DOI:10.2214/AJR.05.0424
AJR 2006; 187:W302-W312
© American Roentgen Ray Society
Surgical and Endovascular Repair of Aortic Coarctation: Normal Findings and Appearance of Complications on CT Angiography and MR Angiography
Ming-Chen Paul Shih1,2,
Ashok Tholpady3,
Christopher M. Kramer1,4,
Malcolm K. Sydnor1,5 and
Klaus D. Hagspiel1
1 Division of Noninvasive Cardiovascular Imaging, Department of Radiology,
University of Virginia Health System, 1215 Lee St., PO Box 800170,
Charlottesville, VA 22908.
2 Department of Medical Imaging, Kaohsiung Medical University Hospital,
Kaohsiung, Taiwan.
3 University of Virginia Medical School, Charlottesville, VA.
4 Division of Cardiology, Department of Medicine, University of Virginia Health
System, Charlottesville, VA.
5 Present address: Department of Radiology, Virginia Commonwealth University
Health System, Richmond, VA.
Received March 10, 2005;
accepted after revision June 7, 2005.
Address correspondence to K. D. Hagspiel
(kdh2n{at}virginia.edu).
WEB
This is a Web exclusive article.
Abstract
OBJECTIVE. A variety of treatment options exist for aortic
coarctation, both surgical and catheter-based. Knowledge of the normal
radiologic appearance of these, as well as their typical complications, is
essential for interpretation of CT and MR angiographic studies in these
patients.
CONCLUSION. CT and MR angiography are noninvasive techniques that
are well suited to follow patients after coarctation repair.
Keywords: aortic coarctation • cardiovascular imaging • CT angiography • MR angiography • vascular imaging
Introduction
Aortic coarctation is a common cardiovascular lesion accounting for 5-7% of
all congenital heart disease
[1]. It is defined as a
discrete stenosis in the proximal descending thoracic aorta, first described
by Morgagni at autopsy in 1760
[2]. Coarctation is twice as
common in males as in females, and is known to occur in conjunction with a
variety of conditions, including Turner's syndrome, Shone syndrome,
ventricular septal defect, bicuspid aortic valve, and aneurysms of the circle
of Willis [3]. Without
appropriate treatment, complications are common and include aortic dissection,
infective endocarditis, severe aortic insufficiency, hypertension, coronary
artery disease, and intracranial hemorrhage
[4]. Up to 90% of patients with
uncorrected coarctation die by the age of 60 years.
A number of surgical and endovascular treatment options are available.
Surgical treatment is preferred in neonates and infants
[5,
6].
In 1982, transcatheter balloon dilation was first described as a potential
alternative to surgery [7].
Another successful alternative to end-to-end anastomosis as the primary
treatment is the placement of balloon-expandable endovascular stents
[8]. Stents are also placed in
the setting of failed or complicated percutaneous transluminal angioplasty.
Endovascular treatment is generally preferred in older children and adults.
Despite more than five decades of experience with many treatment techniques of
this seemingly simple lesion, there continues to be considerable discussion of
what are the best therapeutic approaches for both pediatric and adult
patients.
After repair of aortic coarctation, close follow-up of patients is
recommended because surgery is in many ways not curative. Late complications,
as a consequence of the surgery itself or the systemic arteriopathy, are not
rare. Irrespective of the success of the repair, hypertension frequently
develops and is a major contributor to long-term cardiovascular morbidity,
although early surgical intervention may reduce the risk of developing late
hypertension and other cardiovascular sequelae.
In one large surgical series, the most common causes of death in patients
with successful coarctation repair were coronary artery disease (37%),
congestive heart failure (9%), and complications of reoperation (7%)
[4]. The most frequent
indications for reoperation include recurrent coarctation, ascending aortic
aneurysm, valvular heart disease, and pseudoaneurysm formation
[5]. Therefore, the radiologist
is likely to be confronted with both the normal and complicated appearances
that result from a large variety of treatment techniques. In this pictorial
essay, we review the normal findings and the appearance of complications after
coarctation repair.
