New treatment paradigm for patients with Marfan or Loyes–Dietz syndrome

We would like to congratulate the authors to have provided data [1] supporting endovascular treatment of patients with Marfan syndrome or Loeys–Dietz syndrome, both are types of heritable thoracic aortic diseases (HTAD). For these patients, while there is consensus that endovascular treatment can be considered in life-threatening emergencies or when bridging prosthetic grafts from prior open repairs, landing endografts in native aorta and the role of fenestrated and branched endografting (FBEVAR) remain unclear [2]. However, in recent years, particularly in high-volume aortic centres, there is a trend towards integrating endovascular treatment as a therapeutic option in combination with open surgery in HTAD patients, as outlined in the manuscript. We thus very much agree that patients with HTAD should be referred to aortic centres with expertise in both open and endovascular complex procedures. For patients with HTAD, the decision to land within the native aorta must take into consideration the risk of endograft-related complications as well as the need for future reintervention. In this article, interestingly, when the proximal landing zone was in the native aorta, endograft oversizing was only 10%, which is significantly less than the instructions for use from the manufacturers. This was however an appropriate decision as no patients experienced iatrogenic type A dissection (balloon modelling of the proximal sealing zone is strictly forbidden) nor endograft migration or proximal endoleak at the index procedure. During follow-up, 72% of patients underwent secondary interventions for endoleak and aortic dilatation. This significant disease progression directly proximal or distal to the endograft has to be considered when planning your endovascular repair to facilitate straight forward proximal or distal relining in the future if needed. For example, if an FBEVAR is planned for a complex AAA repair, a long proximal landing zone above the coeliac trunk branch or fenestration is mandatory in case a Thoracic Endovascular Repair (TEVAR) needs to be performed later during follow-up. In one of our patients treated urgently for symptomatic juxta-renal aneurysm, we did not consider future intervention and used an endograft with 4 laser fenestrations with only 1 sealing stent above the coeliac trunk. The patient came back 2 years later with a 10-cm aneurysm proximal to the endograft which had to be relined with a four-branch (T-branch, Cook medical) endograft, which was very challenging and could have been prevented with a better initial planning. On the other hand, for HTAD, landing zone within a prior open prosthetic graft is the best-case scenario, which can be created safely by the surgical team in a multi-disciplinary aortic centre. In fact, the authors stated that recently there was a shift from open Crawford type II repair towards a staged approach by creating a stable landing zone with a frozen elephant trunk, TEVAR extension and then type III/IV open completion. A similar staged approach is also now routinely performed in our department, but we have moved away from the type III/IV open completion. When possible, ie if the patient has recovered from the open repair, we perform the relining of the frozen elephant trunk a week after the sternotomy, before discharge to rehabilitation. The patient is then brought back to the hybrid room 6–8 weeks later for a completion FBEVAR. Most of these patients with HTAD have chronic dissections that involve the common iliac arteries, but few have a dilated false lumen below the renal arteries. In order to reduce the spinal cord ischaemia risk, we land the FBEVAR in the true lumen above the inferior mesenteric artery, and position an occluder (previously called candy plug) in the false lumen at the same level. This prevents back flow to the dilated false lumen above and preserves flow to the distal lumbar, medial sacral, and internal iliac arteries. A subsequent relining with a bifurcated endograft can be performed later during follow-up if required. In the setting of complex dissection involving the internal iliac arteries, we have also performed open repair of the infra renal aorta and of the common iliacs to preserve flow to both internal iliacs. The main body of the aortobiiliac graft is tailored long enough in order to have a nice distal landing zone for the FBEVAR that will be next implanted. A specific concern when treating HTAD patients with FBEVAR is the selection of the appropriate bridging stents. We have now switched in this subset of patients to a combined bridging stent strategy. We first implant a self-expandable stent in the target vessel (Viabahn, Gore), and then connect it to the fenestration or branch with a balloon-expandable stent (Begraft Plus, Bentley). This avoids inflating a balloon in a native vessel on 1 side and secures the connection to the fenestration or branch on the other side. During BEVAR, self-expandable stents alone (i.e. not reinforced by a balloon expandable stent) positioned in the true lumen of the aortic dissection usually end up compressed because of a conflict with the main aortic graft in the narrow true lumen, which is associated with a high risk of branch occlusion. Finally, the majority of patients (90%) in the author’s experience already had open surgery, including replacement of aortic valve and/or aortic root, before endovascular treatment. This indicates that many HTAD patients may be on anti-coagulation therapy, which is consistent with our experience, and has an impact on FBEVAR outcomes in patients with chronic dissections. We have observed that these patients have high flow type 2 endoleaks that require secondary embolization procedures, or undiagnosed endoleaks (endotension?) associated with stable or growing aneurysms. We now routinely perform, in this young cohort of patients, sac resection through a left thoracotomy [3] or lumbar incision with intercostal or lumbar arteries ligation, to secure the endovascular repair and to reduce the risk of oesophageal or bronchial fistula. This procedure does not require any aortic clamping, and has been associated frequently with wedge lung resection, as often lung parenchyma is attached to the inflammatory aortic wall. Ultimately, we congratulate again the authors for this paper that highlighted the need for a multidisciplinary approach to offer a staged strategy combining open and endovascular repair to offer good long-term outcomes in these patients with complex aortic pathologies and HTAD. Conflict of interest: Stéphan Haulon has intellectual property and is a consultant of Cook Medical, Bentley and GE Healthcare.

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