Surveillance imaging should be performed at intervals similar to
what is recommended for aortic dissection.
IV. THORACIC AORTIC ANEURYSM
A. Definitions and Terminology
Aortic aneurysm is a pathologic entity that is distinct from aortic
dissection. The vast majority of aortic dissections (longitudinal
splitting of the media) occur without preceding aneurysms. True an-
eurysms result from stretching of the entire thickness of the aortic
wall; thus, the wall of an aneurysm contains all three of its layers
(intima, media, and adventitia). TAAs may involve one or more
aortic segments (the aortic root, ascending aorta, arch, or descending
thoracic aorta). Sixty percent of TAAs involve the aortic root and/or
ascending tubular aorta, 40% involve the descending aorta, 10%
involve the arch, and 10% involve the thoracoabdominal aorta.
243
In 1991, the joint councils of the Society of Vascular Surgery and
the North American Chapter of the International Society for
Cardiovascular Surgery appointed an ad hoc committee to define
the standards for reporting on arterial aneurysm.
244
Aneurysm
was defined as a permanent focal dilatation of an artery having a
$50% increase in diameter compared with the expected normal
diameter of the artery in question. This definition was also adopted
by the 2010 American College of Cardiology Foundation, American
Heart Association, American Association for Thoracic Surgery,
American College of Radiology, American Surgical Association,
Society for Cardiac Angiography and Interventions, Society of
Interventional Radiology, Society of Thoracic Surgeons, and
Society for Vascular Medicine guidelines for the diagnosis and man-
agement of patients with thoracic aortic disease.
1
Figure 47
Diagram of some of the various shapes of aortic root and ascending aortic aneurysms. (A) Normal. (B) Characteristic ‘‘mar-
fanoid’’ or ‘‘pear-shaped’’ aortic root with dilatation localized to the annulus and sinuses of Valsalva. (C, D) Two patterns of dilatation
involving the annulus, sinuses of Valsalva, and ascending aorta. (E) Dilatation beginning at the STJ, but sparing the aortic annulus and
sinuses of Valsalva.
Table 16
Goal of imaging of TAAs
1 Confirm diagnosis
2 Measure maximal diameter of the aneurysm
3 Define longitudinal extent of the aneurysm
4 Measure the diameters of the proximal and distal margins of the
aneurysm
5 Determine involvement of the aortic valve
6 Determine involvement of the arch vessel(s)
7 Detect periaortic hematoma or other sign of leakage
8 Differentiate from aortic dissection
9 Detect mural thrombus
Journal of the American Society of Echocardiography
Volume 28 Number 2
Goldstein et al 153
Although our writing committee endorses this definition in
general, we would like to point out some practical considerations.
First, the current definition lacks an outcomes correlate; second,
many publications use the term aortic dilatation with different arbi-
trary cutoff values to define the significance of dilatation.
245-248
Last, if a common echocardiographic upper limit of 37 mm is
considered for the ascending aorta (see the section on normal
anatomy and reference values), then the ascending aorta is dilated
at a diameter of >55.5 mm (37 + 18.5 mm), which seems
excessive given that it is larger than the typical threshold for
surgery. General descriptions, such as ‘‘there is an ascending aortic
aneurysm,’’ are inadequate.
243
It is preferable to state that ‘‘there is
a 5-cm ascending thoracic aortic aneurysm,’’ because it conveys
prognostic, follow-up, and management implications. Descriptive
terms such as small, large, and giant to describe aneurysms should
also be avoided.
Other commonly used terms are included in the 2010 guidelines
1
:
ectasia is arterial dilatation < 150% of normal arterial diameter.
Arteriomegaly is diffuse arterial dilatation involving several arterial
segments, with an increase in diameter > 50% in comparison with
the expected normal arterial diameter.
Aortic dilatation is an acceptable nonspecific term that encom-
passes both ectasia and aneurysm. Again, imaging reports must
include the diameters of the affected aortic segments. Moreover,
it is ideal to perform serial imaging studies at the same center
with the same technique, so that direct comparisons can be
made.
243
When this is not practical, direct comparison with previ-
ous examinations should be made to confirm that serial changes
are genuine.
B. Classification of Aneurysms
Aortic aneurysms can be classified according to morphology, location
(as above), and etiology. The etiologies of TAAs are listed in
Table 15
.
These are discussed in other sections of this document.
