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

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.