thorac Surg， 2009， 88 （5）：1705-1707.
15.	Bassano C， De Matteis GM， Nardi P， et al. Mid-term follow-up　 of aortic root remodeling compared to Bentall operation. Eur J　 Cardiothorac Surg， 2001， 19 （5）：601-605.
16.	Joo HC， Chang BC， Youn YN， et al. Clinical experience with the Bentall procedure：28 years. Yonsei Med J， 2012， 53 （5）：915-923.
17.	Alexiou C， Langley SM， Charlesworth P， et al. Aortic root replacement in patients with Marfan's syndrome：the Southampton experience. Ann Thorac Surg， 2001， 72 （5）：1502-1507.
18.	Bortolotti U. Preventing bleeding from the proximal anastomosis after the Bentall procedure. Ann Thorac Surg， 2010， 90 （2）：698.
19.	Cebi N， Frömke J， Walterbusch G. Safe hemostasis by application of a new strict graft inclusion technique for replacement of the aortic root. Ann Thorac Surg， 2003， 76 （2）：631-632.
20.	Erkut B， Ceviz M， Becit N， et al. Pseudoaneurysm of the left coronary ostial anastomoses as a complication of the modified Bentall procedure diagnosed by echocardiography and multislice computed tomography. Heart Surg Forum， 2007， 10 （3）：E191-E192.
21.	Milano AD， Pratali S， Mecozzi G， et al. Fate of coronary ostial anastomoses after the modified Bentall procedure. Ann Thorac Surg， 2003， 75 （6）：1797-1801.
22.	符竣，陈绪发，陶凉，等．腔内吻合技术在Bentall手术中的应用．中国胸心血管外科临床杂志， 2011， 18 （6）：571-573．.
23.	Sun LZ， Qi RD， Chang Q， et al. Surgery for acute type A dissection using total arch replacement combined with stented elephant trunk implantation：experience with 107 patients. J Thorac Cardiovasc Surg， 2009， 138 （6）：1358-1362.
24.	Shimamura K， Kuratani T， Matsumiya G， et al. Long-term results of the open stent-grafting technique for extended aortic arch disease. J Thorac Cardiovasc Surg， 2008， 135 （6）：1261-1269.
25.	Fukunaga N， Shomura Y， Nasu M， et al. Aortic pseudoaneurysm detected on external jugular venous distention following a Bentall procedure 10 years previously. Interact Cardiovasc Thorac Surg， 2010， 11 （5）：673-674.
26.	Etz CD， Bischoff MS， Bodian C， et al. The bentall procedure：is it the Gold standard ? a series of 597 consecutive cases. J Thorac　 Cardiovasc Surg， 2010， 140 （6 Suppl）：S64-S70.
27.	Groenink M， Lohuis TA， Tijssen JG， et al. Survival and complication free survival in Marfan's syndrome：implications of current guidelines. Heart， 1999， 82 （4）：499-504.
28.	孙晓刚，常谦，李汉美，等. 马凡综合征主动脉根部手术后远端主动脉病变再次外科治疗. 中华胸心血管外科杂志， 2011， 27 （8）：452-455.
29.	Engelfriet PM， Boersma E， Tijssen JG， et al. Beyond the root：dilatation of the distal aorta in Marfan’s syndrome. Heart， 2006， 92 （9）：1238-1243.

1. 　Kim EK， Choi ER， Ko SM， et al. Comparison of aortic dissection in korean patients with versus without the marfan syndrome. Am J Cardiol， 2012， 109 （3）：423-427.
2. 　Linggen G， Lin Z， Xiaohan F， et al. Comparison of aortic dissection　 in Chinese patients with and without Marfan syndrome. Postgrad Med J， 2011， 87 （1027）：325-330.
3. 　Tsai TT， Evangelista A， Nienaber CA， et al. Long-term survival in patients presenting with type A acute aortic dissection. Insights from the international registry of acute aortic dissection （IRAD）.　 Circulation， 2006， 114 （1 Suppl）：I350-I356.
4. 　陈灏珠，王乐民，戍卫海，等. 主译. BRUANWALD心脏病学. 第7版. 北京：人民卫生出版社， 2007. 1776-1777.
5. 　Gilon D， Mehta RH， Oh JK， et al. Characteristics and in-hospital outcomes of patients with cardiac tamponade complicating type A acute aortic dissection. Am J Cardiol， 2009， 103 （7）：1029-1031.
6. 　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.
7. 　Tagusari O， Ogino H， Kobayashi J， et al. Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome ? J Thorac Cardiovasc Surg， 2004， 127 （5）：1373-1380.
8. 　Bachet J， Larrazet F， Goudot B， et al. When should the aortic arch be replaced in Marfan patients ? Ann Thorac Surg， 2007，83 （2）：S774-S790.
