A 64-year-old woman presented to the emergency department with repeated “bleeding in her neck” for 2 years. She was hemodynamically stable with no active signs of infection. She reported no other symptoms, including those of heart failure. She was heavily sedentary and engaged in self-limitation. On physical examination, there was a small yet actively bleeding cutaneous orifice in the suprasternal notch. Additionally, she had a 2/6 systolic crescendo-decrescendo murmur on auscultation.

• Innovative procedures developed through solid industry partnerships may allow previously undertreated patients to receive appropriate care if they are carefully selected and procedures are carefully planned and performed.

• The ascending aorta is one of the last frontiers for endovascular and catheter-based management.

The patient had undergone conventional surgical aortic valve replacement in 2014. A bioprosthetic Carpentier-Edwards Perimount Magna 23-mm aortic xenograft (Edwards Lifesciences, Irvine, California) was implanted then after a diagnosis of severe aortic stenosis. Symptoms improved from New York Heart Association (NYHA) functional class IV before surgery to NYHA functional class I afterwards. The patient had been lost to follow-up since. The patient presented at the authors’ institution with visible skin bleeding arising from the suprasternal notch.

A computed tomography (CT) angiogram revealed a pseudoaneurysm in the suture line of the ascending aorta. The pseudoaneurysm had, over time, fistulized with the skin, leading to the bleeding orifice. The same test results showed a severely calcified porcelain aorta. Echocardiography was ordered, given the murmur, and showed a 28-mm Hg mean gradient with reduced leaflet mobility. Further evaluation included a coronary angiogram, which showed no lesions in the coronary arteries, and carotid and vertebral Doppler ultrasonograms which showed no atherosclerosis.

Management

Initially, percutaneous closure of the pseudoaneurysm was attempted, using Amplatzer vascular occluders (Abbott Laboratories, Lake Bluff, Illinois). In total, 7 devices were deployed inside the lesion without success (Figure 1). Given the porcelain aorta and a EuroSCORE of 28.5%, conventional surgery was contraindicated by multiple surgical teams.

Figure 1 Pre-Operative Computed Tomography Three-dimensional reconstruction demonstrates an ascending aortic pseudoaneurysm, porcelain aorta, and multiple vascular plugs.

The case was brought to the institutional heart team, to whom the authors proposed a compassionate and completely endovascular procedure that was able to simultaneously treat the failing aortic valve and the pseudoaneurysm in the ascending aorta, mimicking a Bentall-De Bono procedure, dubbed the Endo-Bentall procedure (Figure 2). The patient provided informed consent, and ethical approval of the Institutional Review Board was obtained.

Figure 2 Endo-Bentall Prosthesis Concept Illustrated

A custom-made device was designed (Figure 3), based on the patient’s CT, including in its proximal segment a balloon-expandable transcatheter prosthesis connected to a self-expandable aortic stent. The transcatheter valve is a modified 22-mm Braile Inovare (Braile Biomedica, São José do Rio Preto, Brazil), with a lozenge chromium-cobalt frame, 20 mm in height, with 3 radiopaque markers identifying the base, valve, and skirt, and a single sheet of bovine pericardium (1).

Figure 3 Endo-Bentall Prosthesis (A) Prosthesis design with coronary branches and key reference markers. (B) Prosthesis final characteristics.

The main stent body had 2 branches positioned with internal and external segments, in such a way that after its release, the branches were 2 cm to the coronary ostia. At this height, the graft is reduced in diameter to the same size of the transcatheter aortic valve replacement (TAVR) (22 mm), thus providing additional space for coronary access and bridging stent deployment.

The diameter of the prosthesis at the distal ascending aorta segment was 40 mm, considering an oversize of approximately 20% (33 mm distal aorta). The distance between the sinus tubular junction (STJ) and the brachiocephalic trunk was approximately 6.3 cm, allowing a prosthetic segment length of 34 mm × 40 mm in diameter, to be used to reduce the endoleak risk. Additionally, a proprietary Proseal (Braile Biomedical) sealing was applied to the distal prosthesis segment. The defect was a circular-shaped tear located 6 mm below the STJ, originating posteriorly near the noncoronary sinus and extending anteriorly and superiorly. Distance from the right coronary artery to the defect was just 5 mm, making isolated aortic stenting impossible due to the absence of an adequate proximal landing zone.

Connection stents would then ensure coronary flow. The full set of devices was tested in a 3-dimensional (3D) printed model of the patient’s aorta, using fluoroscopy.

After the patient was pre-medicated with acetylsalicylic acid (ASA) (200 mg) and clopidogrel (75 mg), anticoagulation with unfractionated heparin (4 mg/kg) was initiated. A temporary pacemaker was positioned in the right ventricle. Two 0.014-inch guidewires were positioned in the right and left coronary arteries. A pigtail was also positioned in the aortic root with the aid of a guidewire (Video 1).

The left ventricular apex was punctured with a 6-F introducer. A stiff guidewire was used to cross the failed bioprosthetic aortic valve, and the introducer was exchanged for a 30-F introducer. Rapid pacing was used to achieve controlled hypotension. The main body of the stent was slowly deployed in the ascending aorta (Video 2), and afterward, the balloon was expanded to inflate the transcatheter heart valve (Video 3). Pressure was then restored. Both transesophageal echocardiography and angiography showed effective release (Video 4). The ventricular apex was then closed. Extracorporeal membrane oxygenation (ECMO) support was initiated prior to any attempts at coronary cannulation.

The right coronary artery was cannulated first through the inside of the main stent body by using a 6- × 60-mm Fluency stent (Becton Dickinson, Franklin Lakes, New Jersey) (Videos 5 and 6). In the left coronary artery, the Fluency stent positioning was unsuccessful. The operator switched to a 7- × 57-mm Ventus BX stent (Jotec GmbH, Hechingen, Germany), which worked adequately (Videos 7 and 8).

A final angiogram showed no leaks and patent ostia. Control echocardiography showed normal valve function with a low mean gradient of 10 mm Hg. Main procedural steps are shown in Figure 4. Total procedural time was 390 min. The patient was transferred to the intensive care unit for 4 days. A new CT angiogram was ordered, documenting zero endoleak and patent coronary connecting stents. After a week of hospital stay, the patient was discharged home with a prescription for ASA (200 mg) and clopidogrel (75 mg). There was no myocardial infarction, vascular complications, stroke, conduction abnormalities, or renal failure.