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Case Reports
. 2024 Jan 28;10(3):e25235.
doi: 10.1016/j.heliyon.2024.e25235. eCollection 2024 Feb 15.

Aortic arch rupture in a patient with Marfan syndrome and previous aortic root repair: A stepwise approach to intraoperative catastrophe

Talon Jones  1 , Joel Price  2 , Sean R McLean  1
Affiliations
Case Reports

Aortic arch rupture in a patient with Marfan syndrome and previous aortic root repair: A stepwise approach to intraoperative catastrophe

Talon Jones et al. Heliyon. .

Abstract

Marfan syndrome (MS) is an autosomal dominant connective tissue disease associated with significant morbidity and mortality due to progressive dilatation of the thoracic aorta which can lead to aortic rupture. Survival from an aortic rupture is predicated on immediate organized and goal directed care by both surgical and anesthesia teams. This case highlights how coordinated care from a cardiac operating room team, including early preparation of autologous blood products, expeditious placement of intravascular access for rapid high volume transfusion, and intentional communication between anesthesia, perfusion, surgery and nursing during the resuscitation in the OR, can all lead to an improved outcome.

Keywords: Aortic rupture; Cardiac anesthesia; Marfan syndrome; Massive transfusion; Resuscitation.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1
Fig. 1
CT Angiography demonstrating severe pectus excavatum, sternal wire from previous sternotomy, contained ascending aortarupture and associated left mainstem bronchus compression and small left pleural effusion.
Fig. 2
Fig. 2
CT Head- Postoperative day 3, demonstrates loss of grey-white differentiation consistent with severe hypoxic brain injury.
See this image and copyright information in PMC

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