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J. F. LaDisa Jr. et al. Chapter 37 Endovascular Treatment Strategies for Coarctation of the Aorta
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371
cise [74]. Subsequent perfusion of the coronary arteries
during diastole is also compromised, resulting in decreased flow and an increase in precursors of coronary
artery disease [75]. Reduced coronary artery perfusion
and concomitant increases in afterload may also explain
the high instance of indices associated with heart failure
in these patients [58, 76]. Relief of these deleterious hemodynamics was observed following the alleviation of
an experimental coarctation [74], suggesting that treatment strategies that optimize vascular hemodynamics
may provide the greatest chronic benefit to patients.
Hypertension is the most frequent complication associated with repair of coarctation regardless of treatment modality. In a study of patients subjected to exercise testing approximately 20 years after treatment for
coarctation by surgical repair, nearly 50% of patients
were found to have ambulatory and exercise-induced
hypertension, a finding that is commoner when treatment is obtained after 1 year of age [77]. Residual
coarctation caused by scarring at the suture site or persistent aortic arch hypoplasia after treatment may contribute to this finding [5]. Although long-term data
after stent implantation for coarctation are not yet
available, it seems possible that the presence of a rigid
stent in the compliant aorta may also cause hypertension. Persistent pathologic arterial modifications such
as increased systemic vascular resistance, aortic stiffness, elevated left ventricular contractility [43, 78] and
anatomical abnormalities of the transverse arch not
unique to a particular treatment strategy are also
thought to contribute to hypertension [79].
Coronary artery disease, cerebral aneurysms and
stroke also occur despite ªsuccessfulº coarctation repair,
indicating that the current perception of success may
be incorrect and the ongoing severity of treatment-specific hemodynamic alterations manifested in the aorta
and coronary, head and neck vessels during ambulatory
or exercise conditions may contribute to long-term
morbidity. For example, studies conducted on canine
coronary arteries have demonstrated that the compliance mismatch between a stent and a native vessel is
masked during conditions of resting blood flow, and
causes deleterious alterations in local flow patterns during maximum vasodilation [80]. Similarly, the coarctation causes drastic reductions in the capacitive function
of the aorta and there are likely hemodynamic ramifications of the compliance mismatch caused not only by
coarctation prior to surgical or catheter-based intervention at rest, but also during ambulation.
It is clear from the clinical literature that parametric
alterations within a single treatment, or relying on the
gradual empirical modification of these treatments, will
only modestly increase the life expectancies of patients
with aortic coarctation. Alternatively, more favorable
long-term results may be possible by examining the origin of coarctation symptoms that emanate from altera-
tions in vascular hemodynamics within the ascending
aorta.
Researchers in the Cardiovascular Biomechanics Research Laboratory at Stanford University, in collaboration with Departments of Pediatric Cardiology and Cardiothoracic Surgery, are currently investigating a new
paradigm to improve our understanding of the hemodynamic and physiologic conditions before and after
treatment for coarctation. This research is based on the
hypothesis that treatment strategies that optimize vascular hemodynamics at rest and during exercise will
minimize known risk factors for long-term morbidity
associated with aortic coarctation. Rather than modifying the technique of a given treatment or evaluating
strategies based on the current standards for mortality,
recoarctation, aneurysm formation and hypertension,
treatment strategies could be scrutinized according to
their ability to restore optimal hemodynamics in the ascending and descending aorta and head and neck vessels. A similar approach to treatment planning has previously been described for occlusive vascular disease in
adults [81].
