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Management of thoracic aortic aneurysm in adults

AUTHOR: Christopher R Burke, MD
SECTION EDITORS: James Hoekstra, MD, Heidi M Connolly, MD, FACC, FASE, Gabriel S Aldea, MD, Edward Verrier, MD
DEPUTY EDITOR: Kathryn A Collins, MD, PhD, FACS

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Apr 2023.

This topic last updated: Aug 29, 2022.

INTRODUCTION

Thoracic aortic aneurysm (TAA) can be due to one of several etiologies. The natural history of
TAA is one of progressive expansion, the rate of which depends upon the location of the
aneurysm and its underlying cause. Although most TAAs produce no symptoms, patients who
become symptomatic or have complications related to the aneurysm (eg, acute aortic
regurgitation, dissection, aortic rupture) should undergo repair [1-5]. Conservative management
of asymptomatic TAA aims to lessen stress on the aorta and limit further aortic expansion.
Asymptomatic patients who do not meet criteria for repair also require ongoing aneurysm
surveillance. Any patient with additional clinical risk factors (eg, Marfanoid habitus, positive
family history) should be evaluated for possible underlying genetic conditions known to be
associated with thoracic aortic aneurysm and dissection (TAAD). Where expertise in the
management of thoracic aortic disease is not available, the patient should be transferred to a
high-volume cardiovascular center to provide the best possible outcome [6].

The management of thoracic aortic aneurysm is reviewed here. The etiology, natural history,
clinical features, and diagnosis of TAA, as well as specific techniques for repair of the thoracic
aorta, are discussed separately. (See "Epidemiology, risk factors, pathogenesis, and natural
history of thoracic aortic aneurysm and dissection" and "Clinical manifestations and diagnosis of
thoracic aortic aneurysm" and "Endovascular repair of the thoracic aorta" and "Overview of open
surgical repair of the thoracic aorta".)
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INDICATIONS Folder
REPAIR

We agree with major cardiovascular society guidelines from the American College of Cardiology,
American Heart Association, and Society of Vascular Surgery that recommend repair for all
symptomatic thoracic aortic aneurysm (TAA; ruptured, associated with dissection, causing pain)
[1-5,7]. In general, repair of asymptomatic TAA is not recommended until the risk of rupture or
other complications exceeds the risks associated with repair. Asymptomatic TAAs are selected
for repair depending upon diameter, location, expansion rate, family history of
rupture/dissection, and the presence of associated coronary heart disease or valve pathology
requiring surgical intervention, with special considerations depending on the presence of
underlying contributing etiologies (eg, connective tissue disorders, bicuspid aortic valve, familial
thoracic aortic aneurysm/dissection). For patients who meet criteria for repair, survival is
improved for open surgery compared with medical therapy alone [4,5]. This should also be the
case for endovascular repair given that endovascular repair compares favorably with open
surgery. Asymptomatic patients with TAA who do not meet criteria for repair are managed
medically with routine surveillance. (See 'Management of asymptomatic TAA' below.)

Summary of indications — Recommendations for repair for TAA in the ascending ( table 1)
and descending thoracic aorta are summarized and discussed in detail below. (See 'Symptomatic
(nonruptured) and ruptured TAA' below and 'Asymptomatic TAA' below.)

● Symptomatic (nonruptured) or ruptured TAA.

● Asymptomatic ascending TAA:

• End-diastolic aortic diameter >5.5 cm [5,7], or aortic size index (aortic diameter [cm]
divided by body surface area [m2]) ≥2.75 cm/m2 [8] or aortic area over height ratio (ie,
maximal aortic cross sectional area [cm2] divided by height [m]) >10 cm2/m [4,5]. A body
surface area calculator can be found in the link (calculator 1). (See 'Degenerative TAA'
below and 'Accounting for body size' below.)

For patients with genetically mediated thoracic aortic aneurysm and dissection (TAAD),
including syndromic conditions such as Marfan, Loeys-Dietz, vascular Ehlers-Danlos,
and Turner syndromes, as well as nonsyndromic conditions like familial TAAD or
bicuspid aortic valve, a lower diameter or aortic index is suggested as an indicator for
repair ( figure 1). The specific threshold diameter depends on the condition. (See
'Genetically mediated thoracic aortic aneurysm and dissection' below and 'Accounting
for body size' below.)
 • For patients undergoing aortic valve surgery or coronary artery bypass grafting: End-
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diastolic aortic diameter >4.5 cm in diameter.

● Asymptomatic descending TAA:

• For most average-sized adults: Diameter of >5.5 cm [5,7].

• Patients with high surgical risk (eg, Society of Thoracic Surgeons Predicted Risk of
Mortality [PROM] score >8 percent, frailty score index >2, two compromised organ
systems [9]): Diameter ≥6 to 7 cm.

• For patients with genetically mediated conditions (syndromic or nonsyndromic), a

smaller diameter is suggested as an indicator for repair. The specific threshold diameter
depends on the condition. (See 'Genetically mediated thoracic aortic aneurysm and
dissection' below.)

• For smaller patients, including many females, a diameter greater than twice the
diameter of the nonaneurysmal aorta (normal segment) or the aortic size index can be
used. (See 'Accounting for body size' below.)

● Asymptomatic TAA with rapid expansion ≥5 mm per year for aneurysms <5 cm in diameter
[5,10].

Symptomatic (nonruptured) and ruptured TAA — Although most TAAs are asymptomatic, TAA
can produce a variety of symptoms and complications, which can be life-threatening. Chest pain
associated with TAA could represent rapid aneurysm expansion, dissection, or rupture. Chest
pain associated with evidence of acute dissection in a true thoracic or thoracoabdominal aortic
aneurysm can be the first and only sign of a TAA [11]. Others may rarely present with dysphagia
or dyspnea, usually related to compression of adjacent organs [4,5]. Patients whose symptoms
can be attributed to the aneurysm should undergo repair [4]. (See "Clinical manifestations and
diagnosis of thoracic aortic aneurysm", section on 'Symptomatic TAA' and 'Thoracic aneurysm
repair' below.)

It has been suggested that patients with untreated large ascending or descending thoracic
aneurysms are more likely to die of complications associated with their aneurysms than from
any other cause [12]. In several series of patients, aneurysm rupture occurred in 32 to 68
percent of medically treated patients, and rupture accounted for 32 to 47 percent of deaths [13-
17]. One-, three-, and five-year survival rates for unrepaired thoracic aneurysms are
approximately 65, 35, and 20 percent, respectively. After rupture (or other complications),
cardiovascular disease is the second most common cause of death among candidates who do
not undergo repair. The overall mortality associated with ruptured TAA is high. Only
approximately one half of patients with ruptured TAA live long enough to be transferred to the
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emergency department for treatment [18]. Without repair, ruptured TAA is nearly uniformly
fatal. Unfortunately, regardless of the method of repair, a large portion of patients with ruptured
TAA still do not survive [4,19].

Asymptomatic TAA — Elective repair of asymptomatic TAA may reduce the morbidity and
mortality associated with TAA complications, but elective repair of asymptomatic TAA is not
recommended until the risk of rupture or other complications exceeds the risks associated with
repair. Favorable outcomes for open or endovascular repair reinforce the need to repair TAA
before it reaches the thresholds described below (diameter, expansion rate), above which the
risk of rupture increases sharply [20,21].

The most important determinant of TAA rupture is the diameter of the aneurysm [12,22-25].
Other factors, such as a rapidly expanding aortic diameter, concomitant bicuspid aortic valve, or
connective tissue disease, also increase the risk for rupture [8]. (See "Epidemiology, risk factors,
pathogenesis, and natural history of thoracic aortic aneurysm and dissection".)

Diameter criteria — The most important determinant of TAA rupture or other complications
(eg, dissection) is the diameter of the aneurysm [16,22-24,26]. The diameter threshold to
recommend elective repair varies by the location and etiology of the aneurysm, among other
factors [16]. One group has shown that the clinical threshold for rupture mirrors the innate
physical limits of the aortic wall. As the aorta approaches 6 cm, its distensibility rapidly falls [27].
At this diameter, the aorta loses its natural elasticity and effectively becomes a rigid tube. At a
blood pressure of 200 mmHg, easily achieved through strenuous exercise or emotional distress,
the stress generated in the wall of a 6 cm aorta can attain or exceed the maximum tensile
strength of aortic tissue [27].