C. Morphology
Aneurysms of the aorta can be classified into two morphologic types:
fusiform and saccular (
Figure 46
). Fusiform aneurysms, which are
more common than saccular aneurysms, result from diffuse weak-
ening of the aortic wall. This process leads to dilatation of the entire
circumference of the aorta, producing a spindle-shaped deformity
with a tapered beginning and end. Saccular aneurysms result when
only a portion of the aortic circumference is weakened, producing
an asymmetric, relatively focal balloon-shaped out-pouching. There
are also various morphologic shapes of the aortic root and ascending
aorta, some of which suggest specific etiologies (
Figure 47
).
The major goals of imaging TAAs are listed in
Table 16
, and
recommendations for choice of imaging modalities are listed in
Table 17
.
D. Serial Follow-Up of Aortic Aneurysms (Choice of Tests)
Aortic diameter is the principal predictor of aortic rupture or dissec-
tion.
249
The risk for rupture or dissection of TAAs from different eti-
ologies increases significantly at sizes > 60 mm. The mean rupture
rate is only 2% per year for aneurysms <50 mm in diameter, rising
slightly to 3% for aneurysms with diameters of 50 to 59 mm, but
increasing sharply to 7% per year for aneurysms
$60 mm in
Table 17
Recommendations for choice of imaging modality for TAA
Modality
Recommendation
Advantages
Disadvantages
CT
First-line
First-line technique for staging, surveillance
Contrast: enhanced CT and MRI very accurate for
measuring size of all TAAs (superior to echocardi-
ography for distal ascending aorta, arch, and de-
scending aorta)
All segments of aorta and aortic branches well visu-
alized
Use of ionizing radiation and ICM
Cardiac motion can cause imaging artifacts
MRI
Second-line
Ideal technique for comparative follow-up studies
Excellent modality in stable patients
Preferred for follow-up for younger patients
Avoids ionizing radiation
Can image entire aorta
Examination times longer than CT
Benefits from patient cooperation (breath hold)
Limited in emergency situations in unstable patients
and patients with implantable metallic devices
Benefits from gadolinium
TTE
Second-line
Usually diagnostic for aneurysms effecting aortic
root
Useful for family screening
Useful for following aortic root disease
Excellent reproducibility of measurements
Excellent for AR, LV function
Distal ascending aorta, arch, and descending aorta
not reliably imaged
TEE
Third-line
Excellent for assessment of AR mechanisms
Excellent images of aortic root, ascending aorta,
arch, and descending thoracic aorta
Less valuable for routine screening or serial follow-
up (semi-invasive)
Distal ascending aorta may be poorly imaged
Does not permit full visualization of arch vessels
Limited landmarks for serial examinations
Aortography
Third-line
Reserved for therapeutic intervention
Useful to guide endovascular procedures
Invasive; risk for contrast-induced nephropathy
Visualizes only aortic lumen
Does not permit accurate measurements
LV, Left ventricular.
154 Goldstein et al
Journal of the American Society of Echocardiography
February 2015
diameter.
250
The rate of growth is significantly greater for aneurysms
of the descending aorta, at 1.9 mm per year, than those of the
ascending aorta, at 0.07 mm per year.
249
The clinical importance of the maximum aortic diameter for deter-
mining the timing of prophylactic surgical repair implies makes it critical
that measurements be made as accurately as possible. It is essential for
the same observer to compare measurements side by side using the
same anatomic references. Tomographic scans in a situation for which
the aorta does not lie perpendicular to the plane of the scan produce an
elliptical image with major (maximum) and minor (minimum) diame-
ters. Because the major diameter is typically an overestimate, in most
natural history studies of aneurysm expansion, the minimum diameter
has been reported to avoid the effect of obliquity.
Aortic root dilatation can be followed by TTE in most cases.
Diameter expansion, severity of AR, and left ventricular function
may be accurately evaluated when the echocardiographic window
is adequate. However, when dilatation involves the ascending aorta
above the STJ, TTE does not always adequately visualize the affected
segment, in which case CTor MRI should be performed. TEE may be
warranted when the type of surgical treatment (repair or valve
replacement) is being considered. Both TTE and TEE have limitations
for adequate measurement of distal ascending aorta, aortic arch, and
descending aorta diameters. In addition, if the aorta is tortuous, trans-
esophageal echocardiographic images may be difficult to measure
accurately. The multiplanar capacity of MDCT, together with its sub-
millimeter spatial resolution, renders it an excellent technique for in-
terval surveillance of both thoracic and abdominal aortic aneurysms.
