Arch-First Technique for Extensive Thoracic Aortic Replacement: A Case Series of Patients With Complex Thoracic Aortic Disease

Introduction

Aortic dissection is a life-threatening condition that involves a tear in the inner layer (intima) of the aorta. Type A aortic dissection encompasses the ascending aorta and requires immediate surgical intervention due to the imminent risk of rupture and malperfusion [1]. Risk factors include hypertension, connective tissue disorders (Marfan, Ehlers-Danlos, and Loeys-Dietz syndromes), bicuspid aortic valve, and aortic aneurysm [2]. Patients typically present with severe chest or back pain. Because initial management targets the pathology of the ascending aorta and the primary entry point, meticulous follow-up is required; a substantial proportion of patients – especially those with connective tissue disease – will require reintervention [3]. Management strategies often involve staged procedures to address aortic pathology at the level of the arch, descending thoracic aorta, and abdominal aorta, given that the risk of rupture persists in nonoperated dilated segments [1].

Prior to the development of antegrade perfusion strategies, cerebral protection during extensive aortic arch and descending aortic repair commonly relied on deep hypothermic circulatory arrest combined with retrograde cerebral perfusion via the superior vena cava. Although retrograde cerebral perfusion was intended to provide cerebral cooling and flush embolic debris, its effectiveness in sustaining adequate cerebral blood flow remained limited; the technique also was associated with prolonged ischemic times and variable neurological outcomes [4,5].

To address this limitation, the arch-first technique was first reported in 1999 by Dr. Rokkas and Dr. Kouchoukos [6]. This surgical approach was designed to minimize neurological injury during single-stage open extensive replacement of the thoracic aorta. The technique includes early anastomosis of the aortic graft to the arch vessels, as well as continuous hypothermic antegrade cerebral perfusion. Aortic exposure is achieved through bilateral submammary thoracotomies at the fourth intercostal space, combined with transverse sternotomy. Additional procedures (coronary artery bypass graft or valve/root replacement) are performed as needed. A branched Dacron graft is placed in an anatomic position, and arch vessel anastomoses are constructed, followed by the distal aortic anastomosis, which is performed in an open manner. The proximal graft is then sutured to the ascending aorta or to an existing graft in reoperation cases [6].

Primary benefits include selective antegrade cerebral perfusion and single-stage extensive thoracic aortic replacement involving the arch and proximal descending thoracic aorta in both de novo and reoperation cases. Exposure of the proximal descending thoracic aorta with this approach is satisfactory, making this technique favorable for simultaneous replacement of the arch and the proximal half of the descending thoracic aorta [6].

In the current endovascular era, the frozen elephant trunk (FET) hybrid technique has become a widely used approach for management of such cases. FET involves suturing a Dacron graft to reconstruct the aortic arch under deep hypothermic circulatory arrest, typically with selective antegrade cerebral perfusion. The graft incorporates an integrated distal stent-graft segment that is deployed into the descending aorta to stabilize the aortic wall, promote true lumen expansion, and create a durable landing zone for subsequent endovascular interventions [7–9].

Although the FET technique has become a popular alternative, particularly in acute dissections, the arch-first strategy remains an important option when the descending aorta cannot safely accommodate a stented graft, or in reoperation cases where prior root or ascending repairs limit endovascular access [9].

The aim of this case series is to describe our institutional experience with the arch-first technique in patients with extensive thoracic aortic disease – including Marfan and reoperation cases – and to evaluate its feasibility, morbidity profile, and neurologic outcomes in comparison with contemporary hybrid and staged approaches.

Case Reports

CASE 1:

A 24-year-old man with Marfan syndrome, previously managed via the Bentall procedure and mitral valve replacement, presented with progressive aneurysmal dilatation of the aortic arch and proximal descending thoracic aorta. Complete replacement of the ascending aorta, arch, and proximal descending segment was achieved. Postoperatively, the patient required prolonged ventilation and tracheostomy (intensive care unit [ICU] stay, 28 days; total hospital stay, 34 days). He was discharged in stable condition (Figures 1, 2).

