Case report

We present the case of a 44-year-old male patient admitted to hospital in January 2011 with retrosternal pain and hypertension. The patient was diagnosed with Marfan syndrome, characterised by an elongated skeleton, pectus deformity, and an ophthalmological disorder. Computed tomography angiography (CTA) revealed a type A aortic dissection, prompting emergency surgery to replace the aortic valve (mechanical) and the ascending aorta. In subsequent years, the patient developed a type II thoraco-abdominal aortic aneurysm following residual dissection, and open repair was indicated. Nevertheless, in January 2014, the aneurysm ruptured at the level of the thoracic aorta, necessitating urgent open repair with an interposition graft (30 mm tube graft) in the descending thoracic aorta via a left thoracotomy, Figure 1.

Figure 1 Computed tomography angiography revealing rupture of descending thoracic aorta in January 2014 (sagittal view) 

One year later, in July 2015, a multidisciplinary team performed an open thoraco-abdominal repair with an interposition aorto-bi-iliac graft and individual bypasses to the visceral and renal arteries, Figure 2.

Figure 2 Intra-operative image of the thoraco-abdominal interposition graft, with individual bypass to the visceral and renal arteries 

The patient recovered well from these procedures, but in September 2019, presented with thoracic pain and fever. Additionally, an abnormally elevated leucocyte count and increased C-reactive protein levels were identified, prompting further imaging that revealed a perigraft collection and air bubbles. Furthermore, a blood culture grew E. coli, and a positron emission tomography (PET) scan confirmed a diagnosis of prosthetic infection. Antibiotic therapy was initiated, stabilising the inflammatory/infectious process. During this period, progressive dilation of the last segment of the native aorta, the arch, was observed. The patient was discussed in a multidisciplinary aortic team, which decided that redo sternotomy and open repair of the arch carried prohibitive risk, and thus he was kept under conservative management.

However, the aortic arch kept enlarging, reaching 7cm, Figure 3. Since open surgery was not an option, a compassionate endovascular strategy was planned, knowing the risks of endovascular repair in a connective tissue patient and given the high risk of endograft infection.

The case was discussed with the COOK^® Planning Centre, and a custom-made arch endograft (COOK^® Zenith) with two inner branches (one antegrade for the left carotid and one retrograde for the left subclavian) was planned, Figure 4. The device was built with a short tip to pass the mechanical valve and relied on the previous surgical grafts as proximal and distal landing zones (graft-to-graft repair). The left carotid and left subclavian were planned as target vessels to ensure sufficient proximal sealing length in the remaining native aorta and ascending graft without covering the coronary ostia, which was not possible with an inner branch for the innominate artery.

Figure 3 Computed tomography angiography image of the infected aortic arch aneurysm (axial view) 

Figure 4 Graft plan - COOK^® Zenith Custom-made stent graft with two inner branches 

A staged repair was planned and performed, comprising cervical debranching followed by endovascular arch repair. Left carotid-to-right carotid and prosthetic-to-right subclavian bypasses were performed. Additionally, because the right vertebral artery originated directly from the arch, it was re-implanted onto the right common carotid artery.

The endovascular procedure was performed under general anaesthesia with fusion guidance. Access was obtained via surgical exposure of the right common femoral artery, the left common carotid artery, and the left brachial artery, together with a left percutaneous femoral artery access. The left ventricle was catheterised after passing the mechanical valve (bileaflet valve) through the side of one of the leaflets to avoid complete aortic insufficiency during endograft deployment (Figure 5). The main graft was then positioned through the mechanical valve and deployed under rapid pacing to reduce cardiac output. During this stage, after the device had been fully deployed under rapid pacing, the patient developed cardiorespiratory arrest (ventricular fibrillation), which was rapidly reversed with defibrillation without complications.

Figure 5 Intra-operative fluoroscopy image of the aortic arch procedure. 

The tip of the delivery system is seen crossing the aortic mechanical valve

Catheterisation of the antegrade inner-branch was performed via the carotid access, and a covered balloon-expandable stent was delivered (2x Bentley^® Begraft Aortic 12x59). The retrograde inner-branch was catheterised via the brachial access, and a through-and-through wire was established via the right femoral access. Due to the large size of the subclavian artery, covered balloon-expandable stents were used (2x Bentley^® Begraft Aortic 14x59), which were advanced over the through-and-through wire from the femoral access. The final angiogram showed aneurysm exclusion and patency of all devices. The patient was discharged after 30 days, and a control CTA showed aneurysm exclusion and patency of all stented target vessels and the cervical debranching. He was kept on lifelong antibiotic treatment with amoxicillin + clavulanic acid and doxycycline.

The patient continues outpatient follow-up with regular appointments every 3 to 6 months. A new CTA was performed at 1 month, 3 months, 6 months, and 1 year after the last surgery. Currently, the patient undergoes regular imaging follow-up annually, with stable infection control and no aortic complications, maintaining a good quality of life.