Degenerative TAA — For patients with degenerative TAA (ie, not associated with
genetically mediated conditions), we suggest elective repair of ascending TAA for end-diastolic
aortic diameter >5.5 cm, and elective repair of descending TAA with a diameter >5.5 cm for
patients with a low risk for perioperative complications. For higher-risk patients, a descending
aortic diameter >6 cm should be repaired [5,7,16,22-24,26]. What constitutes high risk is not well
defined. The use of the Society of Thoracic Surgeons Predicted Risk of Mortality (PROM) score >8,
frailty score >2, and more than one major organ system impairment has been used in an effort
to stratify risk for aortic surgery in those with bicuspid aortic valves [9].

Based on observational studies, the risk of complications of TAA (rupture, dissection) increases
with larger aortic diameter, in particular, once the ascending aorta expands beyond 6 cm, or the
descending aorta expands beyond 7 cm [26].
 ● An early study of 133 patients with ascending TAA, descending TAA, or both found that the
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overall five-year risk of rupture was 0 percent for those less than 4.0 cm compared with 16
and 31 percent for TAA 4.0 to 5.9 and ≥6.0 cm, respectively [22]. TAA diameter >6.0 cm was
associated with a 15.6 percent per year combined endpoint of rupture, dissection, or
death.

● In another series of 370 patients, the median diameter at the time of rupture or dissection
was 5.9 cm for ascending TAA and 7.2 cm for descending TAA; a diameter greater than 6
cm increased the risk of rupture or dissection by 25 percent for an ascending TAA, while a
diameter greater than 7 cm increased the risk by 37 percent for descending TAA [24].

● In a later prospective study that included 304 patients with mostly ascending TAA (72
percent), overall rates of dissection or rupture were 2, 3, and 7 percent per year for TAA less
than 5.0 cm, 5.0 to 5.9 cm, and ≥6.0 cm, respectively [16]. The combined endpoint of
rupture, dissection, or death overall occurred at a rate of 16 percent per year among
patients with TAA ≥6.0 cm. The overall five-year survival rate in patients with TAA >6.0 cm
was 56 percent. In patients who did not undergo surgery (diameter not specified), 54
percent were alive at five years. By comparison, elective surgery restored survival to a rate
similar to a matched control population.

● In another review of 216 patients assessed, 41 percent of patients were considered high
risk for any intervention [17]. Median TAA diameter was 6 cm. Over a median follow-up of
12 months, 49 of 81 patients died, of which 23 deaths were due to rupture; the others were
not aneurysm related.

Even though the risk of dissection or rupture increases with aortic diameter, most patients who
present with dissection have smaller-diameter aneurysms. In a review of 591 patients with type
A dissection enrolled in the International Registry of Acute Aortic Dissection (IRAD), the mean
diameter was 5.3 cm [11]. Nearly 60 percent of patients had an ascending aortic diameter <5.5
cm, and 40 percent had an aortic diameter <5.0 cm.

Clinicians should understand the limitations of basing treatment decisions solely on diameter
criteria. Data have called into question the traditional cutoff of 5.5 cm for degenerative TAA, and
some have called for potentially lowering this threshold [28]. Reasons for this include
prospective natural history studies showing a "hinge-point" for TAA complications at 5.25 cm (in
addition to previously described 5.75 cm), recognition that the aorta "increases" in diameter by 7
mm at the moment of dissection, along with increased safety of contemporary elective TAA
repair. Further work will be needed in this area to better predict those patients who would
benefit from prophylactic TAA repair at smaller diameters.
 Inflammatory TAA — Inflammatory diseases such as Takayasu arteritis, giant cell arteritis,
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Behçet disease, and ankylosing spondylitis are commonly associated with TAAD [4,5]. TAA
formation may occur at multiple sites over a period of follow-up. Initial medical therapy for
active Takayasu arteritis and active giant cell arteritis reduces the active inflammatory state.
Elective surgical repair of TAA for patients with Takayasu arteritis and giant cell arteritis should
be delayed until the acute inflammatory state is treated and quiescent. The diameter criteria for
inflammatory TAA repair are the same as those for noninflammatory, degenerative TAA [4,5].
(See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm
and dissection", section on 'Inflammatory disorders' and "Overview of the management of
vasculitis in adults".)

Genetically mediated thoracic aortic aneurysm and dissection — Genetically mediated

TAAD can be part of a syndrome (ie, syndromic) such as Marfan syndrome ( table 2), Loeys-
Dietz syndrome, vascular Ehlers-Danlos syndrome, or Turner syndrome, or nonsyndromic, as
with familial TAAD and bicuspid aortic valve [29,30]. Prophylactic surgery in the setting of many
hereditary TAA disorders is uncertain and may depend upon the specific mutation, aneurysm
location, family history, absolute aortic size, and growth rate. It is recognized that TAA due to
mutations in SMAD3, ACTA2, and MYLK may lead to dissection at relatively small aortic diameters
( figure 1). Decision making in patients with genetically mediated TAAD, particularly in younger
patients (<60 years old), takes into account the diameter of the ascending aorta/proximal arch
but also the diameter of the aortic root and aortic valve function ( table 1) [31].

Marfan patients should generally undergo elective operation at a smaller aortic diameter than
patients with degenerative aortic aneurysms to reduce the risk of TAA complications [3,4,32,33].
Elective repair is warranted for aortic root diameter ≥5.0 cm, with repair at a diameter >4.5 cm in
those with a family history of dissection, or other high-risk features suggested for those with
progressive aortic regurgitation, family history of dissection, or rapid expansion (increase by 5
mm or more per year). Elective repair at a smaller ascending aortic diameter in surgical centers
with expertise in the repair of the aortic root and ascending aorta is reasonable [3,5,34,35]. (See
"Management of Marfan syndrome and related disorders" and "Pregnancy and Marfan
syndrome".)

A decision for elective repair in patients with bicuspid-aortic-valve-associated aortic dilation

depends upon the diameter of the ascending aorta but also on the functional status of the valve
[36-40]. Although the aortic valve eventually requires replacement in over 75 percent of patients
due to progressive aortic stenosis and/or regurgitation, bicuspid aortic valve is often associated
with ascending aortic aneurysm in the absence of significant valvular disease. For some patients,
repair of TAA becomes necessary before aortic valve operation is indicated [41]. For
asymptomatic patients with bicuspid aortic valve, an ascending aortic diameter >5.5 cm
warrants repair (lower for smaller stature); however, for patients with additional risk factors for
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complications (eg, family history of aortic dissection, rapid expansion >0.5 cm/year), repair at an
ascending aortic diameter 5.0 to 5.5 cm, or >4.5 cm in those with severe aortic stenosis or
regurgitation, is reasonable to reduce the risk for complications [5]. For those at low risk for
aneurysm resection with surgery performed in a center with expertise, surgery at >5.0 cm can
be considered [9]. This recommendation is based on data from single-center observational
studies of patients who underwent aortic valve replacement for valvular dysfunction. Patients
with ascending aortic diameter of 4.0 to 4.5 cm at the time of isolated aortic valve replacement
had an increased risk of ascending aortic complications [3,42]. Another retrospective study has
also shown that the probability of aortic dissection increases dramatically at an ascending aortic
diameter of 5.3 cm and increases gradually at a sinus diameter of 5.0 cm [43]. (See "Bicuspid
aortic valve: General management in adults".)

The Loeys-Dietz syndrome has the smallest recommended diameter threshold among the
genetically mediated TAA syndromes owing to its aggressive natural history, with some
suggesting prophylactic replacement for an ascending aortic diameter between 4.0 to 4.5 cm
(including consideration for repair at a diameter >4.0 cm for females with small size and TGFBR2
mutations) [4,29,44]. (See 'Rapid expansion' below and "Management of Marfan syndrome and
related disorders", section on 'Management of related conditions'.)

Newly identified hereditary TAA disorders including mutations in SMAD3, TGFB2, TGFB3, MYH11,
and MLCK also have aortic surgery recommendations from 4.5 to 5 cm depending upon many
factors.

