Skip to main content
SpringerLink
Log in
Book cover

Controversies in Aortic Dissection and Aneurysmal Disease pp 423–434Cite as

The Thoracoabdominal Aorta in Marfan Syndrome

  • Chapter
  • First Online:

Abstract

Although most aneurysms in patients with Marfan syndrome develop in the aortic root, aneurysms can also develop in the thoracoabdominal aorta. Most thoracoabdominal aortic aneurysms in these patients are caused by progressive aortic dilatation after aortic dissection. Many patients with Marfan syndrome who undergo successful repair of ascending aortic dissection subsequently suffer progressive expansion of the chronically dissected distal aorta. Among patients with Marfan syndrome and aortic dissection, those who undergo surgical repair of the thoracoabdominal aorta include patients with acute dissection and complications involving the thoracoabdominal segment, acute dissection superimposed on a preexisting thoracoabdominal aortic aneurysm, or chronic dissection that has led to a large or symptomatic thoracoabdominal aortic aneurysm. Contemporary surgical approaches balance the need for extensive aortic replacement with the need to protect the spinal cord and other organs and, thereby, prevent postoperative complications. Despite the extensive aortic replacement that typifies these procedures, early outcomes after these operations are excellent. In our contemporary series of 96 thoracoabdominal aortic aneurysm repairs in patients with Marfan syndrome, the early survival rate was 97 %, and major complications were rare: permanent spinal cord deficit occurred in one patient, stroke occurred in one patient, and permanent renal failure necessitating dialysis occurred in five patients. Recent literature indicates that long-term survival and freedom from repair failure are also excellent.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD   199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Finkbohner R, Johnston D, Crawford ES, Coselli J, Milewicz DM. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation. 1995;91:728–33.

    Article  CAS  PubMed  Google Scholar 

  2. LeMaire SA, Carter SA, Volguina IV, Laux AT, Milewicz DM, Borsato GW, et al. Spectrum of aortic operations in 300 patients with confirmed or suspected Marfan syndrome. Ann Thorac Surg. 2006;81:2063–78.

    Article  PubMed  Google Scholar 

  3. Song HK, Kindem M, Bavaria JE, Dietz HC, Milewicz DM, Devereux RB, et al. Long-term implications of emergency versus elective proximal aortic surgery in patients with Marfan syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry. J Thorac Cardiovasc Surg. 2012;143:282–6.

    Article  PubMed Central  PubMed  Google Scholar 

  4. Midulla M, Renaud A, Martinelli T, Koussa M, Mounier-Vehier C, Prat A, et al. Endovascular fenestration in aortic dissection with acute malperfusion syndrome: immediate and late follow-up. J Thorac Cardiovasc Surg. 2011;142:66–72.

    Article  PubMed  Google Scholar 

  5. Rolf T, Qanadli SD, Rey J, von Segesser LK. Intussusception like lesion after fenestration in aortic type B dissection. Interact Cardiovasc Thorac Surg. 2010;11:376–7.

    Article  PubMed  Google Scholar 

  6. Howell JF, LeMaire SA, Kirby RP. Thoracoabdominal fenestration for aortic dissection with ischemic colonic perforation. Ann Thorac Surg. 1997;64:242–4.

    Article  CAS  PubMed  Google Scholar 

  7. Pradhan S, Elefteriades JA, Sumpio BE. Utility of the aortic fenestration technique in the management of acute aortic dissections. Ann Thorac Cardiovasc Surg. 2007;13:296–300.

    PubMed  Google Scholar 

  8. Trimarchi S, Jonker FH, Muhs BE, Grassi V, Righini P, Upchurch GR, et al. Long-term outcomes of surgical aortic fenestration for complicated acute type B aortic dissections. J Vasc Surg. 2010;52:261–6.

    Article  PubMed  Google Scholar 

  9. Dardik A, Krosnick T, Perler BA, Roseborough GS, Williams GM. Durability of thoracoabdominal aortic aneurysm repair in patients with connective tissue disorders. J Vasc Surg. 2002;36:696–703.

    Article  PubMed  Google Scholar 

  10. Kalkat MS, Rahman I, Kotidis K, Davies B, Bonser RS. Presentation and outcome of Marfan’s syndrome patients with dissection and thoraco-abdominal aortic aneurysm. Eur J Cardiothorac Surg. 2007;32:250–4.

    Article  PubMed  Google Scholar 

  11. Mommertz G, Sigala F, Langer S, Koeppel TA, Mess WH, Schurink GW, et al. Thoracoabdominal aortic aneurysm repair in patients with Marfan syndrome. Eur J Vasc Endovasc Surg. 2008;35:181–6.

    Google Scholar 

  12. Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey Jr DE, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121:e266–369.

