The Journal of Thoracic and Cardiovascular Surgery
Volume 137, Issue 6 , Pages 1327-1333 , June 2009

Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome

  • Melanie D. Everitt, MD

      Affiliations

    • Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
    • ,
  • Nelangi Pinto, MD

      Affiliations

    • Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
    • ,
  • John A. Hawkins, MD

      Affiliations

    • Intermountain West Marfan Cardiology Center, Division of Cardiovascular Surgery at Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
    • ,
  • Max B. Mitchell, MD

      Affiliations

    • The Children's Hospital, University of Colorado, Denver, Colo
    • ,
  • Peter C. Kouretas, MD

      Affiliations

    • Intermountain West Marfan Cardiology Center, Division of Cardiovascular Surgery at Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
    • ,
  • Anji T. Yetman, MD

      Affiliations

    • Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
    • The Children's Hospital, University of Colorado, Denver, Colo
    • Corresponding Author InformationAddress for reprints: Anji T. Yetman, MD, Professor of Pediatrics, Director, Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, 100 N. Mario Capecchi Drive, Salt Lake City, UT 84113.

Received 19 June 2008 ,Revised 27 September 2008 ,Accepted 1 February 2009.

References 

  1. Silverman DI, Burton KJ, Gray J, Bosner MS, Kouchoukos NT, Roman MJ, et al. Life expectancy in the Marfan syndrome. Am J Cardiol. 1995;75:157–160
  2. Van Karnebeek CD, Naeff MS, Mulder BJ, Hennekam RC, Offringa M. Natural history of cardiovascular manifestations in Marfan syndrome. Arch Dis Child. 2001;84:129–137
  3. Bentall H, de Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968;23:338–339
  4. 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;115:1080–1090
  5. David TE, Feindel CM, Webb GD, Colman JM, Armstrong S, Maganti M. Aortic valve preservation in patients with aortic root aneurysm: results of the reimplantation technique. Ann Thorac Surg. 2007;83:732–735
  6. 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;340:1307–1313
  7. Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788–798
  8. Williams JA, Loeys BL, Nwakanma LU, Dietz HC, Spevak PJ, Cameron DE, et al. Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease. Ann Thorac Surg. 2007;83:757–763
  9. Gillinov AM, Zehr KJ, Redmond M, Gott VL, Deitz HC, Cameron DE, et al. Cardiac operations in children with Marfan's syndrome: indications and results. Ann Thorac Surg. 1997;64:1140–1145
  10. Vricella LA, Williams JA, Ravekes WJ, Holmes KW, Dietz HC, Gott VL, et al. Early experience with valve-sparing root replacement in children. Ann Thorac Surg. 2005;80:1622–1626
  11. Carrel T, Berdat P, Pavlovic M, Sukhanov S, Englberger L, Pfammatter JP. Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur J Cardiothorac Surg. 2003;24:249–254
  12. De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 1996;62:417–426
  13. Sluysmans T, Colan SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol. 2005;99:445–457
  14. Gott VL, Pyeritz RE, Cameron DE, Green PS, McKusick VA. Composite graft repair of Marfan aneurysm of the ascending aorta: results in 100 patients. Ann Thoracic Surg. 1991;52:38–45
  15. 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;125:789–796
  16. De Paulis R, De Matteis GM, Nardi P, Scaffa R, Buratta MM, Chiariello L. Opening and closing characteristics of the aortic valve after valve-sparing procedures using a new aortic root conduit. Ann Thorac Surg. 2001;72:487–494
  17. Cameron DE, Vricella L. Valve-sparing aortic root replacement with the Valsalva graft. Oper Tech Thorac Cardiovas Surg. 2005;10:259–271
  18. Cameron DE. Mitral valve surgery in children with the Marfan syndrome. Prog Pediatr Cardiol. 1996;5:205–210
  19. Kaplan EL, Meier P. Non-parametric estimation from incomplete observations. J Am Stat Soc. 1958;53:457–481
  20. Bethea BT, Fitton TP, Alejo DE, Barreiro CJ, Cattaneo SM, Dietz HC, et al. Results of aortic valve-sparing operations: experience with remodeling and reimplantation procedures in 65 patients. Ann Thorac Surg. 2004;78:767–772
  21. Patel ND, Williams JA, Barreiro CJ, Bethea BT, Fitton TP, Dietz HC, et al. Valve-sparing aortic root replacement: early experience with the De Paulis Valsalva graft in 51 patients. Ann Thorac Surg. 2006;82:548–553
  22. Fuzellier JG, Chauvaud SM, Fornes P, Berrebi AJ, Lajos PS, Bruneval P, et al. Surgical management of mitral regurgitation associated with Marfan's syndrome. Ann Thorac Surg. 1998;66:68–72
  23. Yetman AT. Cardiovascular pharmacotherapy in patients with Marfan syndrome. Am J Cardiovasc Drugs. 2007;7:117–126
  24. Matt P, Habashi J, Carrel T, Cameron DE, Van Eyk JE, Dietz HC. Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan?. J Thorac Cardiovasc Surg. 2008;135:389–394

PII: S0022-5223(09)00163-9

doi: 10.1016/j.jtcvs.2009.02.007

The Journal of Thoracic and Cardiovascular Surgery
Volume 137, Issue 6 , Pages 1327-1333 , June 2009

Article Tools

* Image Usage & Resolution