Accounting for body size — Absolute diameter criteria for intervention on the aorta do
not take into account natural variation in aortic diameter for sex and body size ( figure 2A-B).
As an example, a small individual (body surface area [BSA] 1.5 m2 ) with an aortic measurement
of 4.5 cm may be at a higher risk for aortic complications compared with a larger individual with
a BSA of 2.1 and the same aortic measurement. As such, smaller patients may have a smaller
diameter threshold for elective intervention due to the larger relative aneurysm diameter. The
role of body size has also been evaluated in patients with Marfan syndrome, who tend to be
taller [4].

One suggested criterion for smaller patients is to perform elective repair for aortic diameters
greater than twice the diameter of a normal segment of aorta (nonaneurysmal segment). For
smaller patients, including many females, we suggest elective repair using this criterion.

While not addressed in the later guidelines [45], some have suggested using an indexing
method that helps correct for body size. Elective ascending aortic repair is recommended before
the ascending aortic size (ASI) index (aortic diameter [cm] divided by body surface area [m2]) is
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2.75 cm/m2 [8]. Additional investigation has explored the possible importance of using height
alone, consisting of the aortic height index (AHI [cm/m]), which may be more predictive of
aneurysm complications compared with using BSA [46]. However, this approach has not been
widely adopted clinically.

Another method to account for body size suggests that for cases of Marfan syndrome, it may be
reasonable to perform elective aortic root replacement for patients with an ascending aortic
area (cm2) to height (m) ratio of 10 cm2/m [4,5,47]. This index is also being used for those with
bicuspid aortic valve-associated aneurysm [48].

Rapid expansion — Once a TAA develops, the natural history is one of progressive
enlargement with variable expansion rates [49-51]. Expansion rates range from 1 to 10 mm per
year, depending upon TAA etiology, diameter, and location within the aorta [16,17,20,49,52-55].
Reports of rapid expansion can reflect measurement errors; however, when rapid expansion is
seen, it should raise concern for aortic dissection or aortic infection [20]. Rapid expansion
increases the risk of TAA complications; thus, we suggest repair of asymptomatic ascending TAA
<5 cm that exhibits rapid expansion, defined as ≥5 mm per year [4,5,10,56]. It is important to
note that follow-up should be at the same institution, on the same scanner, so that interobserver
variability in assessing size or changes in size does not come into the decision-making process to
justify a high-risk operation.

Similar to abdominal aortic aneurysm, larger-diameter TAAs expand more rapidly than smaller-
diameter TAAs [57]. This was illustrated in a study of 67 patients with TAA who underwent serial
computed tomography (CT) [53]. The rate of expansion for aneurysms >5.0 cm in diameter at
diagnosis was 7.9 mm per year but was approximately 1.7 mm per year for aneurysms <5.0 cm.
A history of hypertension did not affect the rate of aneurysm expansion. However, these studies
are older, and the location and specific etiology of the aneurysm may not have been taken into
account. Larger-diameter aortas (>5.0 cm at the time of diagnosis) can have expansion rates up
to 15 mm/year ( figure 3).

The anatomic location of the aneurysm is another factor associated with the rate of expansion.
In a series of 87 patients who underwent vascular imaging, aneurysms located within the mid-
portion of the descending aorta showed the most rapid expansion, while those in the ascending
aorta had the slowest expansion rate, despite having a greater initial diameter ( figure 4) [52].
In general, isolated degenerative ascending aortic aneurysms have an average expansion rate of
1 mm per year, whereas descending TAAs expand by approximately 3 mm per year [57,58].
Saccular aneurysm morphology is associated with aortic infection and increased rates of
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expansion and rupture compared with fusiform. In one study, the expansion rate of saccular
aneurysms was 28 mm/year [59]. As such, saccular aneurysms are often treated more
aggressively.

Patients with familial TAAs have faster average rates of expansion at 2 mm per year (combined
ascending and descending TAA) [57,60-63]. Marfan syndrome is associated with expansion rates
of up to 3 mm per year. An increased aortic expansion rate has also been reported during
pregnancy for those with Marfan syndrome compared with those with Marfan syndrome who
are not pregnant both during and following pregnancy, in some but not all studies [64-67]. TAAs
associated with the particularly aggressive Loeys-Dietz syndrome can expand very rapidly up to
10 mm per year [44].

MANAGEMENT OF ASYMPTOMATIC TAA

Asymptomatic thoracic aortic aneurysms (TAAs) may be detected on routine chest radiograph or
during surveillance in a patient with an underlying genetically mediated condition such as
Marfan syndrome. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm",
section on 'Incidental TAA'.)

Asymptomatic patients without indications for repair are managed conservatively, which
includes the following (see 'Indications for repair' above):

● Measures to reduce cardiovascular risk. (See 'Cardiovascular risk reduction' below.)

● Aggressive blood pressure control, and other measures to limit aneurysm expansion. (See
'Therapies to limit aortic expansion' below.)

● Patient education regarding the symptoms and signs that may indicate the development of
complications. (See 'Counseling and other evaluation' below.)

● Counseling for those suspected of having genetically mediated disease. First-degree

relatives of those with TAA disease should be screened, based on family studies
demonstrating an approximately 20 percent chance of another first-degree relative having
a TAA [57,60]. (See 'Identifying associated genetic conditions' below.)

● Screening for associated aneurysmal disease. (See 'Identifying associated aneurysm'

below.)
 ● Serial imaging of the aneurysm to evaluate for expansion and changes in extent. (See
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'Aneurysm surveillance' below.)

● Counseling on exercise and activity limitations is individualized, but in general, contact

sports are avoided and most patients are counseled against heavy lifting (defined as half of
ideal body weight) and exercises involving sustained Valsalva maneuver.

Cardiovascular risk reduction — Managing cardiovascular risk is important for improving

overall outcomes, including outcomes associated with aortic repair, when it is required. Lifestyle
modification, including smoking cessation, is an important aspect to medical management in
these patients [68-70]. Although it is unknown whether statins reduce the rate of thoracic aortic
expansion, most patients with TAA have other indications for statin therapy. Statin therapy
should be used to meet low-density lipoprotein (LDL) goals as outlined by multidisciplinary
guidelines [4,5]. (See 'Statin therapy' below and "Overview of primary prevention of
cardiovascular disease" and "Prevention of cardiovascular disease events in those with
established disease (secondary prevention) or at very high risk".)

We agree with major society guidelines that advise patients with aneurysmal disease to stop
smoking; these patients should be offered cessation interventions [4]. The management of
smoking cessation is discussed in detail elsewhere. (See "Overview of vascular intervention and
surgery for vascular anomalies" and "Overview of smoking cessation management in adults".)

Cigarette smoking is strongly associated with aneurysm formation, aneurysm expansion, and
aneurysm rupture and is an important modifiable risk factor. Although the majority of studies
that have defined these associations have been in patients with abdominal aortic aneurysm
(AAA), smoking has also been linked to alterations in mechanical properties of the thoracic aorta
in animal models [71]. (See "Management of asymptomatic abdominal aortic aneurysm", section
on 'Smoking cessation'.)

Therapies to limit aortic expansion — Much of the data regarding pharmacologic therapy to
limit expansion of TAA are derived from mouse model studies, such as fibrillin-1–deficient
(Marfan) mice [68,70]. There are emerging studies on the treatment of TAA disease in humans.
But, similarly, the majority of these studies have been performed on patients with Marfan
syndrome. Whether these results can be generalized to the non-Marfan population is uncertain,
but given some shared pathophysiologic pathways, findings of these investigations may
indirectly support pharmacologic treatment (antihypertensive therapy, statin therapy) in non-
Marfan subpopulations [70,72]. (See "Epidemiology, risk factors, pathogenesis, and natural
history of thoracic aortic aneurysm and dissection", section on 'Pathogenesis'.)
 Antihypertensive therapies — Based primarily on studies performed in Marfan patients, for
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patients with asymptomatic TAA who are being conservatively managed, we suggest blood
pressure control primarily using beta blocker therapy with the aim of limiting further aortic
expansion [4,73,74]. While the theoretical benefit of anti-impulse therapy is widely accepted, no
controlled studies have demonstrated a benefit. The goal systolic pressure is 105 to 120 mmHg,
if tolerated. The adequacy of beta blockade is usually judged by the heart rate response. An
angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) is an
acceptable alternative for blood pressure control among those who do not tolerate beta blocker
therapy.

