Keywords
aortic disease - aneurysm - aorta/aortic - endovascular procedures/stents - stents
Introduction
Marfan syndrome, an autosomal dominant disorder, is a disease of the connective tissue,
especially with manifestation of the cardiovascular system. Aneurismal formation,
dissection, and rupture of the aorta may be consequences of the disease with a high
mortality. Despite early correction, the disease is not curative but progressive.
Several redo surgeries or interventions with high mortality are common in Marfan patients.
Endovascular therapy is controversial discussed as an alternative option for Marfan
patients. Some case reports and series describe the experience of endovascular procedures
in Marfan patients.[1]
[2] However, in cases of involved aortic arch, endovascular repair could be difficult
because it needs surgical debranching of the supra-aortic trunk vessels. In these
cases, custom-made stent-graft systems could help treating complex aortic geometries.
We describe a combined endovascular and operative management for aortic arch repair
in a Marfan patient with complex aortic arch geometry previously treated with several
open surgeries for acute type A dissection and who deemed to be at high operative
risk for another open aortic surgery due to massive aortic calcification. It is an
unusual method of placing a custom-made stent-graft system in the false aortic lumen
(FL) with operative and endovascular treatment of the supra-aortic vessels.
Case Presentation
A 57-year-old female Marfan patient with a medical history of treatment for acute
type A dissection with a mechanical Bentall procedure and coronary artery bypass in
1991 was admitted to our department with dorsal pain. In 1998 and 2001, redo surgeries
were necessary due to pseudoaneurysm formation including a covered rupture at the
distal Bentall anastomosis. In 2009, the patient had also undergone another surgery,
after heavily calcification of the residual intimal flap with a pressure gradient
of 100 mm Hg in the aortic arch.
The computer tomography (CT) scan shows a dilatation of the aortic arch up to 8.3
cm with signs of a new covered rupture ([Fig. 1A]) and complex dissection of the supra-aortic vessels with a porcelain aorta ([Figs. 1B] and [2A, B]). A preoperative angiogram showed multiple reentries between the true lumen (TL)
and FL. An occlusion of the TL was impossible. Therefore, we planned to create a stent-graft
system for placing in the FL. Due to the patient's high-risk profile, conventional
surgery was refused and a minimally invasive hybrid–endovascular and operative approach
was planned.
Fig. 1 (A) Preoperative CT scan of the aneurysmal dilatation of the aortic arch up to 8.3 cm.
The image shows also the origin of the supra-aortic vessels. (B) The image shows the heavily calcification of the aorta (white arrows). CT, computed
tomography; FL, false lumen; TL, true lumen.
Fig. 2 Cross section of the aortic arch at the level of the innominate artery (IA; A) and for the left common carotid artery (LCCA; B). Both images show the dissected branches.
A three-dimensional (3D) reconstruction of the CT scan was required to create a custom-made
stent-graft system. A silicon 3D model, exactly corresponding to the patient's aorta
was manufactured ([Fig. 3]), and the fenestrated tapered stent-graft system (length: 260 mm; proximal diameter:
46 mm; distal diameter: 34 mm; Bolton Medical Inc., Florida, United States) was designed.
The graft was constructed with two side holes corresponding to the innominate artery
(IA) and the left common carotid artery (LCCA).
Fig. 3 A silicon three-dimensional model with exact correspondence to the patient's aorta.
First stage of the procedure involved creation of bilateral subclavian–carotid bypasses.
After heparinization, an activating clotting time (ACT) of > 200 seconds and partial
clamping, an 8.0 mm synthetic graft (Hemashield, Maquet Getinge Group, Rastatt, Germany)
was anastomosed in an end-to-side fashion from the carotid to the subclavian artery.
A 6-Fr sheath was then placed into the right femoral artery. Through this sheath,
a soft wire (Terumo, Terumo Corp., Tokyo, Japan) was positioned in the FL reaching
the ascending aorta and was exchanged over a pigtail catheter by a double curved extrastiff
Lunderquist wire (Cook Inc., Bloomington, Indiana, United States). Similarly, another
6-Fr sheath was placed into the left femoral artery and another pigtail catheter was
established in the TL of the descending aorta.
Through another sheath, placed in the right subclavian–carotid bypass, a third pigtail
catheter marked the origin of the IA. Over the stiff Lunderquist wire, the custom-made
graft system was advanced through the FL with its proximal part in the TL of the ascending
aorta, 5 cm above the aortic valve and its distal part ending in the FL. After confirming
the side holes in the correct position, the graft was deployed under rapid pacing
and hypotension condition.
Then, two Gore ViaBahn grafts (Gore, Flagstaff, Arizona, United States) with 10.0
and 7.5 cm length, respectively, were positioned via the side holes into the right
carotid artery and LCCA. After these stages, a 22.0-mm AMPLATZER Vascular Plug (AGA
Medical, Golden Valley, Minnesota, United States) occluded the proximal part of the
TL of the dissected descending aorta. Perfusion of the abdominal organs was carried
retrograde through several re-entries and a large distal entry tear at the level of
the renal arteries. The right and left subclavian arteries were then occluded at their
origin by two additional AMPLATZER Vascular Plugs.
The postoperative course was uneventful. The patient did not experience any symptoms
of upper-extremity ischemia. A CT scan before discharge showed satisfactory results
with a complete exclusion of the large aneurysm and sufficient supply of the supra-aortic
vessels ([Fig. 4]).
Fig. 4 Postoperative CT scan shows a successful reconstruction of the aortic arch with a
new custom-made stent-graft system placed in the false lumen with occlusion of the
true lumen, left and right subclavian arteries. The dark arrow marks the stent graft
with its origin in distal aorta ascendens. The white arrows mark the two Gore ViaBahn
grafts positioned in the right and left common carotid arteries. The supra-aortic
vessels were sufficiently supplied through the implanted grafts. * marks the occlusion
of the left subclavian artery with an AMPLATZER Vascular Plug. CT, computed tomography.
Discussion
Surgical treatment of the aortic arch is a challenging procedure, especially in patients
with a complex previous surgeries and aortic anatomy. A lot of methods have been developed
during the last years to treat aortic arch pathologies. Hybrid aortic arch procedures,
endovascular aortic arch repair with fenestrated stent grafts, or in situ fenestration
have all been introduced as alternative options for managing complex aortic arch pathologies.[1]
[2]
[3]
Endovascular treatment has become more popular also in Marfan patients but is still
in controversial discussion. Early experiences of endovascular management of aortic
arch pathologies in Marfan patients showed beneficial results.[1]
[2]
Recently, Botta et al presented their experience of reconstructing the aortic arch
treating a penetrating atherosclerotic ulcer in a 75-year-old patient.[4] They also used a custom-made branched thoracic stent-graft system manufactured by
Bolton Medical. First experience with multibranched stent-graft systems for repairing
aortic arch aneurysms were described by Lioupis et al.[5] They successfully demonstrated the technical feasibility of branched stent-graft
systems to treat aortic arch aneurysms in six patients.
According to the high-risk profile of our patient, we decided to treat the covered
rupture with this complex hybrid procedure by positioning a custom-made stent graft
in the FL and occluding the TL of a dissected aorta. Due to the large re-entries,
an occlusion of the TL was impossible. In the literature, such a case has not been
described yet.