Through this interdisciplinary collaboration, computer models can be created from time-resolved 3D
phase-contrast magnetic resonance imaging data obtained at rest and during lower limb exercise using a
Fig. 37.3. Average wall shear stress (WSS) in a patient with
coarctation of the aorta before (
tion. Aortic coarctation causes ascending aortic dilation and
pre- and poststenotic dilatation that is responsible for low WSS
in the arch, ascending and descending aorta and branch arteries. Most of these low WSS regions are alleviated after stent
implantation, but some areas of the aortic arch, branch vessels
and anomalous vertebral artery remain and may be deleterious
as low WSS is known to correlate with sites of atherogenesis
and vascular inflammation
a) and after (b) stent implanta-

372
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VIII. Congenital Diseases of the Thoracic Aorta
supine stationary ergometer. Treatment-specific alterations in vascular wall motion, indices of wall shear
stress and pressure wave reflection and amplification
manifested in the head and neck vessels and throughout
the aorta can then be quantified and interpreted as surrogates of the potential for morbidity (Fig. 37.3). The
results may reveal hemodynamic adaptations associated
with the acceptable systolic pressure gradient of
20 mmHg and determine if treatment-specific guidelines may be more appropriate for minimizing morbidity. In addition, the simulations can reveal 3D spatial
and temporal hemodynamic ramifications of compliance mismatch caused by the coarctation prior to intervention, and the surgical suture line or rigid stent afterward. This hemodynamic characterization process may
be amenable to predicting which treatment strategies
will be advantageous for a particular patient and to
identifying deleterious processes that lead to morbidity
decades before they are clinically apparent.
In the future, computational models may be used to
determine which strategy will benefit the patient from a
hemodynamic and physiologic perspective. If the development of these computational models based on patient-specific anatomy and physiology is successful, they
may provide the potential to increase our scientific understanding of this problem and the various treatment
options. In the long term, patient-specific modeling
may provide clinicians with a resource to decrease disease- and procedure-related morbidity and mortality.
37.5 Summary
Balloon angioplasty and stent implantation are now
widely accepted as treatment options for coarctation of
the aorta. Both of these strategies, as well as surgical repair, have advantages and disadvantages in specific patient populations. In the future, changes in stent design
and materials and better predictive models of appropriate candidates for endovascular treatment will optimize
treatment outcomes. As additional long-term data regarding procedural success, morbidity and mortality become available and surgical and transcatheter techniques progress, management strategies will also continue to evolve. As always, close collaboration between
surgeons and cardiologists will remain imperative.
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Current Multicentric Studies
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and Those to Plan
for the Descending Thoracic
Aortic Diseases
Herv Rousseau, Jean Philippe Bolduc,
Francis Joffre
Chapter
38
Contents
38.1 Introduction .......................
38.2 Descending Thoracic Aortic Aneurysms ....... 375
38.3 Dissection .........................376
38.3.1 Timing of Treatment ............... 377
38.3.2 Length of Coverage ............... 377
38.4 Trauma ........................... 377
375
38.1 Introduction
Cardiovascular disease is the leading cause of death in
most Western societies and is increasing steadily in
many developing countries. Longer life expectancy, hypertension and the proliferation of modern noninvasive
imaging modalities have contributed to the growing
awareness of acute and chronic aortic syndromes. Despite recent developments in epidemiology, diagnostic
and therapeutic modalities, there is still a lot of progress to be made to understand the spectrum of aortic
syndromes and to define an optimal approach to managing aortic diseases.
In the 1990s, endovascular stent-graft treatment
emerged as a new and less invasive method to treat abdominal aortic aneurysm. It soon led to the use of stent
grafts in the treatment of thoracic aortic diseases, but
their exact role remains approximate.
Although only midterm study results are now available, they indicate a better outcome compared with
conventional surgery, especially in elderly patients with
significant comorbidities such as pulmonary and renal
insufficiency, coronary heart disease, hypertension and
diabetes mellitus, where morbidity and mortality rates
after an open surgical repair are as high as 50%. However, despite the good published results, endoluminal
stent grafts are not risk-free: endoleaks, prosthesis dislocations, neurological complications, acute or late rup-
ture of the aorta and side branch occlusions are described leading to therapy failure. Owing to the actual
restrained number of patients treated by endovascular
repair, the blur in the indications and the different
types of devices used, it is nearly impossible to identify
if the complications are device-, procedure- or patientrelated and the exact place of this new therapy.