For patients with bicuspid aortic valves and a dilated ascending TAA without moderate or severe
aortic regurgitation, guidelines on the treatment of valvular disease suggest any effective
antihypertensive to control blood pressure. Beta blockers and ARBs have a conceptual
advantage but have not been shown to be effective in clinical studies [45,75]. (See "Genetics,
clinical features, and diagnosis of Marfan syndrome and related disorders".)

● Beta blockers – Beta blockers reduce the inotropic state of the heart, decreasing left
ventricular contractility (dP/dt) and shear stress, and the impact force of ejected blood on
the aorta [76]. A clinical benefit has been demonstrated only in studies of Marfan patients
[77-79]. It is not known if these results can be extrapolated to patients without Marfan
syndrome, but it is biologically plausible to attempt such therapy. In a 10 year study in
which 70 patients with Marfan syndrome were randomly assigned to propranolol or no
beta blocker therapy, the treated group had a 73 percent slower rate of aortic expansion
and lower mortality after the first four years of follow-up [77]. A retrospective
nonrandomized study of beta blocker use in children with Marfan syndrome also
demonstrated a slowing of aortic expansion in patients treated with beta blockers
compared with untreated controls [79]. However, some studies suggest that beta blockers
do not protect against TAA expansion [52,70,80].

● ACE inhibitors and ARBs – Experimental research suggests a role for the renin-angiotensin
pathways in the formation of aneurysms [79,81-83]. Studies on ACE inhibitors and ARBs
have reported conflicting results [70,84-90]. ACE inhibition may have beneficial effects by
modifying the inflammatory mediators and by decreasing vascular smooth muscle
apoptosis [86]. ARBs may also be important in preventing aneurysm expansion in Marfan
syndrome due to downregulation of transforming growth factor beta (TGF-beta) and its
effects [87,88]. Losartan may have a protective effect by antagonizing the activity of
transforming growth factor (TGF) [87]. In a nonrandomized study of young patients with
Marfan syndrome and aortic root dilation, ARB therapy significantly reduced the rate of
aortic expansion [87]. Another study reported no benefit of an angiotensin-receptor
 blocker (Losartan) over a beta blocker (atenolol) with respect to the rate of aortic-root
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dilation in patients with Marfan syndrome [89]. One interpretation of this study is that ARBs
are as effective as beta blockers in the treatment of patients with Marfan syndrome;
however, such an interpretation assumes that beta blockers are an effective treatment
option. (See "Management of Marfan syndrome and related disorders", section on 'Drug
therapy'.)

Statin therapy — Although mostly studied in the context of AAA [91-94], statin therapy may
provide a protective effect by inhibiting matrix metalloproteinases (MMPs) and plasminogen
activator [95]. In a Marfan mouse model, pravastatin was found to attenuate aortic root
aneurysm formation [96]. In one retrospective review of TAA patients, mortality rates were
significantly lower among those taking statins compared with those who were not (20 versus 33
percent); this effect was independent of angiotensin blockade [97]. Survival was attributed to a
decreased need for surgical repair due to reduced aortic expansion. In another retrospective
review, statin therapy was associated with a significant reduction in complications, including
aortic dissection [95,98].

Other pharmacologic therapies — There are no data reporting a benefit for any other
pharmacologic therapy for limiting aortic expansion for TAA in humans.

Doxycycline also reduces matrix metalloproteinase (MMP) activity, and thus elastin destruction,
and has been shown to prevent aortic root aneurysm in fibrillin-1-deficient mice [99]. Small
studies using doxycycline have demonstrated attenuation of AAA expansion in human subjects.
(See "Management of asymptomatic abdominal aortic aneurysm", section on 'Doxycycline'.)

Avoidance of fluoroquinolones — Fluoroquinolones have been linked to an increased risk of

aortic dissection and aneurysm rupture [100]. The US Food and Drug Administration (FDA) has
issued a safety advisory that this class of drugs should be avoided in patients with aortic
aneurysm or who are at risk for aortic aneurysm [101]. (See "Epidemiology, risk factors,
pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on
'Rupture/dissection' and "Management of asymptomatic abdominal aortic aneurysm", section
on 'Fluoroquinolone use'.)

Aneurysm surveillance — Asymptomatic patients who do not meet the diameter criteria for
elective TAA repair (open or endovascular) require long-term aneurysm surveillance. Aneurysm
surveillance includes ongoing clinical evaluation for the development of symptoms and signs of
aneurysm complications, and serial imaging to evaluate the diameter and structure of the
aneurysm. The preferred imaging technique depends on the location of the aortic aneurysm;
options include echocardiography, computed tomographic (CT) angiography, or magnetic
resonance (MR) angiography. Ideally, the serial studies should be performed using the same
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technique in the same center for consistency with future comparisons [102]. If a TAA is only
moderate in size and remains relatively stable over time, MR rather than CT is reasonable to
minimize the patient's radiation exposure [4,5].

In general, we suggest imaging at six months after the initial diagnosis to ensure the stability of
the aneurysm diameter and extent [4,51,56]. Further imaging can be performed annually if there
is no expansion or extension. However, the surveillance schedule may be modified based upon
the etiology, site, and diameter of the aneurysm at presentation. If the aorta demonstrates rapid
expansion or is approaching the threshold for surgery or endovascular repair, we suggest more
frequent imaging at every three to six months.

A proposed approach to imaging these patients was adapted from guidelines on the
management of thoracic aortic disease [4].

● Degenerative aortic root or ascending aortic aneurysm:

• 3.5 to 4.4 cm: Annual CT or MR angiography, echocardiogram to follow valvular disease

(if needed)

• 4.5 to 5.4 cm: Biannual (every six months) CT or MR angiography, echocardiogram to

follow valvular disease (if needed)

● Genetically mediated aortic root or ascending aortic aneurysm:

• 3.5 to 4.4 cm: Annual echocardiogram, CT, or magnetic resonance imaging (MRI)

• 4.5 to 5.0 cm: Biannual (every six months) echocardiogram, CT, or MRI

● Descending aortic aneurysm:

• 4.0 to 4.9 cm: Annual CT or MR angiography

• 5.0 to 6.0 cm: Biannual (every six months) CT or MR angiography

When an aortic root or ascending aortic aneurysm is present, transthoracic echocardiogram

should be performed to evaluate for the presence of a bicuspid aortic valve (BAV), one of the
most common causes of a dilated aorta [3,5]. It is also important to consider body size when
evaluating the significance of aortic root dimensions [4]. (See 'Accounting for body size' above.)

COUNSELING AND OTHER EVALUATION

Identifying associated genetic conditions — Patients identified as having thoracic aortic
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aneurysm/dissection (TAAD) should be evaluated for possible underlying genetic or familial
disorders known to be related to TAAD, which may increase their individual risk of aneurysm
progression or complications. The evaluation should include a thorough family history to
evaluate for sudden or unexplained death and history of aneurysm or dissection. (See
"Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and
dissection", section on 'Genetic predisposition'.)

TAA can occur as part of complex genetic syndromes, such as Marfan syndrome (most common),
Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and Turner syndrome. However, increasing
numbers of genetic loci have been linked to nonsyndromic familial TAAD and suggest that the
true proportion of familial TAADs is approximately 20 percent [103,104].

Young age at presentation is an important clue that a patient has genetically mediated TAAD. In
a retrospective review at a single institution of 760 patients between 16 and 60 years of age
diagnosed with aortopathy, differences in presentation and outcomes were studied [105]. The
etiology of genetically mediated TAAD was nonsyndromic TAA in 311, Marfan syndrome in 221,
and bicuspid aortic valve disease in 228. The average age was 37. Marfan patients were younger
than nonsyndromic TAA and bicuspid aortic valve patients. However, a symptomatic
presentation with aortic dissection was more common for nonsyndromic TAA compared with
Marfan syndrome or bicuspid aortic valve. (See "Clinical manifestations and diagnosis of thoracic
aortic aneurysm", section on 'Determining TAA etiology'.)