Noninvasive Imaging
Because of its ability to provide both anatomic and hemodynamic
information, angiography remains the gold standard for the pretherapeutic
workup of patients with coarctation. However, the noninvasive cross-sectional
imaging techniques, including echocardiography, CT, and MRI, are the primary
techniques for posttreatment evaluation and surveillance. The preferred
techniques for patient follow-up are MDCT angiography and MRI, including
contrast-enhanced MR angiography, which are the focus of this pictorial
essay.
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Fig. 1 —Surgical techniques for repair of aortic coarctation. Top row
shows end-to-end anastomosis. Segment containing coarctation is resected, and
proximal and distal aortic segments are apposed directly end to end. Second
row shows subclavian flap procedure. Distal subclavian artery is divided, and
flap of proximal portion of vessel is used to widen segment with coarctation.
Third row shows patch aortoplasty. Elliptic woven Dacron (DuPont) patch is
inserted to expand diameter of lumen. Fourth row shows interposition grafting.
If resected segment of coarctation is too long to allow end-to-end
anastomosis, interposition graft is inserted, creating proximal and distal
anastomoses. Bottom row shows extraanatomic bypass graft. Extraanatomic
ascending aorta-to-descending thoracic aorta bypass grafting is created
through median sternotomy and posterior pericardial approach.
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The decision as to what technique to use largely depends on the equipment
and expertise available at the institution. Ungated MDCT angiography is
generally sufficient for cases where only the aorta and its branches are of
interest. Additionally, ECG-gated CT angiography allows evaluation of the
aortic valve. Similarly, contrast-enhanced MR angiography is, for the most
part, sufficient for evaluation of the aorta and its branches, whereas cine
MRI and phase-contrast MRI allow assessment of the hemodynamic significance of
the coarctation, as well as cardiac and valvular function.
Maximum-intensity-projection (MIP) and multiplanar reconstructions are
preferable for contrast-enhanced MR angiography, whereas volume-rendered and
multiplanar reconstructions are better for CT angiography data display.
Surgical Repair of Coarctation
The first surgical repair of aortic coarctation was performed by Crafoord
in 1944 [5]
(Fig. 1). Since then three
major types of surgical techniques for coarctations have evolved: prosthetic
patch aortoplasty, subclavian flap aortoplasty, and extended end-to-end
anastomosis (Figs. 2 and
3). Less frequently used
techniques include insertion of a graft in situ (Figs.
4,
5A,
5B, and
5C) or in an extraanatomic
location. Figure 1 shows all
currently used techniques.
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Fig. 2 —39-year-old man after operative repair of aortic coarctation
using end-to-end anastomosis technique in early childhood. Oblique sagittal
multiplanar reformatted CT angiogram shows normal postoperative appearance of
repaired coarctation; arrowheads point to site of anastomosis. Note relatively
distal origin of left subclavian artery close to repair (arrow).
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Fig. 3 —3-year-old girl after surgical repair using end-to-end
anastomosis technique for aortic coarctation. Oblique sagittal
maximum-intensity-projection 3D contrast-enhanced MR angiography shows normal
end-to-end anastomosis features (arrowheads).
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Fig. 4 —27-year-old man who underwent interposition graft repair of
aortic coarctation in early infancy. Oblique sagittal
maximum-intensity-projection 3D contrast-enhanced MR angiography shows
postoperative condition with residual hypoplastic arch (single
arrowhead) and coarctation treated with interposition graft. Arrows show
proximal and distal anastomoses. Note ascending aortic ectasia (double
arrowhead).
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Fig. 5A —3-week-old girl born with preductal coarctation of aorta and
patent ductus arteriosus (PDA) treated with pulmonary homograft repair of
coarctation and ligation of PDA. Maximum-intensity-projection (MIP) oblique
sagittal MDCT angiography shows aortic coarctation (arrow) and PDA
(arrowhead).
