Graft-to-graft endovascular aortic arch repair in a Marfan patient – a case report

Resumo

BACKGROUND: Patients with Marfan syndrome have a high risk of aneurysms or dissection of different segments of the aorta, representing a challenge in their treatment, as well as in the management of associated complications.

CASE REPORT: We present a 44- year- old patient with Marfan syndrome who had an acute type A dissection in January 2011 and underwent replacement of the aortic valve (mechanical) and the ascending aorta.
During follow- up, the patient developed an extent II thoracoabdominal aneurysm in the distal residual dissection area, which ruptured in the descending thoracic aorta in January 2014. Urgent open aortic repair was performed, with an interposition graft of the descending thoracic aorta.
In July 2015, he underwent replacement of the remainder of the thoraco- abdominal aorta with individual bypasses to the visceral and renal arteries.
In September 2019, the diagnosis of prosthetic infection led to multiple hospitalisations, necessitating prolonged antibiotic therapy. Although the inflammatory/infectious process was controlled, the aortic arch remained the last segment requiring intervention due to progressive aneurysmal dilation.
Given a patient with multiple interventions and a latent infection, he was refused open repair of the aortic arch and thus proposed for endovascular repair as a last option. We aimed to use the previous surgical grafts as proximal and distal landing zones (graft- to- graft repair). To achieve sufficient proximal sealing length, we performed a left carotid- to- right carotid and right subclavian bypass, vertebral artery re- implantation (direct arch origin), and used the left common carotid and left subclavian artery as target vessels for an arch endograft (COOK® a- branch, CMD platform). The graft was designed with two inner branches (one antegrade for the left carotid and one retrograde for the left subclavian). The graft was placed with the nose tip advancing through the mechanical aortic valve, achieving technical and clinical success.

CONCLUSIONS: Patients with Marfan syndrome are frequently affected by extensive post- dissection aortic aneurysms. Aortic replacement by traditional surgery yields good long- term results, but an endovascular approach may be the solution in cases that would otherwise be considered untreatable.