If one or more first-degree relatives of a patient with known TAAD are found to have thoracic
aortic dilatation, aneurysm, dissection, an abdominal aortic aneurysm, or brain aneurysm, then
referral to a geneticist is recommended. First-degree relatives of those with TAA disease should
be screened, based on family studies demonstrating an approximately 20 percent chance of
another first-degree relative having a TAA [57,60]. Family members (siblings, children) may also
require screening. For those with a positive family history, genetic testing can be based on
phenotypes most commonly associated with TAA, when they are present ( algorithm 1) [106].
A panel of genes can be checked in those patients who do not have clinical characteristics that
suggest a specific syndrome.

The 2010 American College of Cardiology (ACC)/American Heart Association (AHA) guidelines for
the diagnosis and management of patients with thoracic aortic disease recommend the
following [4]:

● Sequencing of genes ( table 3) (eg, TGFBR1, TGFBR2, MYH11, among others) known to
cause familial thoracic aortic aneurysms and/or dissection may be considered in patients
 with a family history and clinical features associated with mutations in these genes. When
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sequencing is not informative, duplication/deletion testing may be warranted.

● If the mutant gene with aortic aneurysm and/or dissection is identified in a patient, first-
degree relatives should undergo counseling and genetic testing. Then, only the relatives
with the genetic mutation should undergo aortic imaging.

● Sequencing of the ACTA2 gene is reasonable in patients with a family history of thoracic
aortic aneurysms and/or dissections.

Inheritance patterns for familial bicuspid aortic valve associated with TAA are consistent with an
autosomal dominant pattern with incomplete penetrance [107]. Recommendations for
screening are discussed separately. (See "Clinical manifestations and diagnosis of bicuspid aortic
valve in adults", section on 'Genetics'.)

Identifying associated aneurysm — Thoracic aortic aneurysm is associated with aneurysmal

disease affecting other vascular beds. This association is likely related to a common
pathophysiology, which is distinct from that of the ascending aorta [108]. As such, we have a low
threshold to screen TAA patients for abdominal aortic aneurysm (AAA), intracranial aneurysm,
and aneurysms affecting the lower extremities (iliac, femoral, popliteal). For patients with Loeys-
Dietz syndrome, head-to-pelvis imaging is recommended upon diagnosis and periodically
thereafter. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic
aneurysm and dissection", section on 'Pathogenesis'.)

AAA is present in approximately 20 percent of patients diagnosed with TAA [109]. In an imaging
review, AAA was more positively associated with arch and descending TAAs, particularly those
located near the diaphragm [109]. The associations of lower extremity aneurysms with AAA and
TAA are reviewed elsewhere. (See "Clinical features and diagnosis of abdominal aortic
aneurysm", section on 'Imaging asymptomatic patients' and "Iliac artery aneurysm" and
"Popliteal artery aneurysm".)

TAA is also associated with intracranial aneurysm (ICA). In a review of 212 patients with TAA, the
prevalence of ICA was 9.0 percent, which was ninefold greater than that in the general
population [110]. (See "Screening for intracranial aneurysm".)

Counseling the high-risk patient — Asymptomatic patients with TAA meeting criteria for repair
who have severe medical comorbidities who are judged to be high risk for repair (open surgical
or endovascular) may be best managed conservatively, reserving repair only if symptoms
develop (including rupture).
Patient with Marfan syndrome who are pregnant, bicuspid aortic valve, Ehlers-Danlos syndrome,
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and Loeys-Dietz syndrome have an increased risk for complications related to TAA [111].
Counseling females with TAA regarding the risks of pregnancy and determining the timing of
repair relative to planned pregnancy is discussed separately. (See 'Symptomatic (nonruptured)
and ruptured TAA' above.)

● (See "Pregnancy and Marfan syndrome".)

● (See "Bicuspid aortic valve: Management during pregnancy".)

● (See "Overview of the management of Ehlers-Danlos syndromes", section on 'Reproductive

options and pregnancy'.)

Recommendations for activity and sports participation among patients with connective tissue
disorders, including Marfan syndrome, are discussed separately. (See "Management of Marfan
syndrome and related disorders", section on 'Restriction of strenuous activity'.)

THORACIC ANEURYSM REPAIR

Prophylactic repair of thoracic aortic aneurysm (TAA) for appropriate diameter/expansion criteria
is recommended to prevent the morbidity and mortality associated with aneurysm
rupture/dissection. The five-year survival rate after elective open surgical repair of a TAA in
contemporary series is approximately 85 percent. Emergency surgery for TAA complications has
much worse outcomes, with a five-year survival rate of 37 percent [16]. There are no trials
comparing endovascular repair versus medical management, but long-term survival is expected
to be similar to open repair. (See 'Open versus endovascular repair of descending TAA' below.)

The timing of repair for a particular individual with TAA is individualized since the natural history
is variable, particularly for aneurysms <5.0 cm in diameter [16], and the majority of patients have
concomitant cardiovascular disease that increases the risks associated with surgery. Many
patients die of other cardiovascular causes before the aneurysm ruptures. (See 'Indications for
repair' above.)

Where expertise in the management of thoracic aortic disease is not available, the patient with
TAA should be referred (or transferred for symptomatic patients) to a high-volume
cardiovascular center to provide the best possible outcome [6].

Prior to TAA repair, the patient should undergo cardiovascular risk assessment. These issues are
discussed in detail separately. (See "Evaluation of cardiac risk prior to noncardiac surgery".)
In addition:
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● Preoperative assessment should include evaluation of left ventricular function, valve
disease, and potential concomitant coronary artery disease.

● Patients with ascending aortic and particularly arch disease should undergo carotid artery
duplex ultrasound examination. (See "Evaluation of carotid artery stenosis".)

● Patients with descending thoracic aortic disease who require left thoracotomy should
undergo pulmonary function testing if clinical symptoms of pulmonary disease are present.
(See "Overview of pulmonary function testing in adults".)

● Patients with TAA that extends distally should be assessed for symptoms and signs of
peripheral artery disease. If present, noninvasive lower extremity pulse-volume recordings
and pressures will determine the level and severity of disease to serve as a baseline for
future comparison. (See "Noninvasive diagnosis of upper and lower extremity arterial
disease".)

Approach to repair — Ascending TAA is managed with an open surgical approach using
cardiopulmonary bypass and sometimes requires aortic root replacement with coronary artery
reimplantation. Descending TAA can be repaired with an open or endovascular approach, or a
combination of the two (hybrid repair).

● Open surgery – The nature of open surgery varies depending upon the location and extent
of the aneurysm. Open repair of ascending TAA is performed via a median sternotomy with
cardiopulmonary bypass including cardioplegia and often requires aortic root replacement
or coronary artery reimplantation. The diseased aortic segment is typically replaced with
either a composite graft (ie, aortic valve replaced) or valve-sparing procedure [112]. For
bicuspid aortic valve, the valve can be replaced or repaired at the time of aortic surgery
[113]. When the aortic arch is involved, hypothermic circulatory arrest needs to be
instituted typically with antegrade or retrograde cerebral perfusion. The aortic arch can be
repaired via a hemi-arch technique or total arch replacement. Open repair of descending
TAA is via a left thoracotomy and often does not require full cardiopulmonary bypass or
cardioplegia; however, measures to protect the spinal cord are important. End-organ
revascularization (visceral, renal) may require native arterial reimplantation with or without
endarterectomy, or bypass grafting. (See "Overview of open surgical repair of the thoracic
aorta".)

● Endovascular repair – Thoracic endovascular aneurysm repair involves the placement of

modular graft components delivered via the iliac or femoral arteries to line the descending
 thoracic aorta and exclude the aneurysm from the circulation. Endovascular repair requires
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fulfillment of specific anatomic criteria, and the greatest experience involves treatment of
descending TAA that does not involve the abdominal visceral segment. The role of stent-
grafting for extensive thoracoabdominal aortic disease, which requires debranching
procedures or specialized grafts (fenestrated, branched endografts), is an area of active
clinical research. Although endovascular repair is associated with lower perioperative
mortality, late complications, including graft migration and aortic rupture, have been
reported. Newer generation endografts specifically designed for use in the aortic arch,
both single-branch and multi-branch, are in clinical trials for use in arch pathology. (See
"Endovascular repair of the thoracic aorta".)

