Two-Stage Elephant Trunk approach for open management of distal aortic arch and descending aortic pathology in patients with Marfan syndrome

Open surgical treatment of an aortic pathology (aneurysm or dissection) afflicting the entire length of the thoracic aorta is a surgical challenge (1). Therefore, a two-stage procedure for “mega-aorta” syndrome was instituted to address this surgical dilemma (2). However, Borst and colleagues observed that with the classical technique (conventional arch replacement followed by descending aortic replacement), mobilization of the previous graft at the distal arch anastomosis was cumbersome and could result in injury to the pulmonary artery, the aorta itself, the esophagus, and other surrounding structures (3). Therefore, in 1992 they introduced the Elephant Trunk technique, which was later modified by Crawford and Svensson (4). Connective tissue disorders such as Marfan syndrome increase the complexity of management of aortic arch and descending aortic pathology. This autosomal dominant disease is characterized by the presence of cardiovascular disorders affecting mostly the thoracic aorta with an associated decrease in life expectancy (5). The weak tissues and characteristic distal progression of aneurysmal disease in Marfan syndrome have led to the recommendation for aggressive early surgical intervention in the ascending aorta and preemptive aortic arch replacement when a Type A dissection is the presenting event (6,7). The two-stage Elephant Trunk technique fits into this aggressive paradigm and facilitates the correction of coexistent or subsequent descending aortic dilatation (5). In this paper, we describe the technical essentials of both stages of the Elephant Trunk procedure as practiced extensively at our institution.