CASE 2:

A 72-year-old man with prior ascending aortic dissection repair developed a progressive arch aneurysm. Single-stage replacement was performed using the arch-first technique. Postoperative recovery was complicated by respiratory insufficiency requiring tracheostomy (total hospital stay, 35 days). The patient recovered and was discharged home (Figure 3).

CASE 3:

A 73-year-old man with previous surgical treatment of acute type A dissection showed contained rupture of a chronic thoracic aortic dissection involving the distal aortic arch. He underwent aortic arch and descending thoracic aorta replacement using the arch-first technique. Recovery was uneventful, and the patient was discharged after 30 days (Figure 4).

CASE 4:

A 24-year-old man with Marfan syndrome, previously managed by ascending aortic replacement for type A aortic dissection, presented with contained rupture of the descending aorta. He was treated via replacement of the ascending aorta, aortic arch, and descending thoracic aorta using a branched graft with the arch-first technique. Intraoperatively, he died of uncontrollable hemorrhage from the distal descending thoracic aorta, attributed to friable aortic tissue at the distal anastomosis.

CASE 5:

A 50-year-old man with Marfan syndrome had previously undergone aortic root aneurysm treatment comprising a Bentall procedure in 2004 and mitral valve replacement in 2015; in this case, he presented with progressive dilatation of the aortic arch and thoracic aorta. The dilatation was successfully managed via replacement of the ascending aorta, aortic arch, and descending thoracic aorta using the arch-first technique. After a prolonged ICU stay of 55 days (due to ventilator-associated pneumonia) and total hospital stay of 73 days, he was transferred to a rehabilitation center in good condition.

CASE 6:

During follow-up for thrombocytopenia, a 69-year-old woman showed extensive aneurysmal disease of the ascending aorta, aortic arch, and proximal descending aorta. This disease was successfully managed through replacement of the ascending aorta, aortic arch, and descending thoracic aorta using the arch-first technique. The postoperative ICU stay was prolonged (32 days) due to chylothorax. The patient’s total hospital stay was 57 days; she was then transferred to a rehabilitation center in good condition (Figures 5, 6).

All patients underwent extensive thoracic aortic replacement without neurological injury, underscoring the cerebral protective efficacy of the arch-first technique. Complications included prolonged ventilatory support with tracheostomy in 3 patients. The average hospital stay was 45.8 days, and there were no cases of paraplegia or significant renal impairment.

Discussion

The arch-first technique represents an important advancement in the surgical management of extensive thoracic aortic disease, particularly in scenarios that require single-stage extensive thoracic aortic replacement [6,10]. Our experience aligns with previous reports demonstrating the technique’s efficacy in maintaining neurologic integrity through cerebral protection with selective antegrade cerebral perfusion [6,10,11]. Paraplegia is unlikely with this approach because it does not involve replacing the distal segment of the descending thoracic aorta that supplies arterial flow to the spinal cord [12].

The clamshell incision, although more extensive, provides excellent visualization and access to both the arch and proximal descending thoracic aortic segments, allowing meticulous dissection and secure anastomoses. However, this exposure carries a risk of considerable morbidity. In our series, 3 patients (50%) required tracheostomy and prolonged mechanical ventilation, consistent with the pulmonary morbidity reported by Kouchoukos et al (17% tracheostomy rate, 48% long-term ventilation) [10]. This high incidence likely reflects the physiologic burden of the extensive clamshell incision and the prolonged operative times required for complex reconstruction, especially in reoperation cases. Meticulous preoperative pulmonary evaluation and aggressive postoperative respiratory management are critical. Minimizing bypass time, implementing early extubation strategies, and utilizing regional or multimodal analgesia may reduce pulmonary complications [13,14].

