An 80-year-old male patient suffering from hypertension and stage 3b chronic renal failure was admitted to the emergency department of another hospital for chest pain. His creatinine level was 1.6 md/dL. Total body contrast enhanced computed tomography (CT) showed impending rupture of an aortic arch pseudoaneurysm, originating in zone 2 extending posteriorly and cranially and adhering to the posterior aspect of the brachiocephalic trunk (BCT), in a gothic type III arch configuration. CT scan also showed a 5 cm ascending aortic aneurysm, and a voluminous suprarenal abdominal aortic aneurysm with maximum diameter 8 cm, celiac tripod and inferior mesenteric artery occlusion, renal arteries and superior mesenteric artery patency, and thrombotic apposition of the infrarenal abdominal aorta down to the iliac bifurcation (see Fig. 1).

Preoperative CT-scan. On the left, the tridimensional reconstruction (the red box highlights the arch pseudoaneurysm; the blue box the supra-renal abdominal aortic aneurysm; the green box the thrombosed dilated infrarenal segment of the abdominal aorta). In the center, three axial images corresponding to the three pathological segments. On the right, schematic drawing of the patient’s aorta before operation

A less invasive approach like total endovascular arch repair by means of a branched/fenestrated endovascular graft (EVG) or SAV by-passes prior to EVG was not possible since an adequate proximal landing zone was lacking (ascending aortic aneurysm). In such a frail patient, a traditional open surgical arch repair performed with CA and distal anastomosis of the vascular prosthesis in Ishimaru zone 3 would have been burdened by high morbidity and mortality risks, mainly related to the higher splanchnic ischemic risk (being the whole splanchnic district receiving blood only from the superior mesenteric artery) and to a possible worsening of renal failure after CA.

Surgery was performed at the Cardiac Surgery Unit of Monaldi Hospital in Naples, Italy, in a hybrid operative room with a mobile fluoroscopy C-arm. Cerebal perfusion and oxygenation was monitored by means of near infrared spectroscopy (NIRS), whereas continuous arterial pressure monitoring was obtained from catheters into left radial, right radial and left femoral arteries. Median sternotomy allowed for heart, aorta and SAV exposition. Only the left subclavian artery origin was markedly posterior in the aortic arch, hampering intra-thoracic access for its revascularization. Thereafter, by bilateral subclavian incisions, left and right subclavian arteries were isolated; left subclavian offspring was then marked with large hemoclips. A right groin incision was used to access right common femoral artery.

A dedicated ECC circuit was created, using 4 Y connectors and 8 tube segments (all with the same diameter) as shown in Fig. 2: after systemic heparinization, 3 lines were connected to the supra-aortic branches. In the meanwhile, under fluoroscopic guide, a Reliant balloon (Medtronic, USA) was driven into TDA percutaneously, while trans-esophageal echocardiography (TEE) ascertained that the target descending aortic segment was free from dilative/atherosclerotic disease. Just distal to the balloon site, a long Revas 18 Fr perfusion cannula (Eurosets, Italy) was positioned through the right femoral artery to allow antegrade perfusion in the TDA and connected to the fourth branch of the customized circuit (Fig. 2). Antegrade flow in the descending aorta was necessary in this patient to avoid the risk of embolization of clot material from the distal abdominal aorta (where parietal thrombosis was present) to the superior mesenteric artery on which the whole splanchnic perfusion depended. The fifth line was left clamped at this stage of the procedure. The Right atrium was cannulated with a double-stage cannula and connected to the venous line for cardio-pulmonary bypass. ECC was then started, by opening the arterial inflows to the three SAVs (inflows 1, 2 and 3; see Fig. 2). A sump sucker was inserted into the right superior pulmonary vein.