9. 　郑斯宏，孙衍庆，孟旭，等. 马凡综合征主动脉根部瘤手术治疗84例经验. 中华医学杂志， 2005， 85 （32）：2279-2282.
10. 　Galicia-Tornell MM， Marín-Solís B， Fuentes-Orozco C， et al. Bentall　 procedure in ascending aortic aneurysm：hospital mortality. Cir Cir， 2010， 78 （1）：45-51.
11. 　Guilmet D， Bonnet N， Saal JP， et al. Long term survival with the Bentall button operation in 150 patients. Arch Mal Coeur Vaiss， 2004， 97 （2）：83-91.
12. 　徐志云，邹良建，宋智钢，等．升主动脉根部瘤的外科治疗．中国胸心血管外科临床杂志， 2006， 13 （6）：374-377．.
13. 　Koshiyama H， Nakajima M， Amenomori S， et al. A refined flanged Bentall technique using valsalva tube graft for proximal reinforcement. Eur J Cardiothorac Surg， 2011， 40 （6）：1537-1539.
14. 　Chen LW， Dai XF， Wu XJ. A modified composite valve dacron graft for prevention of postoperative bleeding from the proximal anastomosis after Bentall procedure. Ann Thorac Surg， 2009， 88 （5）：1705-1707.
15. 　Bassano C， De Matteis GM， Nardi P， et al. Mid-term follow-up　 of aortic root remodeling compared to Bentall operation. Eur J　 Cardiothorac Surg， 2001， 19 （5）：601-605.
16. 　Joo HC， Chang BC， Youn YN， et al. Clinical experience with the Bentall procedure：28 years. Yonsei Med J， 2012， 53 （5）：915-923.
17. 　Alexiou C， Langley SM， Charlesworth P， et al. Aortic root replacement in patients with Marfan's syndrome：the Southampton experience. Ann Thorac Surg， 2001， 72 （5）：1502-1507.
18. 　Bortolotti U. Preventing bleeding from the proximal anastomosis after the Bentall procedure. Ann Thorac Surg， 2010， 90 （2）：698.
19. 　Cebi N， Frömke J， Walterbusch G. Safe hemostasis by application of a new strict graft inclusion technique for replacement of the aortic root. Ann Thorac Surg， 2003， 76 （2）：631-632.
20. 　Erkut B， Ceviz M， Becit N， et al. Pseudoaneurysm of the left coronary ostial anastomoses as a complication of the modified Bentall procedure diagnosed by echocardiography and multislice computed tomography. Heart Surg Forum， 2007， 10 （3）：E191-E192.
21. 　Milano AD， Pratali S， Mecozzi G， et al. Fate of coronary ostial anastomoses after the modified Bentall procedure. Ann Thorac Surg， 2003， 75 （6）：1797-1801.
22. 　符竣，陈绪发，陶凉，等．腔内吻合技术在Bentall手术中的应用．中国胸心血管外科临床杂志， 2011， 18 （6）：571-573．.
23. 　Sun LZ， Qi RD， Chang Q， et al. Surgery for acute type A dissection using total arch replacement combined with stented elephant trunk implantation：experience with 107 patients. J Thorac Cardiovasc Surg， 2009， 138 （6）：1358-1362.
24. 　Shimamura K， Kuratani T， Matsumiya G， et al. Long-term results of the open stent-grafting technique for extended aortic arch disease. J Thorac Cardiovasc Surg， 2008， 135 （6）：1261-1269.
25. 　Fukunaga N， Shomura Y， Nasu M， et al. Aortic pseudoaneurysm detected on external jugular venous distention following a Bentall procedure 10 years previously. Interact Cardiovasc Thorac Surg， 2010， 11 （5）：673-674.
26. 　Etz CD， Bischoff MS， Bodian C， et al. The bentall procedure：is it the Gold standard ? a series of 597 consecutive cases. J Thorac　 Cardiovasc Surg， 2010， 140 （6 Suppl）：S64-S70.
27. 　Groenink M， Lohuis TA， Tijssen JG， et al. Survival and complication free survival in Marfan's syndrome：implications of current guidelines. Heart， 1999， 82 （4）：499-504.
28. 　孙晓刚，常谦，李汉美，等. 马凡综合征主动脉根部手术后远端主动脉病变再次外科治疗. 中华胸心血管外科杂志， 2011， 27 （8）：452-455.
29. 　Engelfriet PM， Boersma E， Tijssen JG， et al. Beyond the root：dilatation of the distal aorta in Marfan’s syndrome. Heart， 2006， 92 （9）：1238-1243.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Surgical Outcomes of Patients with Marfan Syndrome Complicated by Type A Aortic Dissection

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