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Fig. 5B — 3-week-old girl born with preductal coarctation of aorta and
patent ductus arteriosus (PDA) treated with pulmonary homograft repair of
coarctation and ligation of PDA. Postsurgical MIP (B) and thin-slab MIP
(C) contrast-enhanced MR angiography show appearance after successful
ligation of PDA and proximal and distal anastomoses of pulmonary homograft
(arrows). Note hypoplasia of right lung and right pulmonary artery
with extralobar sequestration (star, B), with afferent blood
supply from infradiaphragmatic abdominal aorta (single arrowhead,
B) and venous drainage to suprahepatic inferior vena cava (double
arrowhead, B). Also note aberrant right subclavian artery
origin.
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Fig. 5C — 3-week-old girl born with preductal coarctation of aorta and
patent ductus arteriosus (PDA) treated with pulmonary homograft repair of
coarctation and ligation of PDA. Postsurgical MIP (B) and thin-slab MIP
(C) contrast-enhanced MR angiography show appearance after successful
ligation of PDA and proximal and distal anastomoses of pulmonary homograft
(arrows). Note hypoplasia of right lung and right pulmonary artery
with extralobar sequestration (star, B), with afferent blood
supply from infradiaphragmatic abdominal aorta (single arrowhead,
B) and venous drainage to suprahepatic inferior vena cava (double
arrowhead, B). Also note aberrant right subclavian artery
origin.
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Complications associated with all surgical techniques, but especially with
prosthetic patch aortoplasty and subclavian flap aortoplasty, include aneurysm
formation and high recoarctation rate, respectively. The low complication rate
of end-to-end anastomosis makes it the most popular surgical technique
[6].
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Fig. 6 —15-year-old girl after subclavian flap surgical repair 11
years earlier. Maximum-intensity-projection MR angiography shows
pseudoaneurysm formation (star) at operative repair site. Note
postsurgical occlusion of proximal left subclavian artery (arrow)
whose distal portion is reconstituted by way of vertebral artery
(arrowhead).
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Fig. 7 —53-year-old man who underwent repair of aortic coarctation 20
years earlier. Maximum-intensity-projection 3D contrast-enhanced MR
angiography shows normal appearance of extraanatomic bypass graft from
ascending to descending thoracic aorta (arrows). Aortic arch distal
to left common carotid artery and proximal descending thoracic aorta is
surgically absent.
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In subclavian flap aortoplasty, the left subclavian artery is ligated at
the first branch. An incision is made along the coarctation and the subclavian
artery to create a flap. The posterior wall of the coarctation is then
resected, and the subclavian flap is transposed to enlarge the stenotic area.
This technique is thought to allow growth of the anastomosis and thus is used
in children. However, it is not recommended in adults because of concern for
reduced arterial supply to the arm (Fig.
6).
To decrease potential surgical complications in adult patients with complex
forms of aortic coarctation repair, techniques of extraanatomic ascending
aorta-to-descending thoracic aorta bypass grafting have been described
(Fig. 7).
Endovascular Treatment Options
Transcatheter balloon angioplasty for both native and recurrent coarctation
is increasingly performed, and its indications are expanding. Complications of
this treatment technique include recurrent stenosis, dissection, aneurysm
formation, and, rarely, rupture. Recurrence is a frequent problem, especially
in infants and young children. Balloon dilation may be viewed as a palliative
or staged procedure. Since the mid 1980s, endovascular stents have become an
integral component for the treatment of lesions with recoil after primary
balloon angioplasty.