    Article  PubMed  Google Scholar 

  13. Johnston WF, Upchurch Jr GR, Tracci MC, Cherry KJ, Ailawadi G, Kern JA. Staged hybrid approach using proximal thoracic endovascular aneurysm repair and distal open repair for the treatment of extensive thoracoabdominal aortic aneurysms. J Vasc Surg. 2012;56:1495–502.

    Google Scholar 

  14. LeMaire SA, Green SY, Kim JH, Sameri A, Parenti JL, Lin PH, et al. Thoracic or thoracoabdominal approaches to endovascular device removal and open aortic repair. Ann Thorac Surg. 2012;93:726–32; discussion 733.

    Article  PubMed  Google Scholar 

  15. Dardik A, Perler BA, Roseborough GS, Williams GM. Aneurysmal expansion of the visceral patch after thoracoabdominal aortic replacement: an argument for limiting patch size? J Vasc Surg. 2001;34:405–9; discussion 410.

    Article  CAS  PubMed  Google Scholar 

  16. Schwill S, LeMaire SA, Green SY, Bakaeen FG, Coselli JS. Endovascular repair of thoracic aortic pseudoaneurysms and patch aneurysms. J Vasc Surg. 2010;52:1034–7.

    Article  PubMed  Google Scholar 

  17. de la Cruz KI, LeMaire SA, Weldon SA, Coselli JS. Thoracoabdominal aortic aneurysm repair with a branched graft. Ann Cardiothorac Surg. 2012;1:381–93.

    Google Scholar 

  18. De Rango P, Estrera AL, Miller III C, Lee TY, Keyhani K, Abdullah S, et al. Operative outcomes using a side-branched thoracoabdominal aortic graft (STAG) for thoraco-abdominal aortic repair. Eur J Vasc Endovasc Surg. 2011;41:41–7.

    Article  PubMed  Google Scholar 

  19. Kokotsakis J, Lazopoulos G, Ashrafian H, Misthos P, Athanasiou T, Lioulias A. Thoracoabdominal aneurysm repair using a four-branched thoracoabdominal graft: a case series. Cases J. 2009;2:7144.

    Article  PubMed Central  PubMed  Google Scholar 

  20. Kouchoukos NT, Masetti P, Castner CF. Use of presewn multiple branched graft in thoracoabdominal aortic aneurysm repair. J Am Coll Surg. 2005;201:646–9.

    Article  PubMed  Google Scholar 

  21. Kulik A, Castner CF, Kouchoukos NT. Patency and durability of presewn multiple branched graft for thoracoabdominal aortic aneurysm repair. J Vasc Surg. 2010;51:1367–72.

    Article  PubMed  Google Scholar 

  22. Bozinovski J, Coselli JS. Outcomes and survival in surgical treatment of descending thoracic aorta with acute dissection. Ann Thorac Surg. 2008;85:965–70; discussion 970–1.

    Article  PubMed  Google Scholar 

  23. Coselli JS, LeMaire SA, de Figueiredo LP, Kirby RP. Paraplegia after thoracoabdominal aortic aneurysm repair: is dissection a risk factor? Ann Thorac Surg. 1997;63:28–35; discussion 36.

    Article  CAS  PubMed  Google Scholar 

  24. Acher CW, Wynn MM, Hoch JR, Popic P, Archibald J, Turnipseed WD. Combined use of cerebral spinal fluid drainage and naloxone reduces the risk of paraplegia in thoracoabdominal aneurysm repair. J Vasc Surg. 1994;19:236–46.

    Article  CAS  PubMed  Google Scholar 

  25. LeMaire SA, Miller III CC, Conklin LD, Schmittling ZC, Coselli JS. Estimating group mortality and paraplegia rates after thoracoabdominal aortic aneurysm repair. Ann Thorac Surg. 2003;75:508–13.

    Article  PubMed  Google Scholar 

  26. Bhamidipati CM, Coselli JS, LeMaire SA. Perfusion techniques for renal protection during thoracoabdominal aortic surgery. J Extra Corpor Technol. 2012;44:P31–7.

    PubMed  Google Scholar 

  27. Coselli JS, LeMaire SA. Left heart bypass reduces paraplegia rates after thoracoabdominal aortic aneurysm repair. Ann Thorac Surg. 1999;67:1931–4.

    Article  CAS  PubMed  Google Scholar 

  28. Coselli JS, LeMaire SA. Tips for successful outcomes for descending thoracic and thoracoabdominal aortic aneurysm procedures. Semin Vasc Surg. 2008;21:13–20.

    Article  PubMed  Google Scholar 

  29. Coselli JS, LeMaire SA, Köksoy C, Schmittling ZC, Curling PE. Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial. J Vasc Surg. 2002;35:631–9.

    Article  PubMed  Google Scholar 

  30. Köksoy C, LeMaire SA, Curling PE, Raskin SA, Schmittling ZC, Conklin LD, et al. Renal perfusion during thoracoabdominal aortic operations: cold crystalloid is superior to normothermic blood. Ann Thorac Surg. 2002;73:730–8.