● Hybrid repair – A hybrid approach involves typically an open approach to manage the
ascending aorta or aortic arch, with an endovascular approach for the descending thoracic
aorta (ie, frozen elephant trunk) [114-122]. (See "Overview of open surgical repair of the
thoracic aorta".)

Open versus endovascular repair of descending TAA — The choice of approach to repair
(open versus endovascular) for descending TAA should take into account the etiology
(degenerative, genetically mediated, infectious), location in the descending aorta, and extent of
the aneurysm, and the patient's expected survival (short-term and long-term), which depends
upon the patient's age and medical comorbidities.

Degenerative versus genetically mediated aneurysm — Thoracic aortic aneurysm/dissection

can be degenerative or related to genetically mediated disorders (nonsyndromic or syndromic).
The etiology of descending aneurysmal disease determines the preferred approach to repair.
(See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Determining
TAA etiology'.)

● For patients with degenerative descending TAA (asymptomatic, symptomatic) that does
not involve the visceral segment, and anatomy that is otherwise suitable for endovascular
repair, we use an endovascular rather than open surgical approach to repair. Endovascular
repair is associated with reduced perioperative morbidity and mortality; however, the long-
term durability of endovascular TAA repair compared with open surgical repair remains
uncertain. Endovascular repair combined with a debranching procedure may still be an
option for distal TAA but cannot generally be performed in an urgent setting given the
need for customized endovascular devices. (See 'Thoracic aneurysm repair' above and
"Endovascular repair of the thoracic aorta" and 'Elective repair' below and 'Emergency
repair' below.)
 ● For patients with syndromic descending TAA, we use an open surgical approach to replace
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the descending thoracic aorta, rather than endovascular stent-graft placement.
Unfavorable late aortic remodeling at the site of stent-graft placement has been reported,
likely related to the persistent radial force of these devices against the abnormal aortic
tissue. However, some feel that stent-grafting may be justified in emergencies as a
"bridging" method to later definitive surgical repair, though elective repair has also been
reported [123]. The optimal approach for those with nonsyndromic TAA is still unknown as
there are no large data sets evaluating stent grafting in nonsyndromic TAA. Therefore,
depending on the extent of repair needed, patient age, and other comorbidities, either
approach may be appropriate. (See "Endovascular repair of the thoracic aorta", section on
'Contraindications'.)

Elective repair — There are no randomized trials comparing endovascular with open surgery
for the repair of descending TAA, but the bulk of the evidence from observational studies argues
for an endovascular rather than open approach in those patients who are candidates for both,
largely due to the 30 day morbidity and mortality benefit [124-126]. Long-term durability
continues to be the main concern as the rate of secondary endovascular interventions following
thoracic stent-grafting ranges from 10 to 32 percent, while the rate of secondary open operation
ranges anywhere from 0.4 to 7.9 percent [127].

Systematic reviews of observational studies have found significantly reduced rates of

perioperative morbidity and mortality in the short term (odds ratio [OR] 0.36; 95% CI 0.23-0.58
[124]), and reduced length of hospital stay for an endovascular compared with an open
approach [124-126]. The risk of major neurologic injury was also lower for the endovascular
approach (OR 0.39; 95% CI 0.25-0.62) [124].

● In a prospective study comparing thoracic aortic aneurysm endovascular repair with open
repair, perioperative events, including myocardial infarction, respiratory events such as
pneumonia or ventilation for more than 24 hours, stroke, and paraplegia, were combined
into a composite score [128]. The percentage of patients who experienced at least one
event was significantly lower in the endovascular group compared with open surgery (9
versus 33 percent).

● In two smaller studies, each of which included around 230 patients, perioperative mortality
was significantly lower with endovascular repair (2.1 versus 11.7 percent early after
surgery, and 1.9 versus 5.7 percent at 30 days) [128,129].

● Patients in the study cited above were followed for five years [130]. Aneurysm-related
mortality was lower for endovascular repair (2.8 versus 11.7 percent), most of which was
 attributable to fewer perioperative deaths. Major adverse events, defined as prolongation
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of treatment, new hospitalization, major disability, or death, were also lower (58 versus 79
percent). No significant differences were seen for survival (68 versus 67 percent) or the rate
of aneurysm-related reintervention (3.6 versus 2.1 percent).

Nevertheless, there remain concerns over the validity of these comparisons due to the following
concerns [131]:

● Many studies compare endovascular repair with older surgical techniques or operations
with greater extents of repair [132]. In a later single-center retrospective study of over 700
patients who received either endovascular repair or open surgery, mortality was not
significantly different at 30 days (5.7 versus 8.3 percent, respectively) and 12 months (15.6
versus 15.9 percent, respectively) [133].

● Many patients enrolled in endovascular repair studies were not eligible for open surgery
due to comorbidities.

● Many patients enrolled in open surgery studies include patients who would not be eligible
for endovascular repair because of anatomic constraints [134].

Emergency repair — Patients treated for symptomatic TAA (eg, rupture, dissection) have
increased perioperative mortality. Among this subpopulation, endovascular repair of
symptomatic/ruptured TAA is also associated with improvements in perioperative morbidity and
mortality [135-139]. The outcomes of endovascular versus open surgery in the emergency
setting were specifically addressed in a nonrandomized study of 60 consecutive patients with
acute rupture of the thoracic aorta; 28 patients were treated surgically and 32 were treated with
an endovascular stent-graft [139]. The following findings were reported:

● Perioperative mortality was significantly lower with the endovascular approach compared
with open surgery (3.1 versus 17.8 percent).

● At a mean follow-up of 36 months, four additional deaths occurred in patients who

received stent-grafts; three were attributed to late procedural complications (one
aneurysm, one dissection, and one traumatic transection). No additional procedure-related
deaths occurred in the surgical patients.

● Reintervention rates were similar between the groups. All of the surgical reinterventions
were early in the postoperative course for bleeding. In the patients treated with stent-
grafts, one required early drainage of an empyema, and two required late interventions for
endovascular leaks (one repeat stent, complicated by paraplegia; one surgical repair with
stent-graft removal).
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SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Aortic and other
peripheral aneurysms".)

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics."
The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading
level, and they answer the four or five key questions a patient might have about a given
condition. These articles are best for patients who want a general overview and who prefer
short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading
level and are best for patients who want in-depth information and are comfortable with some
medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print
or e-mail these topics to your patients. (You can also locate patient education articles on a
variety of subjects by searching on "patient info" and the keyword(s) of interest.)

● Basics topics (see "Patient education: Thoracic aortic aneurysm (The Basics)")

SUMMARY AND RECOMMENDATIONS

● Patient evaluation – Patients diagnosed with thoracic aortic aneurysm (TAA) should be
evaluated for possible underlying genetic syndromes known to be related to thoracic aortic
aneurysm and dissection (TAAD). The patient should also be evaluated for other associated
aneurysms (eg, brain, abdominal aorta, mesenteric, iliac, femoral, or popliteal arteries)
using computed tomographic (CT) angiography or ultrasound. Among symptomatic
patients, this evaluation is obtained postoperatively. (See 'Management of asymptomatic
TAA' above and 'Identifying associated genetic conditions' above and 'Identifying
associated aneurysm' above.)

● Repair of symptomatic TAA – Patients who develop symptoms attributable to TAA should
undergo urgent repair (open surgical, endovascular), provided the risk for repair is not
prohibitive. Symptoms in a patient with TAA (known or unknown) like chest pain can
 represent rapid aneurysm expansion or be due to a variety of life-threatening
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complications, including aortic dissection, acute aortic regurgitation, aortic leakage, or
overt aortic rupture. (See 'Symptomatic (nonruptured) and ruptured TAA' above and
"Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on
'Symptomatic TAA'.)

● Surveillance and medical management of asymptomatic TAA – Most TAAs produce no

symptoms; however, the natural history of TAA is one of progressive expansion, which
depends upon the location and diameter of the aneurysm and its underlying etiology.
Aneurysm diameter is the most important factor determining the risk for TAA
complications.

