When Should the Aortic Arch Be Replaced in Marfan Patients?

Type A Aortic Dissection in Marfan Syndrome

“…5,14,20 Our study confirmed this hypothesis. Although aortic arch reintervention was necessary only in patients with initial hemi-arch replacement, elective arch reintervention carried an only moderate risk.…”

“…[14][15][16] However, this was a rather intuitive recommendation made according to experiences with nondissected patients with MFS because the number of patients with MFS with aortic dissection even at high-volume centers is very low, ranging from 3% to 5% of the entire dissection population. 1,10 Until now, no aortic root preservation outcomes in patients with MFS suffering acute dissection have been published.…”

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

“…However, if the surgical procedure is performed after a Type A dissection, the risk of reintervention increases (3% vs. 33%; P=0.0001). Furthermore, Bachet et al (7) found similar results, concluding that aortic arch replacement is not indicated during elective aortic root surgery. Nonetheless, over decades, the significant likelihood of aneurysmal dilatation in the remnant aortic arch after surgery for Type A aortic dissection is an incentive for considering an aggressive approach towards the aortic arch.…”

“…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).…”

Operação de Bentall e De Bono para correção das doenças da raiz aórtica: análise de resultados a longo prazo

“…Current studies has shown that, in the long term, the incidence of aortic insufficiency is low when we perform these new mentioned interventions [20].…”