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Fig. 8A —36-year-old man with medical history of familial non-Williams
supravalvular aortic stenosis and coarctation who had patch repair of
coarctation at age of 7 years. He returns now for endovascular treatment of
aortic recoarctation. Oblique sagittal maximum-intensity-projection
contrast-enhanced MR angiography (A) and right posterior oblique arch
aortogram (B), done for persistent hypertension, show recoarctation of
aorta (arrows) at site of patch repair with associated left carotid
and left subclavian artery orifice stenoses (arrowheads). A 40-mm
gradient is present across lesion.6
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Fig. 8B —36-year-old man with medical history of familial non-Williams
supravalvular aortic stenosis and coarctation who had patch repair of
coarctation at age of 7 years. He returns now for endovascular treatment of
aortic recoarctation. Oblique sagittal maximum-intensity-projection
contrast-enhanced MR angiography (A) and right posterior oblique arch
aortogram (B), done for persistent hypertension, show recoarctation of
aorta (arrows) at site of patch repair with associated left carotid
and left subclavian artery orifice stenoses (arrowheads). A 40-mm
gradient is present across lesion.6
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Fig. 8C —36-year-old man with medical history of familial non-Williams
supravalvular aortic stenosis and coarctation who had patch repair of
coarctation at age of 7 years. He returns now for endovascular treatment of
aortic recoarctation. Follow-up aortogram after stent placement (C),
oblique sagittal multiplanar reconstruction (D), and volume-rendered
MDCT angiogram (E) show complete resolution of stenosis after balloon
dilatation and stent placement (arrows) in region of coarctation. No
pressure gradient is seen at end of procedure.
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Fig. 8D —36-year-old man with medical history of familial non-Williams
supravalvular aortic stenosis and coarctation who had patch repair of
coarctation at age of 7 years. He returns now for endovascular treatment of
aortic recoarctation. Follow-up aortogram after stent placement (C),
oblique sagittal multiplanar reconstruction (D), and volume-rendered
MDCT angiogram (E) show complete resolution of stenosis after balloon
dilatation and stent placement (arrows) in region of coarctation. No
pressure gradient is seen at end of procedure.
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Fig. 8E —36-year-old man with medical history of familial non-Williams
supravalvular aortic stenosis and coarctation who had patch repair of
coarctation at age of 7 years. He returns now for endovascular treatment of
aortic recoarctation. Follow-up aortogram after stent placement (C),
oblique sagittal multiplanar reconstruction (D), and volume-rendered
MDCT angiogram (E) show complete resolution of stenosis after balloon
dilatation and stent placement (arrows) in region of coarctation. No
pressure gradient is seen at end of procedure.
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Fig. 9A —2-day-old girl with critical coarctation of aorta at birth
complicated by thrombosis of distal arch and proximal descending thoracic
aorta underwent aortotomy, thrombectomy, and coarctation repair with pulmonary
homograft insertion. This was complicated by development of membranous
anastomotic stenosis, which was treated with percutaneous transluminal
angioplasty. Since then, she has undergone repeated balloon angioplasties for
residual obstruction. Maximum-intensity-projection (MIP) contrast-enhanced MR
angiography shows initial appearance of thrombosed arch (arrow)
distal to left common carotid artery and proximal descending thoracic aorta
(arrowhead).
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Fig. 9B —2-day-old girl with critical coarctation of aorta at birth
complicated by thrombosis of distal arch and proximal descending thoracic
aorta underwent aortotomy, thrombectomy, and coarctation repair with pulmonary
homograft insertion. This was complicated by development of membranous
anastomotic stenosis, which was treated with percutaneous transluminal
angioplasty. Since then, she has undergone repeated balloon angioplasties for
residual obstruction. MIP contrast-enhanced MR angiography after surgical
revision and percutaneous transluminal angioplasty for recoarctation shows
membranous stenosis (arrow).
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Fig. 9C —2-day-old girl with critical coarctation of aorta at birth
complicated by thrombosis of distal arch and proximal descending thoracic
aorta underwent aortotomy, thrombectomy, and coarctation repair with pulmonary
homograft insertion. This was complicated by development of membranous
anastomotic stenosis, which was treated with percutaneous transluminal
angioplasty. Since then, she has undergone repeated balloon angioplasties for
residual obstruction. Oblique sagittal cine MR angiography shows jet caused by
membrane proving its hemodynamic significance (arrowhead).
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Fig. 10A —29-year-old woman who underwent patch aortoplasty repair of
aortic coarctation and closure of patent ductus arteriosus (PDA) that occurred
at age 3 years. Three-dimensional contrast-enhanced MR angiography maximum
intensity projection shows trilobed pseudoaneurysms of proximal descending
aorta (arrowhead) just distal to left subclavian artery
(arrow) and between origins of left carotid and subclavian
arteries.