Nevertheless, we will attempt, in this chapter, to discuss the ongoing studies and the need for future studies
to better understand and treat the various thoracic aortic pathologies.
38.2 Descending Thoracic Aortic Aneurysms
Aneurysms of the thoracic aorta represent a potentially
life-threatening situation. Surgical resection and interposition with a vascular prosthesis have long been considered the standard treatment despite the substantial
risks of the procedure. The use of an endovascular stent
graft to treat thoracic aortic aneurysms emerged a decade ago propelled by the desire to reduce surgical risks
and induce remodeling of the diseased aorta by initiating a natural healing process after exclusion and depressurization of the aneurismal sac.
So far, all prospective studies and registers have
shown that the stent-graft technique has better immediate results compared with classic open surgery, with
lower 30-day morbidity±mortality and paraplegia rates.
In midterm studies, the complication rates are, however,
not negligible and habitually consist of secondary leaks
which can mostly be treated intravascularly [1, 2, 3].
Compared with stent-graft abdominal aortic aneurysm
repair, complications of thoracic treatment differ considerably. Abdominal complications mostly relate to
changes in aneurysmal volume after successful exclusion, which result in device distortions, kinks or modular disconnections. At the thoracic level, as only one tubular device is needed in most patients, the risks of
type III leaks, kinks, disconnections or thromboses are

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VIII. Congenital Diseases of the Thoracic Aorta
either eliminated or greatly reduced. Furthermore, the
diameter reduction after complete aneurysm exclusion
is probably less than in the abdomen combined with
the use of an oversized device (at least 10% more than
the normal aortic diameter) and this reduces risks even
more. Nevertheless, the most frequent thoracic complications are type8I endoleaks that occur at aortic and
graft junctions allowing the aneurysmal sac to remain
pressurized. They are more frequent because degenerative thoracic aortic disease is usually more diffuse than
abdominal disease; thus, progression of the malady at
attachment sites is more likely. To avoid this problem,
we recommend the placement of longer stent grafts covering healthy aorta up to the visceral arteries. Type II
endoleaks, except from the left subclavian artery, are
rare. If two or more grafts are used, type III endoleaks
can arise at junctions, requiring insertion of another
stent-graft segment. This complication is greatly reduced when we systematically overlap a long segment of
the grafts. Finally, pseudoaneurysms and intimal perforations at distal implementation sites have been reported secondary to stent-graft erosions [4]. Complete
long-standing studies are still needed to determine the
incidence of these complications and their long-term effects.
The question of intentional exclusion of the left subclavian artery is still unanswered. In patients with a
very short neck between the left subclavian artery and
the aneurysm requiring coverage of the former, different treatment attitudes have been described; left subclavian transposition or bypass either systematically before
stent-graft insertion or only if the patient has ischemic
neurological or left arm symptoms after occlusion. Left
subclavian artery coverage is routinely done without
complication in many centers [5, 6]. Nevertheless, it
should be kept in mind that it is crucial to evaluate the
vertebral arteries before occluding the left subclavian
artery to prevent ischemic symptoms in cases of stenotic vertebral arteries or absence of collateral pathways
between the two as observed in up to 6% of cases.
As devices improve, better results should be observed in the future. Therefore, requests to place endografts in patients with small lesions, in which the risk
of rupture is extremely low, should be more frequent. It
will be important to resist these demands until further
data prove otherwise. So, as far as we are concerned,
we recommend that endograft use should be limited to
patients who truly exhibit surgical indications.
