Secondary Thoracoabdominal Aortic Replacement Following Total Arch Repair With a Sutureless Integrated Stented Graft in Marfan Syndrome: A Case Report

Abstract

Patient: Female, 52-year-old

Final Diagnosis: Marfan syndrome post total arch replacement with forzen elephant trunk thoracoabdominal aortic aneurysm

Symptoms: None

Clinical Procedure: —

Specialty: Cardiac Surgery

Objective: Unusual clinical course

Background

Secondary thoracoabdominal aortic replacement after a total arch repair is a complex procedure that has a risk of complications such as stroke and renal failure. Patients with Marfan syndrome are more at risk of distal aortic re-interventions. This report describes a 52-year-old woman with Marfan syndrome and a history of type A aortic dissection treated with total arch replacement and frozen elephant trunk implantation using a sutureless integrated stented graft (SISG), who presented 3 years later with a distal aortic dissection aneurysm requiring secondary thoracoabdominal aortic replacement.

Case Report

A 52-year-old woman with Marfan syndrome underwent ascending aortic and total arch replacement with frozen elephant trunk implantation using a sutureless integrated stented graft (SISG) for acute type A aortic dissection 3 years earlier. One month before the current operation, thoracoabdominal aortic replacement was performed because follow-up imaging demonstrated false lumen expansion of the residual thoracoabdominal aortic dissection, with a maximal diameter exceeding 5.5 cm. Intraoperatively, significant false lumen thrombosis and obliteration were observed in the elephant trunk region. A 2-cm unstented graft segment at the distal end of the elephant trunk facilitated proximal anastomosis in the current surgery. Given the patient’s Marfan syndrome, all branch vessels were reconstructed. Follow-up aortic computed tomography demonstrated satisfactory morphology, and the patient was discharged 2 weeks after surgery.

Conclusions

This case demonstrates successful staged repair of residual thoracoabdominal aortic dissection following initial total arch replacement using a SISG in a patient with Marfan syndrome. The sutureless graft facilitated secondary procedure by providing a suitable proximal landing zone, with complete false lumen thrombosis confirming effective primary repair and favorable outcomes.

Introduction

Acute type A aortic dissection (ATAAD) is a highly lethal condition, with surgical repair as the primary treatment [1]. Total arch replacement with frozen elephant trunk represents an effective therapeutic strategy [2,3]. Frozen elephant trunk is a covered aortic stent graft designed for intraoperative placement under direct visualization. The sutureless integrated stented graft (SISG; Beijing Percutek Therapeutics Inc, Beijing, China) is a novel stented vascular graft that, with its unique tapered design and unstented distal segment, reduces circulatory arrest time and improves operative outcomes [4]. While initial surgery is life-saving, residual distal dissection progresses in some patients, necessitating secondary thoracoabdominal aortic replacement (TAAR) [5]. Patients with Marfan syndrome have a systemic connective tissue disorder caused by FBN1 gene mutations. Most patients develop progressive aortic dilatation, which significantly increases both the rate of aortic progression and surgical complexity [6,7]. Here, we report a rare and technically challenging case of successful secondary thoracoabdominal aortic replacement in a patient with Marfan syndrome, following initial total arch replacement using the SISG. This case highlights the potential long-term utility of the prosthesis design in staged aortic repair [8].

Case Report

A 52-year-old female patient with Marfan syndrome, diagnosed based on aortic root dilatation and FBN1 gene mutation [9]. Three years earlier, she underwent ascending aortic and total arch replacement with frozen elephant trunk implantation using a SISG for ATAAD. This novel graft was chosen for its ability to reduce cardiopulmonary bypass time and provide favorable anatomy for staged procedures. Over the previous 3 years, the patient remained hemodynamically stable, with occasional chest and back pain. One month earlier, the follow-up imaging revealed progressive expansion of the residual distal dissection, with the aneurysm diameter exceeding 5.5 cm (Figure 1). Given that the patient had concurrent Marfan syndrome, open repair was recommended over endovascular repair according to current guidelines [10]. Subsequently, TAAR was planned.

Figure 1.

Serial 3-dimensional computed tomography reconstructions of the aorta.

(A) Before the surgery for acute aortic dissection. (B) One week after surgery for acute type A aortic dissection. (C) Three-year follow-up after surgery for type A aortic dissection. (D) After the total thoracoabdominal aortic replacement.