• Patients with asymptomatic TAA should be followed for the development of signs and
symptoms that may be associated with the TAA. The surveillance schedule is based
upon the etiology, site, and diameter of the aneurysm at presentation, and expansion
rates identified at follow-up. Ideally, serial CT or magnetic resonance (MR) angiography
studies should be performed using the same imaging technique at the same center.
(See 'Aneurysm surveillance' above.)

- Following the initial study that identified TAA, we suggest imaging six months after
the initial study to ensure the stability of the aneurysm diameter and extent.

- If the TAA does not demonstrate expansion after six months, we suggest annual
imaging.

- If the TAA demonstrates expansion or is approaching the diameter threshold for

repair, we suggest imaging every three to six months.

• For patients with asymptomatic TAA who are being conservatively managed, control of
hypertension is recommended to limit further aortic expansion. We suggest beta
blocker therapy rather than other agents (Grade 2C). The goal systolic pressure is 105 to
120 mmHg, if tolerated. An angiotensin-converting enzyme (ACE) inhibitor or
angiotensin receptor blocker (ARB) for blood pressure control is an acceptable
alternative for those who do not tolerate beta blocker therapy. Most patients have
concomitant cardiovascular disease; thus, managing cardiovascular risk factors (eg,
smoking cessation, antiplatelet therapy, statin therapy) is also important. Statin therapy
may also reduce adverse outcomes related to TAA. (See 'Antihypertensive therapies'
above and 'Management of asymptomatic TAA' above.)
● Repair of asymptomatic TAA – Elective repair of asymptomatic TAA is not undertaken until
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the risk of rupture or other complications exceeds the risks associated with repair.
Candidates for repair are selected based upon diameter, location, expansion rate, and
patient comorbidities, accounting the presence of underlying contributing etiologies. For
patients who do not meet the criteria below, we suggest not performing elective repair
(Grade 2C). These patients are followed conservatively and are considered for repair if
symptoms develop. (See 'Indications for repair' above and 'Summary of indications' above
and 'Counseling the high-risk patient' above.)

For most patients with asymptomatic TAA, we use the following thresholds as criteria for
elective repair. (See 'Summary of indications' above.)

• Ascending TAA:

- End-diastolic aortic diameter >5.5 cm or aortic size index (aortic diameter [cm]
divided by body surface area [m2]) ≥2.75 cm/m2 [8] or aortic area over height ratio
(maximal aortic cross sectional area [cm2] divided by height [m]) >10 cm2/m. A body
surface area calculator can be found in the link (calculator 1).

- For patients with genetically mediated TAAD, including syndromic conditions such
as Marfan, Loeys-Dietz, vascular Ehlers-Danlos, and Turner syndromes, and
nonsyndromic conditions like familial TAAD or bicuspid aortic valve, a lower
diameter or aortic size index is suggested as an indicator for repair.

- Patients undergoing aortic valve surgery or coronary artery bypass grafting: End-
diastolic aortic diameter >4.5 cm in diameter at the time of aortic valve surgery.

• Descending TAA:

- For most average-sized adults: Diameter >5.5 cm.

- Patient with high surgical risk: Diameter >6 cm.

- For patients with genetically mediated conditions (syndromic or nonsyndromic), a

lower diameter (eg, 4.0 to 6.0 cm depending on the condition) or aortic size index is
suggested as an indicator for repair. (See 'Genetically mediated thoracic aortic
aneurysm and dissection' above.)

- For smaller patients, including many women, a diameter greater than twice the
diameter of the nonaneurysmal aorta (normal segment) or aortic size index can be
used. (See 'Accounting for body size' above.)
 • Rapid expansion ≥10 mm/year for aneurysms <5.0 cm in diameter. For patients with
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associated genetically mediated conditions, a lower expansion rate (eg, >5 mm/year) is
suggested as an indicator for repair.

● Approach to TAA repair – The approach to TAA repair (open, endovascular) takes into
account the location and anatomic extent of the aneurysm, etiology, and the patient's
expected survival (short-term and long-term), which depends upon the patient's age and
medical comorbidities. (See 'Thoracic aneurysm repair' above and "Endovascular repair of
the thoracic aorta" and "Overview of open surgical repair of the thoracic aorta".)

• Ascending TAA is traditionally managed with an open surgical approach using

cardiopulmonary bypass and often requires aortic root replacement and/or coronary
artery reimplantation.

• Descending TAA can be repaired with an open or endovascular approach, the choice of
which depends upon whether the etiology is degenerative or genetically mediated. For
patients with degenerative descending TAA, we suggest an endovascular approach for
initial repair rather than an open approach, provided the thoracic aortic anatomy is
suitable for endografting (Grade 2C). Endovascular repair is associated with reduced
perioperative morbidity and mortality; however, the long-term durability of
endovascular TAA repair compared with open surgical repair remains uncertain. An
open approach should be used for those with syndromic TAAD. The approach for those
with nonsyndromic TAAD is less well defined.

ACKNOWLEDGMENT

The UpToDate editorial staff acknowledges Emile R Mohler, III, MD (deceased), who contributed
to an earlier version of this topic review.

The UpToDate editorial staff acknowledges Y Joseph Woo, MD and Christina L Greene, MD, who
contributed to an earlier version of this topic.

Use of UpToDate is subject to the Terms of Use.

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Topic 8189 Version 33.0
GRAPHICS
My Research Folder

Indications for ascending and arch aortic aneurysm repair

Aneurysm type/associated condition Aneurysm diameter

Sporadic (not associated with disease below)

Ascending ≥5.5 cm

Isolated arch aneurysm ≥5.5 cm

Marfan syndrome (MFS), familial thoracic aortic aneurysm/dissection (FTAAD), others*

Without risk factors ¶ Δ ≥5.0 cm

With risk factors ¶ Δ ≥4.5 cm

Loeys-Dietz syndrome (LDS)

Without risk factors ¶ Δ ≥4.5 cm

With risk factors ¶ Δ ≥4.0 cm

Bicuspid aortic valve (BAV)

Without risk factors ¶ Δ ≥5.5 cm

With risk factors ¶ Δ ≥5.0 cm

Concomitant aortic valve procedure ≥4.5 cm

The aortic diameter values provided in the table are those suggested by expert consensus.
Determining the optimal timing for elective TAA repair can be challenging and requires clinical
judgment taking into account the patient's age, comorbidities, the rate of aortic expansion, other
indications for surgery (eg, aortic valve pathology), and body habitus, among others. In addition, not
every syndrome has been identified or characterized genetically, so there may be young individuals
who do not obviously have degenerative TAA and will require an individualized approach. Refer to the
discussion in the UpToDate topics discussing TAA.

TAA: thoracic aortic aneurysm; EDS: Ehlers-Danlos syndrome; TGFBR2: transforming growth factor
beta receptor 2.

* Others include Turner syndrome and other non-BAV congenital conditions. It is unclear when to
intervene for EDS; some suggest surgery for acute events only.

¶ Risk factors for aortic complications include family history of dissection, progressive aortic
regurgitation, rapid expansion (increase by 0.5 cm or more per year). For LDS, additional risk factors
include female sex and TGFBR2 mutations.

Δ For those with rapid expansion (increase by 0.5 cm or more per year), a lower diameter may be
warranted (eg, 5.0 cm for sporadic TAA).
 My Research Folder
References:
1. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the
diagnosis and management of patients with Thoracic Aortic Disease: A report of the American College of Cardiology
Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery,
American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for
Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and
Society for Vascular Medicine. Circulation 2010; 121:e266.
2. Hiratzka LF, Creager MA, Isselbacher EM, et al. Surgery for aortic dilatation in patients with bicuspid aortic valves: A
statement of clarification from the American College of Cardiology/American Heart Association Task Force on Clinical
Practice Guidelines. J Am Coll Cardiol 2016; 67:724.
3. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document
covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the
Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2873.

Graphic 115163 Version 4.0

 My Research
Ascending Folder
aorta dimensions for prophylactic surgical intervention

Simplified schematic illustration of ascending aorta dimensions for prophylactic surgical intervention divided
gene category: ECM genes, SMC contractile unit and metabolism genes, and TGF-beta signaling pathway gen
dimension for repair of sporadic aneurysm is shown for reference.

