Managing Complexities in Ascending Aorta Surgeries in Patients with Marfan Syndrome – A Case Series


Authors : Dr. Sandeep More; Dr. Prerana Dixit; Dr. Sanjeeta Umbarkar

Volume/Issue : Volume 10 - 2025, Issue 1 - January

Google Scholar : https://tinyurl.com/nhfpaduz

Scribd : https://tinyurl.com/ymhkyxwj

DOI : https://doi.org/10.5281/zenodo.14716982

Abstract : Connective tissue disorders like Marfan’s Syndrome, Loeyz-Dietz syndrome and Ehlers-Danlos syndrome are associated with variable degrees of annulo- aortic ectasia and cystic medial necrosis of the aortic wall. These patients present to cardiac surgical units with a spectrum of disorders ranging from aortic insufficiency, aortic root dilatation, ascending aortic aneurysm and dissection and pose unique anaesthetic challenges for the cardiac anaesthesiologist. These cases need a systematic, multidisciplinary, case-based understanding for the perioperative management in view of increasing prevalence of aortic root disorders. We came across 25 cases for ascending aorta repair surgeries in the past 1 year at our tertiary cardiac care centre, out of which we present here a case series of four patients who had associated Marfan Syndrome as diagnosed using the Ghent criteria. Through this series, we want to emphasize on a thorough understanding of various aspects of anaesthesia management including preoperative optimization, positioning, intraoperative monitoring and care as well as post-operative complications expected in this patient subset.

Keywords : Marfan’s Syndrome, Connective Tissue Disorders, Aortic Root Disorders, Ascending Aorta Aneurysm, Aortic Dissection, Bentall Procedure.

References :

  1. Gaubitz M. Epidemiology of connective tissue disorders. Rheumatology. 2006 Oct 1;45(suppl_3):iii3-4.
  2. Salik I, Rawla P. Marfan Syndrome. [Updated 2023 Jan 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537339
  3. Schlatmann TJ, Becker AE. Pathogenesis of dissecting aneurysm of aorta: comparative histopathologic study of significance of medial changes. The American journal of cardiology. 1977 Jan 1;39(1):21-6.
  4. Pooja Kamat, Govinda Innani. Anaesthesia Management in a case of Marfans syndrome for Adenotonsillectomy: A case report. MedPulse International Journal of Anesthesiology. May 2022; 22(2):55-57
  5. Marfan.org/dx/rules
  6. Kouchoukos Nicholas, Blackstone Eugene, Hanley Frank, Kirklin James. "chronic thoracic and thoracoabdominal aortic disease" Cardiac Surgery, Kirklin, Baratt/Boyes 4th Edition, Elsevier Saunders, 2013, pp 973-1024
  7. Patel Prakash, Augoustides John, “Thoracic aorta” Cardiac Anaesthesia for Cardiac and Noncardiac surgery, Kaplan Joel, 7th Edition, Elsevier, 2017, pp 843 to 882
  8. Yamaguchi, Yoshikazu, Walter Samora, Jan E. Klamar, Dmitry Tumin, & Joseph D. Tobias. "Anesthetic Management of an Adolescent With Marfan Syndrome During Anterior Spinal Fusion." Journal of Medical Cases [Online], 9.11 (2018): 366-370. Web. 20 Aug. 2024
  9. Maddalo S, Beller J, DeAnda A. A Bentall is not a Bentall is not a Bentall: the evolution of aortic root surgery. Aorta. 2014 Oct;2(05):169-78
  10. Cherry C, DeBord S, Hickey C. The modified Bentall procedure for aortic root replacement. AORN journal. 2006 Jul;84(1):51-70.
  11. Malfait F, De Paepe A. Bleeding in the heritable connective tissue disorders: mechanisms, diagnosis and treatment. Blood Rev. 2009 Sep;23(5):191-7. doi: 10.1016/j.blre.2009.06.001. Epub 2009 Jul 9. PMID: 19592142
  12. Roberts A, Duncan EC, Hargrave P, Kingery DR, Barnes J, Horstemeyer DL, Stahl RF. Complications of Cardiopulmonary Bypass From an Anesthesia Perspective: A Clinical Review. HCA Healthc J Med. 2023 Feb 28;4(1):13-21. doi: 10.36518/2689-0216.1525. PMID: 37426558; PMCID: PMC10327958.
  13. Dorotta I, Kimball-Jones P, Applegate R 2nd. Deep hypothermia and circulatory arrest in adults. Semin Cardiothorac Vasc Anesth. 2007 Mar;11(1):66-76. doi: 10.1177/1089253206297482. PMID: 17484175.
  14. Ghoreishi M, Chahal D, Shah A, Kang J, Hirsch J, Tran D, McCloskey D, Shkullaku M, Gupta A, Strauss ER, Dahi S. First-in-Human Endovascular Aortic Root Repair (Endo-Bentall) for Acute Type A Dissection. Circulation: Cardiovascular Interventions. 2023 Oct;16(10):e013348.

Connective tissue disorders like Marfan’s Syndrome, Loeyz-Dietz syndrome and Ehlers-Danlos syndrome are associated with variable degrees of annulo- aortic ectasia and cystic medial necrosis of the aortic wall. These patients present to cardiac surgical units with a spectrum of disorders ranging from aortic insufficiency, aortic root dilatation, ascending aortic aneurysm and dissection and pose unique anaesthetic challenges for the cardiac anaesthesiologist. These cases need a systematic, multidisciplinary, case-based understanding for the perioperative management in view of increasing prevalence of aortic root disorders. We came across 25 cases for ascending aorta repair surgeries in the past 1 year at our tertiary cardiac care centre, out of which we present here a case series of four patients who had associated Marfan Syndrome as diagnosed using the Ghent criteria. Through this series, we want to emphasize on a thorough understanding of various aspects of anaesthesia management including preoperative optimization, positioning, intraoperative monitoring and care as well as post-operative complications expected in this patient subset.

Keywords : Marfan’s Syndrome, Connective Tissue Disorders, Aortic Root Disorders, Ascending Aorta Aneurysm, Aortic Dissection, Bentall Procedure.

Video Explanation for Published paper

Never miss an update from Papermashup

Get notified about the latest tutorials and downloads.

Subscribe by Email

Get alerts directly into your inbox after each post and stay updated.
Subscribe
OR

Subscribe by RSS

Add our RSS to your feedreader to get regular updates from us.
Subscribe