38.3 Dissection
Despite the frequency of acute aortic dissection, there
are few large series published on the outcomes of dissections and most are long retrospective multicenter
studies confounded by inconsistent methods of treat-
ment and data collection. The IRAD study, a prospective multicenter registry has now been created to address some of these concerns. This study [7, 8] provides
better understanding of the clinical profile and outcomes of patients with acute type B aortic dissection,
helping clinicians in early risk stratification and decision-making. Unfortunately, there is an inherent selection bias because the study results are mainly based on
data from tertiary referral centers that may not necessarily be extrapolated to the general population. Even
though the IRAD study is a step forward, to better evaluate survival predictors, prospective studies are still
needed mainly because the actual registry does not regroup homogeneous patients with similar risk factors
whose outcomes could be rigorously compared nor does
it take into consideration factors such as nonfatal morbidity, quality of life and cost effectiveness.
Actual consensus exists regarding the need for emergency surgical treatment of patients with acute Stanford
type A aortic dissection. The optimal treatment strategy
for Stanford type B dissection remains controversial [9±
12]. Most groups today reserve the surgical replacement
of the descending aorta for patients with aortic rupture,
organ ischemia, refractory pain, uncontrolled hypertension, false lumen dilatation or other life-threatening
conditions. Other teams have advocated early surgery
for young and good operative candidates irrespective of
the presence of complications [13], arguing that if the
surgery is successful, these individuals would be at lower risk of late dissection-related aortic complications.
Finally, f percutaneous interventional techniques, i.e.,
fenestration and stent-graft repair to correct ischemic
complications related to thoraco-abdominal malperfusion, have become a valuable adjunct to both medical
and surgical therapy, but their role is still debated.
For type B dissections with complications, percutaneous stent-graft placement seems to be superior to
surgery on short-term follow-up [14±19]. Recently, it
was shown that percutaneous stent-graft treatment has
an early mortality rate of 16% among patients with
acute Stanford type B aortic dissections associated with
life-threatening complications [16]. If treated surgically,
i.e., an emergency thoracotomy, these patients would be
facing an early mortality risk of 40%. The rate was said
to be 60±70% if treated medically [11, 14, 15] The effectiveness of stent-graft treatment in patients with complicated acute type B aortic dissections must however
still be confirmed by long-term prospective randomized
trials. Such a study was started in early 2003 but regrettably had to be stopped after the intentional retrieval of
the Gore device after cases of nitinol wire fractures.
In cases of acute type B dissection without complications, medical treatment was long the only accepted
treatment until stent grafts were used successfully [17],
complicating the decision-making process. The INSTEAD study was started in Europe in 2002 to compare
medical and stent-graft treatment in patients with un-

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377
complicated acute type B aortic dissections. The aim of
this multicenter randomized controlled clinical trial is
to evaluate the 1-year outcomes, including complication
rates and quality of life, of patients with type B dissection treated either percutaneously or medically. Early
results can be expected in 2005.
Other concerns include the timing of the intervention and the length of coverage necessary to exclude
the false lumen.
38.3.1 Timing of Treatment
Stent-graft placement could become, in the near future,
the standard treatment for most cases of complicated or
uncomplicated aortic dissection mostly because the operative mortality rate approaches 70% if we wait for
complications to occur. Another argument in favor of
early endovascular treatment is the evolution of aortic
morphology with time following dissection. In acute
type B dissections, an isolated tear is more frequent
and usually no thrombus is present in the false lumen,
while in chronic dissections multiple entry and exit
points are seen along the aorta associated with thrombus formation enlarging the vessel diameter. Therefore,
delaying treatment could increase implantation failure
rate or make the intervention no longer possible [20±
22].
38.3.2 Length of Coverage
An unanswered technical question concerns the length
of aortic coverage necessary to achieve dissection healing. The key is to cover the proximal entry site to reduce pressure in the false lumen and consequently
shrink the total aortic diameter and improve flow in the
true lumen expanding the later, resolving ischemic
complications or malperfusion syndromes. Given our
results and those of others, it seems that complete
thrombosis of the false lumen is necessary to reduce
the overall aortic diameter and protect against subsequent aneurismal dilatation and rupture [23, 24]. So,
from these results combined with the fact that the risks
of neurological paraplegic complications are particularly
low in dissections treated by stent grafts, one can suggest covering a long part of the descending aorta above
the diaphragm at the time of initial implantation to exclude all entry points feeding the false lumen. Adjunctive measures to achieve complete thrombosis of the
false lumen such as use of coils or glue have also been
described. Again, long-term controlled trials are needed
to categorically guide our future therapeutic strategies.