Measurements must adhere to a strict protocol that permits compar-
ison between different imaging techniques as well as follow-up of the
patient. MDCT permits one to choose an imaging plane in any arbi-
trary space orientation; thus, it is possible to easily find the maximum
aortic diameter plane, which must be perpendicular to the longitudi-
nal plane of the aortic segment. When the axial data are reconstructed
into 3D images (computed tomographic angiography), one can mea-
sure the tortuous aorta in true cross-section and obtain an accurate
diameter. Measurements should be taken on multiplane reconstruc-
tion images. A further common presentation of data is a parasagittal,
oblique MIP plane that passes through the aortic root, ascending
aorta, aortic arch, and descending aorta. The MIP plane must have
a thickness proportional to the aortic tortuosity to make sure that
the maximum diameter is included in the image. This plane is easily
reproducible and comparable in follow-up studies.
MRI accurately defines aortic diameter, aneurysm extent, and the
aneurysm’s relationship with the main arterial branches. It is recom-
mended to combine MR angiographic images with black-blood spin-
echo sequences, which are useful for detecting pathology of the wall
and adjacent structures that could go unnoticed if only MR angio-
graphic images are acquired. In mycotic aneurysm, postcontrast T1-
weighted images permit the identification of inflammatory changes in
the aortic wall and adjacent fat, secondary to bacterial infection. The in-
formation provided by MRA in aortic aneurysm assessment is similar to
that offered by current MDCT. Both methods permit accurate determi-
nations of aortic diameters in sagittal plane. Furthermore, postprocess-
ing techniques (MIP, multiplane reconstruction, and volume rendering)
facilitate visualization of the aorta in its entirety, together with the rela-
tionship of its principal branches, and are highly useful when planning
treatment. The sagittal plane makes it possible to obtain more repro-
ducible measurements. In asymptomatic patients with aortic aneu-
rysms and those approaching the need for surgery, imaging
techniques should be performed at 6-month intervals until aortic size
remains stable, in which case imaging may be annual.
1. Algorithm for Follow-Up.
TTE can be used for serial imaging of
the dilated aortic root and proximal ascending aorta when agreement
between the dimensions measured by TTE and CT or MRI has been
established. When the aneurysm is located in the mid or upper
portion of the ascending aorta, aortic arch, or descending thoracic
aorta, CT or MRI is recommended for follow-up. Measurements
should be made on multiplane reconstruction images or in parasagit-
tal, oblique MIP plane that passes through the involved aortic
segments. Although annual surveillance MDCT has been recommen-
ded, the strategy is not well established and should be individualized
from annually to every 2 to 3 years depending on the abnormalities
present, history of complications among family members, the present
size, and the degree of change in size over time.
E. Use of TEE to Guide Surgery for TAAs
When patients with aortic root or ascending TAAs undergo aortic
repair, the anatomy of the aorta and aortic valve has usually been
defined preoperatively. Nevertheless, it is always wise to use intrao-
perative TEE to confirm the prior imaging findings. The initial intrao-
perative transesophageal echocardiographic examination should
begin before the initiation of cardiopulmonary bypass, so the physi-
ology of the aortic valve can be assessed. If the valve is bicuspid,
one should determine the presence and severity of associated valvular
aortic stenosis, regurgitation, or both. If there is significant AR, one
should determine the mechanism, looking specifically for prolapse
and/or retraction of the conjoined leaflet, as this is a common cause
of bicuspid AR and may be correctable with BAV repair. One should
also assess the degree of leaflet thickening, calcification, and restric-
tion, because in the setting of significant valve dysfunction, these find-
ings may influence the surgeon’s decision regarding the need for valve
repair or replacement.
Even when the aortic valve is tricuspid with otherwise normal leaf-
lets, the presence of an ascending aortic aneurysm can result in AR.
The commissures of the aortic leaflets are located just below the
STJ; dilatation of the aorta at that level may tether the leaflets, leaving
insufficient slack for the three leaflets to coapt properly in the middle,
resulting in a jet of central AR.
32,194,251
AR due to leaflet tethering can
occur with aneurysms of both the aortic root and the ascending aorta.
Fortunately, with repair of the aneurysm and restoration of normal
aortic geometry, normal leaflet coaptation is often restored, which
in turn leads to resolution of the valvular regurgitation. Therefore,
in a patient with an aneurysm with significant AR, identifying such
aortic leaflet tethering on preoperative TEE may reassure the
surgeon that aortic valve replacement is not necessary.
The ascending aorta, aortic arch, and descending aorta should each
be inspected for the presence of associated pathology, such as an unrec-
ognized aortic dissection, IMH, PAU, or protruding atheromas. Large
atheromas in the ascending aorta or arch may prompt additional imag-
ing of the aorta using intraoperative epiaortic echocardiography and in-
fluence decisions regarding the site of aortic cannulation and perfusion.