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Fig. 10B —29-year-old woman who underwent patch aortoplasty repair of
aortic coarctation and closure of patent ductus arteriosus (PDA) that occurred
at age 3 years. Axial multiplanar reconstruction of contrast-enhanced MR
angiography shows two of three aneurysms (arrows) protruding from
each side of aortic arch (star).
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Fig. 11A —34-year-old man with history of aortic coarctation operated
on at age 14 years with placement of a Dacron (DuPont) patch. Right posterior
oblique digital subtraction angiogram (A) and
maximum-intensity-projection contrast-enhanced MR angiogram (B) show
pseudoaneurysm (arrows) in region of aortic isthmus where coarctation
was repaired.
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Fig. 11B —34-year-old man with history of aortic coarctation operated
on at age 14 years with placement of a Dacron (DuPont) patch. Right posterior
oblique digital subtraction angiogram (A) and
maximum-intensity-projection contrast-enhanced MR angiogram (B) show
pseudoaneurysm (arrows) in region of aortic isthmus where coarctation
was repaired.
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Aortic stent placement is feasible in both patients with coarctation and
those with recoarctation (Figs.
8A,
8B,
8C,
8D, and
8E). The use of endovascular
stents in children is controverial. Concerns about the feasibility of
redilation in growing children generally limits the use of stents to adults
and older adolescents.
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Fig. 12 —28-year-old man after repair of transposition of great
vessels that occurred as infant and patch repair of aortic coarctation that
occurred at age 6 years. Maximum-intensity-projection contrast-enhanced MR
angiography shows large aneurysm in proximal descending thoracic aorta.
Patient subsequently underwent uneventful surgical tube graft repair.
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Fig. 13A —29-year-old man with aortic coarctation patch repair that
occurred at age 6 years presents with abrupt onset of left chest pain.
Maximum-intensity-projection (A) and volume-rendered (B) MDCT
angiograms show acute rupture of descending thoracic aortic pseudoaneurysm
(B) (star, B) at site of previously repaired aortic
coarctation.
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Fig. 13B —29-year-old man with aortic coarctation patch repair that
occurred at age 6 years presents with abrupt onset of left chest pain.
Maximum-intensity-projection (A) and volume-rendered (B) MDCT
angiograms show acute rupture of descending thoracic aortic pseudoaneurysm
(B) (star, B) at site of previously repaired aortic
coarctation.
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Fig. 13C —29-year-old man with aortic coarctation patch repair that
occurred at age 6 years presents with abrupt onset of left chest pain. Axial
MDCT source image shows site of leak (arrowhead) and hemothorax.
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Fig. 14A —21-year-old woman with history of three previous aortic
coarctation repairs, most recent of which was 8 years earlier and consisted of
interposition Dacron (DuPont) graft. She presented emergently with massive
hemoptysis. Oblique sagittal volume-rendered CT angiography reveals large
pseudoaneurysm at proximal descending thoracic aorta.
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Fig. 14B —21-year-old woman with history of three previous aortic
coarctation repairs, most recent of which was 8 years earlier and consisted of
interposition Dacron (DuPont) graft. She presented emergently with massive
hemoptysis. Axial contrast-enhanced CT image shows hemomediastinum,
hemothorax, and site of leak (arrow).
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Fig. 14C —21-year-old woman with history of three previous aortic
coarctation repairs, most recent of which was 8 years earlier and consisted of
interposition Dacron (DuPont) graft. She presented emergently with massive
hemoptysis. Postoperative maximum-intensity-projection CT angiography reveals
extraanatomic Dacron Hemashield (Meadox Medicals) bypass (arrowheads)
from ascending aorta to intraabdominal supraceliac aorta and surgical
occlusion of arch distal to left common carotid artery and proximal descending
thoracic aorta. Also note extraanatomic graft from aortic graft to left
subclavian artery (arrow).
