KD's management has had different approachesdepending on the patient's comorbidities, clinical presentation, anatomy, and the surgeon's expertise and personal preferences (1,2,20). Some authors recommend surgical correction only in symptomatic patients since this population has been found to have an accelerated growth rate and a higher risk of rupture (1,2). It is also proposed to intervene in all patients with a KD diameter >30 mm at the base of the KD or a distance from the apex of the KD to the opposite wall of the aorta of >50 mm (1,8).
Both open and endovascular approaches have shown an early mortality rate of around 11% and 2.6%, respectively. However, after three years of follow-up, both approaches demonstrated similar cumulative mortality (16 and 18.2%, respectively). Moreover, an advantage of the endovascular approach is a shorter hospital stay (2,21,22).
Evidence regarding hybrid approaches has been increasing recently. FET has been more researched, showing similar results in the short-term compared to endovascular management, but with an increased risk of complications such as endoleaks (18,20,24). In addition, other hybrid techniques have been proposed, one of which is the thoracic endovascular aortic repair plus supra-aortic debranching (19,23).
KA has been less studied, and the evidence regarding the management of rKA lacks robust and more extensive studies measuring long-term outcomes. Since the evidence about type-1 non-dissecting rKA is even lower, we attempt the description and analysis of data regarding the selection of the different surgical approaches for all types of rKA as follows:
Hybrid approach: The evidence available for KD with this approach shows very similar results to endovascular management (21–23,25). Since this is a more individualized approach, it is recommended for handling complex cases (22). In our experience, this approach is promising for rKA given their diversity and complexity, allowing the individualization of the three types of KD and their complications. Furthermore, it fulfills the requirements of an imminently life-threatening pathology, being a quick, effective, and safe approach.
Ben Abdallah et al. (15) presented a patient with an incidental finding of rKA with a diameter of 81 mm, who underwent a one-stage hybrid procedure. Initially, a TEVAR was performed associated with cervical debranching of the LSA; then, a left carotid endarterectomy. This decision was related to the risk of hemorrhagic stroke due to cardiac arrest. Although the primary procedure was successful, the patient underwent two secondary procedures, one for vertebrobasilar insufficiency and the other for a right cervical lymphocele, and both were managed appropriately. After a four-month follow-up, the patient had fully recovered.
Another case report by Singh et al. (17) reported a patient with acute onset tearing chest pain. An angioCT identified a dissecting 40 mm Type I rKA with an intramural hematoma. The patient underwent a total arch replacement consisting of a right carotid subclavian bypass, followed by open debranching of the bilateral carotid arteries and a zone II arch replacement with proximal intrathoracic ligation of ARSA and LSCA. Also, a FET was performed, followed by a retrograde TEVAR due to a type of IA endoleak. During the postoperative, the patient presented a right lacunar infarct requiring a tracheostomy and a percutaneous gastrostomy. After discharge, the patient persisted with mild residual left‐sided weakness.
Open approach: Historically, this has been the approach of choice, as shown in a review by Cinà et al. (8). However, it is highly invasive, and there is limited evidence available for its use in rKA.
A study by Ikeno et al. (2) presented two patients with rKA who underwent emergent open surgery. One patient had a dissecting rKA with a diameter of 50.9 mm and underwent an extensive aortic repair with selective anterograde cerebral perfusion. This patient had no long-term postoperative complications. The second patient presented a non-dissecting rKA with a diameter of 52 mm and underwent a total aortic arch replacement with selective antegrade cerebral perfusion, resulting in in-hospital mortality secondary to pneumonia.
A case report by Kaki et al. (11) described a patient with a 35 mm Type II rKA. An initial open proximal anastomosis was performed with a branched vascular graft. Then the vascular graft was clamped, the aLSA was reconstructed, and reperfusion of the upper part of the body was started for distal anastomosis of the rKA. The patient presented postoperative pneumonia treated adequately. The total hospital stay was 70 days.
Endovascular and Conservative approaches: These two approaches have been proposed to manage KD and KA (6,10,21,25). Nevertheless, the evidence related to its utility for rKA is minimal due to the patients’ imminent risk of death and the resources required. Specifically, in centers with adequate experience, a total endovascular arch repair could be considered and might play an important role.
Currently, the technique selection depends on the surgeon's ability and experience, the availability of resources and the patient's status, hemodynamic condition, surgical risk, comorbidities, and the different anatomical variants that may be present. In our experience, FET can be a feasible procedure for patients with rKA requiring emergent surgical repair. However, other hybrid procedures could also be performed. More robust studies are required to assess each approach's quality and long-term results to establish the most effective and safe for management of rKA.