Managing Anaesthesia Complexities in Ascending Aorta Surgeries in patients with Marfan Syndrome


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

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

Google Scholar : https://tinyurl.com/24pbj4t9

Scribd : https://tinyurl.com/3npz7a82

DOI : https://doi.org/10.5281/zenodo.14759712

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 over one 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.

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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 over one 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.

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