In patients with Marfan syndrome, the benefits of the arch-first technique are particularly prominent [6,15]. These patients often present with progressive, diffuse aortic disease and possess fragile aortic tissue that complicates repair [2]. The arch-first approach allows controlled single-stage reconstruction of the ascending aorta, aortic arch, and proximal descending thoracic aorta under hypothermia, without needing the cross-clamp application that can infrequently cause iatrogenic aortic injury [16]. Nevertheless, these patients require close follow-up for early identification of evolving pathology to enable elective management; surgical planning must be highly individualized and anticipatory given the high risk of emergent reoperations [17,18]. In the present series, 1 intraoperative death occurred due to uncontrollable distal hemorrhage in a patient with Marfan syndrome. This event underscores the technical difficulty of distal anastomosis in previously operated or scarred tissue planes; it also highlights the importance of individualized surgical planning and careful intraoperative management of fragile distal aortic segments. Reinforcement techniques, such as Teflon felt or external graft wraps, may help prevent bleeding at these sites.

Although the arch-first technique offers distinct advantages in terms of cerebral protection and direct surgical control, the FET approach has gained popularity in the current endovascular era as an alternative strategy for similar aortic pathologies. FET combines open arch replacement with antegrade deployment of a stented graft into the descending thoracic aorta during deep hypothermia, allowing single-stage treatment of both arch and descending thoracic segments [19]. This hybrid approach provides several benefits: it stabilizes the distal aorta, promotes favorable remodeling in acute dissections through true lumen expansion and false lumen thrombosis, and creates a reliable landing zone for future endovascular extensions [20]. Such benefits may reduce the need for staged procedures and the cumulative risk of multiple operations, particularly in patients with pulmonary disease, while achieving outcomes that are overall equivalent or superior to those of open approaches [21–23]. However, this approach may be suboptimal relative to the arch-first technique in certain situations. When endovascular completion after FET is delayed, the risk of rupture persists during the interim period. In patients with connective tissue disorders, there also is a risk of type IV late endoleak from the distal landing zone as the native aorta continues to enlarge. In contrast, the arch-first technique provides a stable and suitable landing zone for future endovascular repair of the descending or abdominal aorta, if required.

Additionally, chronic dissections with a small true lumen can present technical challenges due to a rigid aortic septum, which may impede stent deployment and lead to critical true lumen stenosis (pseudo-coarctation) with potential spinal or visceral malperfusion [24]. In such cases – particularly among patients with Marfan syndrome – the arch-first technique offers a safer, more controlled open approach that avoids these risks.

When comparing full endovascular techniques (eg, thoracic endovascular aortic repair) with open surgical approaches, open repair appears to be superior – particularly in patients with connective tissue disorders. Although supporting evidence remains limited, the endovascular approach carries a substantial risk of developing various types of endoleaks, attributable to progressive pathologic changes that affect both proximal deployment and distal landing zones over time [25].

When interpreting our results, several limitations must be acknowledged. The series is small and retrospective, and the absence of a control group limits direct comparison with alternative approaches (eg, staged or hybrid repairs). Moreover, the high morbidity indicates that the arch-first technique should be reserved for specialized centers with extensive expertise in complex aortic surgery, along with well-coordinated cardiothoracic anesthesia teams.

Despite these limitations, our findings demonstrate that the arch-first technique remains a viable and valuable method in selected patients, particularly those with Marfan syndrome, reoperation cases, and individuals with extensive aortic involvement not amenable to endovascular management. However, the procedure remains complex and is associated with substantial morbidity, primarily related to pulmonary dysfunction and the need for prolonged mechanical ventilation. Thus, the decision to employ an arch-first or FET strategy must be individualized based on patient characteristics and the surgical team’s experience. FET may be particularly valuable for patients with pulmonary comorbidities and suitable aortic anatomy (eg, aneurysms of atherosclerotic etiology), in whom median sternotomy is preferred. Future multicenter registries or meta-analyses comparing open arch-first and FET outcomes could help refine patient selection criteria and clarify long-term survival, freedom from reintervention, and quality of life maintenance.

Conclusions

The arch-first technique represents an effective surgical strategy for single-stage extensive thoracic aortic replacement, offering reliable cerebral protection and reduced neurological risk in complex aortic disease, particularly among patients with Marfan syndrome and in reoperation settings. Despite its invasive nature and potential for pulmonary morbidity, the arch-first technique remains a valuable option for selected patients. Further multicenter studies are needed to compare its outcomes with those of FET and hybrid approaches.