    Article  PubMed  Google Scholar 

  31. LeMaire SA, Jones MM, Conklin LD, Carter SA, Criddell MD, Wang XL, et al. Randomized comparison of cold blood and cold crystalloid renal perfusion for renal protection during thoracoabdominal aortic aneurysm repair. J Vasc Surg. 2009;49:11–9; discussion 19.

    Article  PubMed  Google Scholar 

  32. Vaughn SB, LeMaire SA, Collard CD. Case scenario: anesthetic considerations for thoracoabdominal aortic aneurysm repair. Anesthesiology. 2011;115:1093–102.

    Article  PubMed  Google Scholar 

  33. Coselli JS, LeMaire SA, Carter SA, Conklin LD. The reversed elephant trunk technique used for treatment of complex aneurysms of the entire thoracic aorta. Ann Thorac Surg. 2005;80:2166–72.

    Article  PubMed  Google Scholar 

  34. LeMaire SA, Jamison AL, Carter SA, Wen S, Alankar S, Coselli JS. Deployment of balloon expandable stents during open repair of thoracoabdominal aortic aneurysms: a new strategy for managing renal and mesenteric artery lesions. Eur J Cardiothorac Surg. 2004;26:599–607.

    Article  PubMed  Google Scholar 

  35. Coselli JS, Bozinovski J, Cheung C. Hypothermic circulatory arrest: safety and efficacy in the operative treatment of descending and thoracoabdominal aortic aneurysms. Ann Thorac Surg. 2008;85:956–63; discussion 964.

    Article  PubMed  Google Scholar 

  36. LeMaire SA, Miller III CC, Conklin LD, Schmittling ZC, Köksoy C, Coselli JS. A new predictive model for adverse outcomes after elective thoracoabdominal aortic aneurysm repair. Ann Thorac Surg. 2001;71:1233–8.

    Article  CAS  PubMed  Google Scholar 

  37. Wong DR, Parenti JL, Green SY, Chowdhary V, Liao JM, Zarda S, et al. Open repair of thoracoabdominal aortic aneurysm in the modern surgical era: contemporary outcomes in 509 patients. J Am Coll Surg. 2011;212:569–79; discussion 579–81.

    Article  PubMed  Google Scholar 

  38. Coselli JS, Bozinovski J, LeMaire SA. Open surgical repair of 2286 thoracoabdominal aortic aneurysms. Ann Thorac Surg. 2007;83:S862–4.

    Article  PubMed  Google Scholar 

  39. Creager MA, Dzau VS, Loscalzo J, editors. Vascular medicine. Philadelphia: WB Saunders; 2006.

    Google Scholar 

Download references

Acknowledgments

The authors thank Matt D. Price, MS, RHIA, Samantha Zarda, RHIA, Michael S. Hughes, and Jeffrey Whorton, RHIA, for clinical data management; Stephen N. Palmer, PhD, ELS, and Susan Y. Green, MPH, for editorial support; and Scott A. Weldon, MA, CMI, and Carol P. Larson, CMI, for creating the medical illustrations.

Disclosure

Dr Coselli serves as a consultant for Vascutek Ltd., a subsidiary of Terumo Corporation, and receives royalties related to Vascutek® Gelweave™ Coselli thoracoabdominal grafts.

Author information

Authors and Affiliations

  1. Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, One Baylor Plaza, BCM 390, Houston, TX, 77030, USA

    Scott A. LeMaire MD, Kim I. de la Cruz MD & Joseph S. Coselli MD

  2. Department of Cardiovascular Surgery, The Texas Heart Institute, Houston, TX, USA

    Scott A. LeMaire MD, Kim I. de la Cruz MD & Joseph S. Coselli MD

Authors
  1. Scott A. LeMaire MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kim I. de la Cruz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Joseph S. Coselli MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Scott A. LeMaire MD .

Editor information

Editors and Affiliations

  1. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Robert S. Bonser

  2. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Domenico Pagano

  3. Thoracic and Cardiovascular Surgery Depts., Hannover Medical School, Hannover, Germany

    Axel Haverich

  4. University Hospitals Birmingham, Queen Elizabeth Hospital & Centre, Birmingham, United Kingdom

    Jorge Mascaro

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this chapter

Cite this chapter

LeMaire, S.A., de la Cruz, K.I., Coselli, J.S. (2014). The Thoracoabdominal Aorta in Marfan Syndrome. In: Bonser, R., Pagano, D., Haverich, A., Mascaro, J. (eds) Controversies in Aortic Dissection and Aneurysmal Disease. Springer, London. https://doi.org/10.1007/978-1-4471-5622-2_37

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-5622-2_37

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-5621-5

  • Online ISBN: 978-1-4471-5622-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation