Fenestrated Endograft Repair of Suprarenal Aortic ...

Fenestrated Endograft Repair of Suprarenal Aortic Patch Aneurysm in a Patient With Marfan Syndrome

Vascular and Endovascular Surgery 46(1) 66-69 ª The Author(s) 2012 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1538574411422275 http://ves.sagepub.com

Doeke Boersma, MD1, Geoffrey T. L. Kloppenburg, MD, PhD2, Jan Albert Vos, MD, PhD3, Danyel van den Heuvel, MD3, and Jean-Paul P. M. de Vries, MD, PhD1

Abstract We present a case of a 4-fenestrated endograft repair of a suprarenal aortic patch aneurysm as useful alternative for complex open reoperation in a patient with Marfan syndrome. Keywords surgery, graft, aneurysm, Marfan, endoprosthesis

Introduction

Case Report

Marfan syndrome is an inheritable connective tissue disease with an autosomal-dominant transmission pattern that may affect the ophthalmologic, musculoskeletal, and cardiovascular systems. Aortic dilatation and dissections are the most lifethreatening manifestations of this syndrome. Aneurysms frequently involve the entire thoracoabdominal aorta, requiring staged replacement. Progress in surgical techniques related to the ascending aorta has substantially improved the life expectancy in patients with Marfan syndrome.1 Unfortunately, even after successful aortic root replacement, the descending aorta remains a source of late complications in Marfan syndrome. Outcome of conventional surgery for descending aorta disease is precarious in patients with Marfan syndrome, resulting in a mortality rate of 7.4% for primary intervention.2 Mortality increases up to 40% in redo surgery for visceral patch aneurysms.3 A recent review, excluding patients with Marfan syndrome, showed that thoracic endovascular aortic repair (TEVAR) reduces early mortality and paraplegia rates compared with open repair, especially for repeated aortic surgery.4 The role of percutaneous stent grafting for primary treatment of thoracoabdominal aorta aneurysms in patients with Marfan syndrome has not been established. Considering the increased risks of redo aortic surgery, endovascular stent graft repair could be a feasible alternative to open surgery. We describe a patient with Marfan syndrome with a patch aneurysm of the descending aorta at the level of the diaphragm after previous staged complete aortic replacement. The patch aneurysm was treated with a 4-fenestrated aortic stent graft.

A 34-year-old man with Marfan syndrome presented with subacute abdominal pain radiating to the back. His medical history was extensive for cardiac and aortic surgery: his ascending aorta was replaced in 1992 because of aneurysmal dilatation, followed by aortic, pulmonary, tricuspid, and mitral valve replacement due to severe insufficiency. He later required spondylodesis by a ventral approach of the thoracic vertebrae 11 to lumbar vertebrae 4, for correction of a severe kyphoscoliosis. After a type B acute aortic dissection with retrograde dissection into the aortic arch, a Dacron aortic graft was implanted from the distal ascending aorta to the descending part, ending with an elephant trunk. Owing to progressive dilatation of the postdissection aneurysm, a Gelseal polyester bifurcation graft (Vascutek/Terumo, Ann Arbor, Michigan) ending bilaterally at the iliac arteries was placed via median laparotomy. An Intergard polyester graft (Maquet Inc, Wayne, New Jersey) was later placed from the elephant trunk to the

1 Department of Vascular Surgery, St Antonius Hospital, Nieuwegein, Netherlands 2 Department of Cardiothoracic Surgery, St Antonius Hospital, Nieuwegein, Netherlands 3 Department of Interventional Radiology, St Antonius Hospital, Nieuwegein, Netherlands

Corresponding Author: Geoffrey T. L. Kloppenburg, Department of Cardiothoracic Surgery, St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, Netherlands Email: [email protected]

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Figure 2. Computed tomography (CT) angiograph showing dilatation of native mesenteric islet.

Figure 1. Schematic illustration of initial state after staged aortic replacement.

bifurcation graft by thoracophrenicolaparotomy. The celiac trunk, superior mesenteric artery, and right renal artery were reimplanted as an islet of native aorta (Figures 1, 2, and 3A). A computed tomography angiography (CTA) performed to identify the cause of the abdominal pain showed a patch aneurysm of the islet of the native aorta around the celiac trunk, superior mesenteric artery, and left renal artery (Figure 2). Maximum diameter of the aneurysm was 6.6 cm with rapid progression (>2 cm) within the last 9 months of follow-up. The history of multiple thoracoabdominal surgical interventions rendered a renewed open procedure as technically demanding, posing significant perioperative risks. In our multidisciplinary meeting with vascular and thoracic surgeons and interventional radiologists, we decided to treat the aneurysm by a custom-made 4-fenestrated endograft (Cook Medical, London, UK). A straight fenestrated tube graft with a diameter of 36 mm (15% oversized compared to the diameter of the aorta

proximal and distal of the aneurysm) was introduced via femoral access. The fenestrations were bridged to the 4 target arteries (celiac trunk, superior mesenteric artery, and both renal arteries) using Advanta covered stents (Atrium, Hudson, New Hampshire) of 8 and 10 mm diameters, respectively. The Advanta stents were flaired into the fabric of the fenestrated endograft to prevent type III endoleaks. The patient’s postoperative recovery was uneventful. No spinal cord, intestinal, or kidney ischemia developed. He was discharged 2 days after the procedure. At the 6-month follow-up, the patient remained in good clinical condition, without intestinal, renal, or neurologic symptoms. A followup CTA showed no endoleak (Figure 3B and C).