Schematic drawing depicting the customization of a multi-branched arterial line with 5 inflow branches. One was temporarily clamped to be used only after the distal anastomosis (inflow 5); 3 of the other 4 served for perfusion of the SAVs and 1 for TDA antegrade perfusion. Note that with this system, all lines derived from a single centrifugal pump. Two 8-mm prosthetic tubular grafts were anastomosed in termino-lateral fashion to the right and left subclavian arteries and connected to inflows 1 and 2 of the ECC circuit. The left carotid artery was clamped at its origin to check for good left hemisphere NIRS: then it was ligated and transected, then another 8-mm prosthetic tubular graft was anastomosed in termino-terminal fashion to its distal stump and connected to inflow 3. Under fluoroscopic guide, a Reliant balloon (Medtronic, USA) was driven into TDA percutaneously through a 12 Fr introducer sheath from the left femoral artery: a centimeter-marked pigtail catheter allowed for correct positioning of the balloon at 15 cm from the previously marked left subclavian offspring. A long Revas 18 Fr perfusion cannula (Eurosets, Italy) was positioned right below the balloon through the right femoral artery to allow antegrade perfusion in the TDA. This cannula was then connected to inflow 4 (clamped until the balloon was inflated after cardioplegic arrest)

When mild hypothermia (33° C) was achieved, ascending aorta was cross-clamped and Del Nido antegrade cardioplegia administered into the aortic root. Once the heart was arrested, the ascending aorta was unclamped, the BCT and left subclavian artery were clamped at their origin, the TDA endo-clamped by inflating the balloon, and the femoral inflow line (inflow 4, Fig. 2) unclamped: thus, the dilated ascending aorta could be resected and the arch opened in a bloodless operative field, maintaining the patient in ECC and avoiding CA. In this setting, flow distribution was subject to auto-regulation, as for a standard ECC: a deeper hypothermia would have hampered native autoregulatory mechanisms. A Thoraflex hybrid prosthesis (Terumo Aortic, Scotland) 30/40 mm, 100 mm length of the stented portion, with three single branches for SAV re-implantation (12 mm, 10 mm, and 8 mm) and a lateral branch (10 mm) for reperfusion was implanted: the stented portion was released into the aortic arch, thus excluding the pseudoaneurysm, with a distal sealing in zone 3 and the vascular prosthetic collar anastomosed in zone 0 to the aortic arch stump reinforced by Teflon strips. Throughout the period of distal anastomosis, the position of the endoclamp was kept under TEE control (the balloon catheter was fixed outside of the femoral access to avoid dislocation). Once completed the distal anastomosis, systemic perfusion was continued through inflows 1, 2, 3 and 5, clamping inflow 4, deflating the balloon, and removing it, as well as the cannula, from the TDA.

The vascular prosthesis was then anastomosed at the sino-tubular junction, de-aired and unclamped. The BCT and the tube graft segment used for left carotid artery perfusion were anastomosed to the first two branches of the prosthesis in a termino-lateral (with side clamping of the BCT) and termino-terminal fashion, respectively (Fig. 3), with the heart beating. ECC was discontinued. The tubular prosthesis previously anastomosed to the left subclavian artery was tunneled into the mediastinum and then anastomosed to the third dedicated branch of the vascular prosthesis (Fig. 3). After protamine administration, BCT and left subclavian artery origins were closed with sutures. Figure 4 shows the final configuration, from 3D reconstruction of postoperative angio-CT-scan. Total ECC time was 203 min, aortic cross clamping time was 84 min. Postoperative course was uneventful and patient was transferred to rehab on postoperative day 18 (creatinine level at discharge from our ward was 1.5 mg/dL).

Schematic drawing depicting the completion phase of the operation: with the FET performed in zone 0, the fifth branch of the arterial line was connected to the lateral branch of the prosthesis (inflow 5). During anastomoses of the SAVs, systemic perfusion was continued through lines 1, 2, 3 and 5, after clamping the tubular vascular prosthesis, deflating the balloon, clamping inflow line 4 and removing the femoral cannula. Note that the native origins of all 3 SAVs are ligated. During weaning from CPB, the only line used is inflow 5

Tridimensional reconstruction of postoperative CT-scan showing complete coverage of the arch and debranching of SAVs from zone 0