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Mid-term outcomes of cardiovascular surgery for patients with Marfan syndrome

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Abstract

Objectives

Cardiovascular manifestations determine the prognosis and survival of patients with Marfan syndrome (MFS). We assessed the early and mid-term outcomes of cardiovascular surgery for this patient population.

Patients and methods

We conducted a retrospective evaluation of patients with MFS who underwent surgery in our department. The endpoint was the requirement for a second cardiovascular surgery, and death from any cause.

Results

From February 2002 to March 2013, we performed 24 surgeries on 13 patients with MFS. At the time of initial surgery, the patients’ aged ranged from 19 to 64 years of age (mean 34.7 ± 11.3 years) and included 5 men (38.5 %). Five of 13 initial surgeries (38.5 %) were emergencies. Seven patients (53.8 %) presented with aortic dissection at initial surgery, and one of the remaining six patients suffered from new aortic dissection during follow-up. Eight patients (61.5 %) underwent a second surgery, and a second surgery was not required for 100, 75.0, and 53.6 % of patients at 1, 3, and 5 years, respectively. The mean time interval between the first and the second surgeries was 52.8 ± 28.2 months. No patient died while hospitalized, and three subsequently died from unknown causes. Survival rates were 100, 90.9 and 64.9 % at 3, 5, and 7 years, respectively.

Conclusion

Although we obtained satisfactory early outcomes for patients with MFS, there were three late deaths from unknown causes. Therefore, we recommend that patients with MFS should remain under close surveillance.

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References

  1. Judge DP, Dietz HC. Marfan’s syndrome. Lancet. 2005;366:1965–76.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  2. Gott VL, Greene PS, Alejo DE, Cameron DE, Naftel DC, Miller DC, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med. 1999;340:1307–13.

    Article  CAS  PubMed  Google Scholar 

  3. David TE, Armstrong S, Maganti M, Colman J, Bradley TJ. Long-term results of aortic valve-sparing operations in patients with Marfan syndrome. J Thorac Cardiovasc Surg. 2009;138:859–64 discussion 63–4.

    Article  PubMed  Google Scholar 

  4. de Oliveira NC, David TE, Ivanov J, Armstrong S, Eriksson MJ, Rakowski H, et al. Results of surgery for aortic root aneurysm in patients with Marfan syndrome. J Thorac Cardiovasc Surg. 2003;125:789–96.

    Article  PubMed  Google Scholar 

  5. Schoenhoff FS, Jungi S, Czerny M, Roost E, Reineke D, Matyas G, et al. Acute aortic dissection determines the fate of initially untreated aortic segments in Marfan syndrome. Circulation. 2013;127:1569–75.

    Article  PubMed  Google Scholar 

  6. De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 1996;62:417–26.

    Article  PubMed  Google Scholar 

  7. Girdauskas E, Kuntze T, Borger MA, Falk V, Mohr FW. Distal aortic reinterventions after root surgery in Marfan patients. Ann Thorac Surg. 2008;86:1815–9.

    Article  PubMed  Google Scholar 

  8. Waterman AL, Feezor RJ, Lee WA, Hess PJ, Beaver TM, Martin TD, et al. Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg. 2012;55:1234–40 disucssion 40–1.

    Article  PubMed  Google Scholar 

  9. Mimoun L, Detaint D, Hamroun D, Arnoult F, Delorme G, Gautier M, et al. Dissection in Marfan syndrome: the importance of the descending aorta. Eur Heart J. 2011;32:443–9.

    Article  PubMed  Google Scholar 

  10. Sarsam MA, Yacoub M. Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg. 1993;105:435–8.

    CAS  PubMed  Google Scholar 

  11. David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992;103:617–21 discussion 22.

    CAS  PubMed  Google Scholar 

  12. Svensson LG, Deglurkar I, Ung J, Pettersson G, Gillinov AM, D’Agostino RS, et al. Aortic valve repair and root preservation by remodeling, reimplantation, and tailoring: technical aspects and early outcome. J Card Surg. 2007;22:473–9.

    Article  PubMed  Google Scholar 

  13. Yacoub MH, Gehle P, Chandrasekaran V, Birks EJ, Child A, Radley-Smith R. Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root. J Thorac Cardiovasc Surg. 1998;115:1080–90.

    Article  CAS  PubMed  Google Scholar 

  14. Settepani F, Szeto WY, Pacini D, De Paulis R, Chiariello L, Di Bartolomeo R, et al. Reimplantation valve-sparing aortic root replacement in Marfan syndrome using the Valsalva conduit: an intercontinental multicenter study. Ann Thorac Surg. 2007;83:S769–73 discussion S85–S90.

    Article  PubMed  Google Scholar 

  15. Kunihara T, Aicher D, Rodionycheva S, Asano M, Tochii M, Sata F, et al. Outcomes after valve-preserving root surgery for patients with Marfan syndrome. J Heart Valve Dis. 2012;21:615–22.

    PubMed  Google Scholar 

  16. Kallenbach K, Baraki H, Khaladj N, Kamiya H, Hagl C, Haverich A, et al. Aortic valve-sparing operation in Marfan syndrome: what do we know after a decade? Ann Thorac Surg. 2007;83:S764–8 discussion S85–S90.

    Article  PubMed  Google Scholar 

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Conflict of interest

All the authors have declared no competing interest.

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Authors and Affiliations

  1. Department of Cardiovascular Surgery, Fujita Health University, 1-98 Dengakugakubo, Kutsukake, Toyoake, Aichi, 470-1192, Japan

    Masato Tochii, Koji Hattori, Hiroshi Ishikawa, Michiko Ishida, Yoshiro Higuchi, Kentaro Amano, Yusuke Sakurai, Mika Noda, Takashi Watanabe & Yasushi Takagi

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  1. Masato Tochii
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  2. Koji Hattori
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  3. Hiroshi Ishikawa
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  4. Michiko Ishida
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  5. Yoshiro Higuchi
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  6. Kentaro Amano
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  7. Yusuke Sakurai
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  8. Mika Noda
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  9. Takashi Watanabe
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  10. Yasushi Takagi
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Correspondence to Masato Tochii.

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Tochii, M., Hattori, K., Ishikawa, H. et al. Mid-term outcomes of cardiovascular surgery for patients with Marfan syndrome. Gen Thorac Cardiovasc Surg 63, 267–272 (2015). https://doi.org/10.1007/s11748-014-0507-6

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  • DOI: https://doi.org/10.1007/s11748-014-0507-6

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