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Complications of Treatment
Restenosis or Recoarctation
Recoarctation is considered to be present whenever the pressure gradient
across the anastomotic stenosis is higher than 20 mm Hg at rest and occurs
after both surgical and endovascular treatment. Residual coarctation is
defined by the presence of a gradient in the early postoperative period.
Recurrent coarctation is inferred when there is a gradient at later stages.
Occasionally, this stenosis can have the appearance of webs, which can make
them hard to visualize on CT angiography and contrast-enhanced MRA, but cine
MRI usually detects the jet caused by the stenosis (Figs.
9A,
9B, and
9C).
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Fig. 15A —46-year-old woman with long history of hypertension and
recent diagnosis of congenital coarctation of aorta presented for balloon
angioplasty of coarctation. Angioplasty was performed with near-complete
resolution of initial pressure gradient of 37 mm Hg, but was complicated by
type B aortic dissection. Oblique sagittal maximum-intensity-projection (MIP)
contrast-enhanced MR angiogram shows type B dissection (arrowheads),
which extends from just below coarctation (arrow) to level of celiac
axis. She subsequently underwent stent placement.
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Fig. 15B —46-year-old woman with long history of hypertension and
recent diagnosis of congenital coarctation of aorta presented for balloon
angioplasty of coarctation. Angioplasty was performed with near-complete
resolution of initial pressure gradient of 37 mm Hg, but was complicated by
type B aortic dissection. Axial (B) and sagittal (C) (MIP) CT
angiograms images performed 6 years later show descending thoracic aortic
stent in true lumen (arrowheads) with stable descending thoracic
aortic and abdominal aortic dissection. Dissecting membrane (arrows,
C) terminates between celiac artery and superior mesenteric artery.
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Fig. 15C —46-year-old woman with long history of hypertension and
recent diagnosis of congenital coarctation of aorta presented for balloon
angioplasty of coarctation. Angioplasty was performed with near-complete
resolution of initial pressure gradient of 37 mm Hg, but was complicated by
type B aortic dissection. Axial (B) and sagittal (C) (MIP) CT
angiograms images performed 6 years later show descending thoracic aortic
stent in true lumen (arrowheads) with stable descending thoracic
aortic and abdominal aortic dissection. Dissecting membrane (arrows,
C) terminates between celiac artery and superior mesenteric artery.
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Aneurysm Formation
Although various definitions exist for the presence of an aneurysm, most
investigators agree that it constitutes a wall contour deformity whose
diameter is 1.5 times that of the aorta at the level of the diaphragm.
Aneurysm formation occurs after both surgical and endovascular treatment
(Figs. 10A,
10B,
11A,
11B,
12,
13A,
13B, and
13C).
Aneurysm Rupture
Aneurysm rupture has been described after both surgical and endovascular
repair. This condition, with few exceptions, is fatal (Figs.
13A,
13B,
13C,
14A,
14B, and
14C).
Aortic Rupture
Both percutaneous transluminal angioplasty and stent placement can lead to
aortic rupture. The risk of death from balloon angioplasty for native
coarctation is estimated at 0.7% from the Valvuloplasty and Angioplasty of
Congenital Anomalies Registry.
Dissection
Dissection of the aorta is primarily a complication of percutaneous
transluminal angioplasty and its treatment is often stent placement to exclude
the false lumen and provide patency of the true lumen (Figs.
15A,
15B, and
15C).
Aortic Thrombosis
Aortic thrombosis has also been described after surgical and endovascular
treatment; it generally requires additional surgery (Figs.
9A,
9B, and
9C).
Conclusion
Familiarity with the appearance of the aorta after both surgical and
endovascular repair and the respective complications of both procedures is
necessary to provide accurate imaging interpretation and to direct appropriate
patient care. Both CT angiography and contrast-enhanced MR angiography allow
assessment of the pertinent vasculature in great detail, whereas MRI is
superior if functional information about hemodynamic significance and cardiac
function is needed.
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Abstract
Introduction