38.4 Trauma
Despite advances in surgical and reanimation techniques, surgery is still associated with significant morbidity and mortality rates ranging between 8 and 15%
depending on whether circulatory assistance to maintain satisfactory perfusion of the distal aorta is used or
not [25]. The postoperative paraplegia rate without circulatory assistance can be as high as 19% and increases
significantly when the aorta is clamped for more than
30 min [26]. With circulatory assistance, the rate is
about 2% [25] However, the systemic anticoagulation
required for the extracorporeal circulation is often undesirable in traumatic patients with multiple fractures
and/or parenchymal or cerebral lesions.
In the last 10 years, several studies showed, for stable
and nonbleeding lesions, that surgical mortality after
aortic injury can be significantly reduced when surgical
repair is deliberately delayed [27±29]. These studies
support the fact that free rupture of a contained acute
traumatic tear of the thoracic aorta is unlikely to occur
under proper blood pressure control. Therefore, it appears safe to allow patients who suffered a major trauma to be stabilized, undergo other emergent operations
if needed and then have elective repair of the aortic
tear. Although this attitude is justified by objective data,
it is not entirely risk-free because as many as 4% of patients awaiting surgery might die of a ruptured aorta
usually within 1 week of the traumatic injury [30].
More recently, the advent of the endovascular stentgraft technology has provided a less invasive alternative
to thoracic aortic injury treatment. This substitute to
open thoracic aortic replacement is attractive for several
reasons but one of its main advantages is the possibility
to avoid heparin use when necessary, decreasing hemorrhagic complications related to associated lesions if
present.
Although some authors reserve endovascular treatment for patients for whom standard surgery is contraindicated [31], one might raise the issue of extending
the indication to all patients with traumatic injury of
the thoracic aorta. Our current experience, as that of
others, has shown encouraging results of the endovascular technique compared with those for conventional
surgery [31±41]. The benefits of aortic endoprosthesis
in terms of morbidity and mortality by far outweigh
those of classic surgery by thoracotomy. Our comparative study with similar lesions and severity scores (ISS)
confirms that stent-graft therapy is an advantageous alternative to conventional open surgery. The mortality
and the paraplegia rates were 21 and 7%, respectively,
for the 35 patients surgically treated compared with 0%
for the 29 patients treated with a stent graft [41]. With
a mean follow-up of 46 months, we did not observe any
aneurysm expansion or rupture. Complete healing of
the aortic wall without any residual pseudoaneurysm

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VIII. Congenital Diseases of the Thoracic Aorta
and total shrinking of the aorta over the stent graft
were seen in all cases.
However, controversy remains regarding the best
method of management. Studies must be carried out to
determine the precise place of endovascular treatment
in the management of acute rupture of the thoracic aorta. An ideal study would compare the outcomes of patients of similar health status subjected to conventional
surgical intervention, to stent-graft placement or to
medical treatment. Unfortunately, such a prospective
study is not feasible for ethical reasons; patients incapable of undergoing conventional surgery for any reason
should of course not be operated. Additionally, since a
small number of patients receive treatment in each center, even a multicenter randomized study comparing the
two treatment methods is illusive. A prospective registry evaluating patients considered unfit for surgical intervention because of comorbidities treated with or
without stent-graft placement would best assess the effect of the endovascular strategy compared with that of
medical treatment. In order to do so, we suggest the
creation of an international registry similar to the one
for aortic dissections to compile the results of endovascular treatment and consequently help to define its indications.