ECM: extracellular matrix; SMC: smooth muscle cell; LDS: Loeys-Dietz syndrome; MFS: Marfan syndrome; EDS
Danlos syndrome.

Adapted with permission from: Brownstein AJ, Ziganshin BA, Kuivaniemi H, et al. Genes associated with thoracic aortic aneurysm and d
An update and clinical implications. Aorta (Stamford) 2017; 5:11. Copyright © 2017 AORTA.

Graphic 115223 Version 2.0

 My Research
Clinical Folder
features of Marfan syndrome and implications for management

Organ system Features that may be present Management implications

Cardiovascular Aortic root aneurysm Monitor the thoracic aortic diameter

Aortic regurgitation
Aortic dissection
Mitral valve prolapse and mitral
regurgitation
and aortic regurgitation to identify
indications for elective aortic root
repair.*
Monitor mitral regurgitation for
indications for mitral valve
intervention.
Treat with beta blocker or
angiotensin receptor blocker
therapy.
Avoid calcium channel blockers and
fluoroquinolones.
Review activity restrictions.
Provide preconception counseling
about maternal risks during
pregnancy.
Manage pregnancy by a high-risk
maternal-fetal medicine, cardiology,
and/or multidisciplinary pregnancy
heart team.*

Skeletal Tall stature Treat scoliosis with bracing and

Facial changes (dolichocephaly, surgical correction if indicated.
enophthalmos, downslanting Surgically correct severe pectus
palpebral fissures, small jaw) deformities or arthropathies if
High-arched palate indicated.
Kyphoscoliosis Provide shoe inserts or orthotics.
Arachnodactyly
Pectus excavatum or pectus
carinatum
Flat feet
Joint hypermobility

Eye Lens dislocation Provide ophthalmologic

Flat cornea surveillance.
Hypoplasia of the iris or ciliary Provide eye care including timely
muscles vision correction for myopia and
Early myopia photocoagulation for retinal tears
and detachment.
Glaucoma
 Risk for retinal detachment
My Research Folder
Neurologic Ectasia of the dural sac Manage symptoms of back pain,
headaches, and leg pain as needed.

Lungs Emphysematous changes and Have a low threshold for evaluating

bullae for pneumothorax.
Spontaneous pneumothorax Provide a definitive surgical
procedure for frequent
pneumothoraces (generally surgical
pleurodesis).

Skin Striae distensae Consider mesh for hernia repair if

Inguinal or incisional hernias needed.

Genetics Autosomal dominant Provide genetic counseling and

Inherited in three-fourths; de novo
in one-fourth
testing for family members.
Provide preconception counseling
(if possible; if not, as soon as
possible after pregnancy is
established).

The features most likely to shorten survival are those affecting the cardiovascular system. All features
may not be present in all individuals. Some of these features are included in diagnostic criteria and
others are not. Management is done by a genetics expert, specialist with expertise in MFS and related
disorders, and/or multidisciplinary team. Refer to UpToDate for details.

MFS: Marfan syndrome.

* Refer to UpToDate for details of aortic imaging and pregnancy management, including
prepregnancy evaluations for vascular changes and dural ectasia, imaging during pregnancy,
medical therapies that are appropriate and can be used during pregnancy, prenatal testing, delivery,
and postpartum management.

Graphic 131337 Version 1.0

 My Research
Ascending Folder
aorta dimensions in women

5 th and 95 th percentiles based upon echocardiographic leading-edge to leading-edge diameters of the sinus
Valsalva and ascending aorta in 984 women with normal cardiac findings on transthoracic echocardiogram.

Reproduced from: Biaggi P, Matthews F, Braun J, et al. Gender, age, and body surface area are the major determinants of ascending ao
dimensions in subjects with apparently normal echocardiograms. J Am Soc Echocardiogr 2009; 22:720. Illustration used with the perm
of Elsevier Inc. All rights reserved.

Graphic 97883 Version 3.0

 My Research
Ascending Folder
aorta dimensions for men

5 th and 95 th percentiles based upon echocardiographic leading-edge to leading-edge diameters of the sinus
Valsalva and ascending aorta in 815 men with normal cardiac findings on transthoracic echocardiogram.

Reproduced from: Biaggi P, Matthews F, Braun J, et al. Gender, age, and body surface area are the major determinants of ascending ao
dimensions in subjects with apparently normal echocardiograms. J Am Soc Echocardiogr 2009; 22:720. Illustration used with the perm
of Elsevier Inc. All rights reserved.

Graphic 97882 Version 3.0

 My Research
Expansion Folder
rates of thoracic aortic aneurysm

Segments with initial diameter ≥60 mm manifested a median

expansion rate of 4 mm/year, while smaller aneurysms (<50 mm
diameter) had a median expansion rate of 1.33 mm/year (p<0.01).

Data from Bonser, RS, Pagano, D, Lewis, ME, et al, Heart 2000; 84:277.

Graphic 59360 Version 2.0

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Thoracic aorticFolder
aneurysm expansion by location

Linear expansion rate is shown according to segment level; aneurysms

located in the mid portion of the thoracic aorta (segments D and E)
have the greatest rate of expansion, while those in the ascending aorta
(segments A to C) had the slowest rate of growth.

Data from Bonser, RS, Pagano, D, Lewis, ME, et al. Heart 2000; 84:277.

Graphic 51157 Version 2.0

 My Research
Genetic testingFolder
in thoracic aortic aneurysm or dissection

Several issues are still open about genetic testing, in particular in sporadic TAADs. In familial TAADs, existing
disease genes provides the basis for implementing disease-specific diagnostic work-up and genetic testing. A
technologies for DNA sequencing NGS platforms will rapidly modify the current scenario and increase the se
specifically diagnosed on the basis of the disease genes. In parallel, clinical phenotypes associated with muta
different disease genes will be confirmed.

TAADs: thoracic aortic aneurysms and dissections; DNA: deoxyribonucleic acid; NGS: next generation sequen
Dietz syndrome; PDA: patent ductus arteriosus; AOS: aneurysm-osteoarthritis syndrome.

Original figure modified for this publication. Arbustini E, Narula N. Extra-aortic identifiers to guide genetic testing in familial thoracic a
dissections syndromes: it is all about the company one keeps. J Am Coll Cardiol 2012; 60:404. Illustration used with the permission of E
reserved.
My Research Folder
Graphic 105596 Version 1.0
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Gene Folder
sequencing thoracic aortic aneurysm

Syndrome Gene

MFS FBN1

LDS TGFBR1 – TGF-beta receptor 1

TGFBR2 – TGF-beta receptor 2
SMAD3 – SMAD family member 3
TGFB2 – TGF-beta 2 ligand
TGFB3 – TGF-beta 3 ligand

vEDS COL3A1 – Type III procollagen

FTAAD ACTA2 – Smooth muscle alpha-2-actin

MYH11 – Smooth muscle myosin heavy chain
TGFBR2 – TGF-beta receptor 2
MYLK – Myosin light chain kinase
PRKG1 – Type I cGMP-dependent protein kinase regulating smooth muscle cell
relaxation

MFS: Marfan syndrome; LDS: Loeys-Dietz syndrome; vEDS: vascular Ehlers-Danlos syndrome; FTAAD:
familial thoracic aortic aneurysm and dissection; cGMP: cyclic guanosine monophosphate.

Adapted from: Goyal A, Keramati AR, Czarny MJ, et al. The genetics of aortopathies in clinical cardiology. Clin Med Insights
Cardiol 2017; 11:1.

Graphic 115164 Version 1.0

Contributor Disclosures
My Research Folder
Christopher R Burke, MD Consultant/Advisory Boards: Artivion [Aortic root educational course]. All of the
relevant financial relationships listed have been mitigated. James Hoekstra, MD No relevant financial
relationship(s) with ineligible companies to disclose. Heidi M Connolly, MD, FACC, FASE No relevant
financial relationship(s) with ineligible companies to disclose. Gabriel S Aldea, MD No relevant financial
relationship(s) with ineligible companies to disclose. Edward Verrier, MD No relevant financial
relationship(s) with ineligible companies to disclose. Kathryn A Collins, MD, PhD, FACS No relevant
financial relationship(s) with ineligible companies to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.

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