Discussion Marfan syndrome is an inherited connective tissue disorder affecting 1 in 10 000 individuals, which originates from mutations in the fibrillin 1 gene. Fibrillin is a major building block of the microfibrils that serve as substrate for elastin in the aorta. Affected microfibrils weaken the aortic wall.4 Progressive mitral and tricuspid valve disease is the most common cause of infant morbidity. If untreated, Marfan syndrome is lethal, with an average life expectancy of 30 to 40 years. Most deaths after infancy are usually caused by ascending aortic dissection and chronic aortic regurgitation. The success of current medical and surgical treatment of aortic disease, especially prophylactic aortic root replacement, in patients with Marfan syndrome has increased their life expectancy, extending it up to 70 years.1


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Figure 3. A, Three-dimensional (3D) reconstruction of computed tomography (CT) angiograph before placement of endograft. B and C, Reconstruction of CT angiograph after placement of a 4-fenestrated endograft with branches in celiac trunk, superior mesenteric artery, and both renal arteries.

Even after successful aortic root replacement, progressive dilatation of degenerating thoracoabdominal aneurysms or the dissected descending aorta is likely to occur, necessitating surgical repair. Outcomes of primary open repair in thoracoabdominal aneurysms have dramatically improved over the last decades due to implementation of protective measures such as distal aortic perfusion, cerebrospinal fluid drainage, and neurologic monitoring. In a large retrospective study that included 178 patients with Marfan syndrome undergoing thoracoabdominal aortic repair, neurologic deficit occurred in 4% and renal failure occurred in 8%. A 30-day mortality rate of 3.3% was reported.5 A recently published meta-analysis suggests that TEVAR reduces paraplegia, renal insufficiency, and length of stay—but not the overall survival—compared with open surgery for primary treatment of thoracoabdominal aneurysms.2 However, authors excluded studies concerning patients with Marfan syndrome.

The role of TEVAR for primary treatment of aortic pathologies in patients with Marfan syndrome is heavily debated. Current consensus implies that stent grafts should be used with great caution in patients with Marfan syndrome or other connective tissue disorders. The aorta in these patients is prone to continuing dilatation, rendering endovascular treatment of limited durability.6 This consensus is supported by case series of patients with Marfan syndrome who received a TEVAR, which failed in 7 of 13 patients, leading to open reoperation or death. The thoracic aorta continued to dilate in all patients.7,8 The recent availability of endostapling devices may play a future role in proximal and distal sealing and fixation of stent grafts in patients with Marfan syndrome, thereby possibly improving outcome, although no cases have been published. Pseudo and patch aneurysms are life-threatening late complications after open thoracoabdominal aortic aneurysm repair. A 17.6% incidence of visceral patch aneurysms is reported in


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patients with connective tissue disease, such as Marfan, compared with 5.6% in those with atherosclerotic disease. Limiting patch size is recommended in patients with a high risk of recurrent aneurysmal dilatation.3 In contrast to the primary operation, the results for redo open surgery of the descending aorta, occurring in 10% to 27% of the patients with Marfan syndrome, remain poor and carry an increased mortality risk3 of up to 40%. In view of the increased risk of redo aortic surgery, endovascular stent graft repair could be a reasonable alternative to open surgery in selected cases. We considered endovascular repair as being particularly attractive because in our patient it was possible to land both proximal and distal ends in the existing aortic grafts. This is the first case that reports an exclusion of a visceral patch aneurysm with a 4-fenestrated endoprosthesis. Previous unilateral subclavian bypass and contralateral subclavian aneurysm forced us to place the stent graft and all 4 branches through bilateral groin incisions. Although challenging, the procedure was uncomplicated, thereby undermining the necessity of a subclavian access for the placement of a 4-fenestrated endoprosthesis. In the study by LeMaire and colleagues, endovascular stent grafts were used to successfully treat 3 patients with false aneurysms at the distal anastomosis.5 In another study of 4 patients with Marfan syndrome, the same group described successful endovascular treatment of thoracic aortic pseudoaneurysms after extensive aortic replacement surgery. No endoleaks were observed after a mean follow-up of 52 months.9 A role may therefore exist for more minimally invasive techniques in surgically challenging redo cases to limit the physical effect and operative risks even in patients with Marfan syndrome, although larger series with long-term follow-up are necessary.

Conclusion Fenestrated endograft repair of a patch aneurysm can be a useful alternative for complex open reoperation in patients with Marfan syndrome, especially in cases where landing zones in previously placed open surgical grafts exist. Bilateral femoral access is possible, even with 4-fenestrated endografts, and avoids subclavian artery access.

Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.

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