The surgical procedure was as follows. The patient was positioned in right lateral decubitus position during surgery. Through a left thoracoabdominal approach, the thoracoabdominal aorta was fully mobilized. Temporary perfusion was established via side-to-end anastomosis between a branch graft and the left common iliac artery. The proximal clamp was placed on the previous elephant trunk stent, and the distal clamp was positioned above the aneurysm. Upon opening the aortic aneurysm, the previous stent-covered region showed good remodeling without evidence of endoleak. A 2-cm unstented graft segment was identified distal to the stent, surrounded by thickened aortic wall. Continuous 4-0 Prolene sutures were used to perform end-to-end anastomosis between a 26-mm main graft and the original stent graft. After proximal release, abdominal perfusion was restored through the self-established arterial bypass to the femoral artery. The abdominal aorta was cross-clamped, followed by intercostal artery reconstruction. After aortotomy, selective oxygenated blood perfusion was maintained for the celiac trunk and superior mesenteric artery, while cold crystalloid perfusion was administered to the bilateral renal arteries. Using 5-0 Prolene continuous sutures, sequential end-to-end anastomoses were performed between the branch grafts and the following vessels in the order of celiac trunk, superior mesenteric artery, left renal artery, and right renal artery. Finally, bilateral common iliac artery reconstruction was completed. The surgery was associated with minimal blood loss and a shorter operative time. Postoperatively, no organ dysfunction was observed, and the patient remained hemodynamically stable. The patient was transferred from the intensive care unit to the general ward after 3 days. Postoperative aortic computed tomography demonstrated patent thoracoabdominal prosthetic grafts without evidence of endoleak (Figure 1). Figure 1 was created through 3-dimensional reconstruction of contrast-enhanced aortic computed tomography images using iFlux Arterial Image Processing Software (School of Medical Technology, Beijing Institute of Technology). The patient had an uneventful recovery and was discharged on postoperative day 14.

Discussion

This case demonstrates that SISG facilitates secondary thoracoabdominal aortic replacement. The high mortality and complication rates associated with initial surgery for ATAAD make risk reduction a common concern among clinicians [11]. Based on our center’s 10-year experience [12], 14.1% of patients experienced perioperative adverse events, including death, paraplegia, and stroke. The SISG effectively reduces circulatory arrest time and the overall operative time. Postoperatively, the patient remained hemodynamically stable with no organ dysfunction. The patient was transferred from the intensive care unit to the general ward after 3 days and was discharged home in good condition after 14 days. During the secondary surgery, complete thrombosis of the false lumen in the stented segment was observed, and the ease of proximal anastomosis supports the effectiveness of the prosthesis in both the initial and secondary repairs [8]. As reported in the literature, approximately 10% to 40% of patients who undergo ATAAD repair require secondary TAAR, with mortality rates ranging from 8% to 25% [13]. In the present case, intervention was necessitated by progressive expansion of the residual dissection. Although previous literature has reported that endovascular treatment has also achieved good efficacy [14], given the patient’s Marfan syndrome, we opted for individual reconstruction of each branch vessel rather than using a multi-branch patch technique with island anastomosis (Figure 2). This approach was chosen to minimize the risk of residual aortic wall expansion and potential pseudoaneurysm formation. During the initial surgery, a 180-mm intraoperative stent graft was deployed, with its distal end at the T7 level, showing excellent remodeling of the stented segment. For the secondary procedure, access through the sixth intercostal space provided adequate exposure for proximal anastomosis. Although this patient achieved favorable outcomes, as a single case report, this study is inherently limited by the inability to draw broad clinical conclusions and the lack of comparative data with alternative treatment approaches.

Figure 2.

Six-branched prosthetic graft for thoracoabdominal aortic replacement.

A 26-mm 4-branched prosthetic graft for total thoracoabdominal aortic replacement, with 2 additional 10-mm grafts anastomosed to the distal end, creating a 6-branched prosthetic graft configuration.

Conclusions

This case successfully demonstrates staged aortic repair in a patient with Marfan syndrome with residual thoracoabdominal dissection. The initial use of a SISG facilitated the secondary TAAR by providing an ideal proximal anastomotic site. Complete false lumen thrombosis confirmed effective primary repair, leading to excellent clinical outcomes. While the outcome in this case was favorable, further studies are warranted to evaluate long-term durability and broader applicability of this prosthesis.

Abbreviations

ATAAD

acute type A aortic dissection

SISG

sutureless integrated stented graft

TAAR

thoracoabdominal aortic replacement