As a whole, we can actually consider that endovascular stent-graft treatment of the aorta is a less invasive
strategy for most of the thoracic aortic diseases, particularly in patients with comorbidities; however, large
prospective studies for the complete evaluation of this
new therapeutic option are still needed.
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Ten Years to Come
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Jean-Philippe Verhoye, Jean-Franois Heautot,
Alain Leguerrier
Chapter
39
In the management of thoracic aorta lesions, in contrast
to that of those of the abdominal aorta, endovascular
techniques were immediately considered not as a substitute, but rather as an adjunct to surgical techniques
whose specific morbidity (spinal, pulmonary and renal)
is still important.
Indeed, when stent-grafts came to be used to treat
abdominal aorta aneurysms, the surgical technique was
associated with a very acceptable morbidity rate, close
to 5%, and to a perioperative mortality mainly related
to myocardial infarction. The initial enthusiasm for this
new technique was directly related to this significant reduction of perioperative mortality owing to the minimal
invasivity and to the absence of aortic clamping. Today,
this is weighted by the uncertainty about midterm and
long-term durability of the aneurysm sack exclusion,
and, as an effect, by the quality of the treatment, not to
mention the rather unbalanced cost-efficacy ratio due
to follow-up imaging studies and to the management of
late complications.
The situation is quite different at the thoracic level.
Ten years have passed since the first stent-graft was deployed to treat an aortic lesion. The feasibility of this
technique is now well demonstrated and accepted, this
book having been written to state it. Regarding the thoracic aorta, the benefit of the stent-graft became progressively obvious in acute diseases (complicated type B
dissection, aortic rupture, etc.) with the idea of bridging a gap, to stabilize, if not definitely manage a situation too delicate for surgery, without hindering delayed
intervention. Evidently, to deploy is not to cure, and the
current concept of stent-grafts allows us in a minimally
invasive way, well suited to an emergency, to quickly
and safely blind an intimal tear or to restore the continuity of a ruptured aortic wall. The absence of endothelization with current stent-grafts does not allow us, today, to foresee the durability of the treatment, making
unavoidable a continuous follow-up. On the other hand,
the late results in degenerative aneurisms and chronic
type B dissections are less convincing, such as the results of abdominal aorta aneurysm endovascular repair.
Thoracic aortic stent-grafts were not as frantically
marketed as abdominal ones, and were initially limited
to three types: the first-generation Stanford homemade
stent-grafts and two industrially made ones, Medtronic's
Talent and Gore's Excluder. This allowed relatively
homogeneous international registers to be built up,
avoiding the potential bias due to excessively different
devices. This controlled maturity allows past experience
to be taken into account for clinical evaluation research
to develop new concepts, such as a better fitting to arch
lesions or related to stent coating.
Feasibility studies reported in the literature mainly
regarded four disorders: degenerative aneurysms, type B
dissections, ulcers and hematomas, and isthmus rupture. After 10 years the first midterm results have now
been published and it seems crucial for us to insist on
the need for evaluation studies based not any more on
the feasibility of the stent-graft concept, but rather on
the results related to each pathology, defining two main
groups: acute and chronic diseases, and separating the
results by pathology.
l Dissection
± Type A vs type B
± Complicated vs not complicated
l Aneurysms
± Degenerative
± Posttraumatic pseudoaneurysms
± Suturing false aneurysms
± Mycotic aneurysms
l Aortic rupture
± Isthmus
± Descending aorta
l Hematomas and penetrating ulcers
In this decade of endovascular progress which brought
about a new look at the physiopatholgy of dissection,
ulcers and hematomas, we also must insist on the fantastic complementary advances in diagnostic imaging.
The wider availability of multislice computed tomography scanner angiography has dramatically decreased
the risk of misdiagnosed posttraumatic aortic rupture,
thus lowering to nearly zero the likelihood of pseudoaneurysms in the future. Again, these acute disorders
represent, in our opinion, the best application field for
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