Postoperative TEE should begin as soon as the patient comes off
cardiopulmonary bypass. The examination should begin with inspec-
tion of the aortic valve, as unanticipated valve dysfunction may neces-
sitate a return to bypass. If preoperatively there had been significant
AR due to leaflet tethering, one should confirm appropriate leaflet
coaptation and alleviation of AR after repair. If repair of bicuspid valve
prolapse was performed, one should confirm that the prolapse has
resolved and that the AR is no longer significant. If a valve-sparing
root repair was performed, one should confirm that the three aortic
valve leaflets coapt normally and that there is little or no AR. If the
Journal of the American Society of Echocardiography
Volume 28 Number 2
Goldstein et al 155
aortic valve had been replaced, one should confirm that the prosthetic
leaflet or disk motion is normal, that there is no more than physiologic
AR, and that there are no paravalvular leaks.
After aortic valve replacement or aortic root replacement, it is typical
to see focal thickening around the aortic root. It is important to docu-
ment this so as not to confuse this finding with pathology on subsequent
imaging. For more detailed information, readers are referred to the
recently published Society of Thoracic Surgeons aortic valve and
ascending aorta guidelines for management and quality measures.
252
F. Specific Conditions
1. Marfan Syndrome.
Marfan syndrome is an inherited disorder of
connective tissue that occurs as a result of a mutation in the FBN1
gene, which encodes fibrillin. One of the hallmark features of this dis-
order is dilatation or dissection of the proximal ascending aorta (aortic
root).
253
The remaining portions of the aorta may also dilate and
dissect, but involvement of the aortic root is expected when there is
associated vascular disease. Noninvasive aortic imaging with subse-
quent elective aortic replacement has contributed to the dramatic
improvement in survival noted in patients with Marfan syndrome
over the past few decades.
254
a. Aortic Imaging in Unoperated Patients with Marfan Syn-
drome.–TTE is generally the initial imaging tool used for the identifica-
tion and serial follow-up of ascending aortic enlargement in patients
with known or suspected Marfan syndrome, because of its availability,
noninvasive nature, reliability, and lack of need for radiation or contrast
material. Characteristic aortic features include dilatation of the aortic
root, whereas the STJ and remaining portions of the ascending aorta
generally are normal in size (
Figure 48
). Normative values are used
to determine the presence and extent of aortic enlargement on the ba-
sis of age and BSA.
9
The leading edge–to–leading edge measurement
technique is generally performed in patients with Marfan syndrome
<18 years of age, and the size of the aorta is reported along with the
Z score.
255
Although there is dispute regarding the best echocardio-
graphic aortic measurement method, the most important concept is
that serial measurements for each individual patient are performed us-
ing the same method to determine aortic dimension change over time.
Some patients with Marfan syndrome have suboptimal transthoracic
echocardiographic images, and in these patients, serial CT or MRI is
required to monitor aortic diameter.
At the time of initial diagnosis of aortopathy in Marfan syndrome
by TTE, additional imaging with CT or MRI is generally recommen-
ded to confirm that the size of the aorta measured by TTE is accurate
and correlates with the computed tomographic or MRI measurement
and to document the diameters of the distal ascending aorta, aortic
arch, and descending aortic segments, which may also be enlarged
but are often incompletely visualized by TTE (
Figure 49
).
For the follow-up of aortic root enlargement in patients with
Marfan syndrome, follow-up imaging in 6 months is recommended.
If at that time the aortic diameter remains stable, is <45 mm, and
there is no family or personal history of aortic dissection, then annual
aortic imaging is reasonable. Patients with Marfan syndrome who do
not meet these criteria should undergo repeat aortic imaging every 6
months.
TTE can be used for serial imaging follow-up of the dilated
ascending aorta when correlation between the dimensions measured
by TTE and CT or MRI has been documented. Occasionally, patients
with Marfan syndrome do not demonstrate aortic enlargement until
well into adulthood. These patients can be referred for transthoracic
echocardiographic screening at 2- to 3-year intervals.
Repeat CT or MRI is suggested at least every 3 years in patients
with Marfan syndrome to reassess the aortic arch and descending
Figure
48
Transthoracic
long-axis
image
demonstrating
marked dilatation of the aortic root (sinuses) in a patient with
Marfan syndrome. Note the normal dimension at the STJ.
Figure 49
Computed tomographic 3D volume-rendered recon-
struction of the thoracic aorta demonstrating aortic root dilata-
tion (arrow) and proximal descending thoracic aorta dilatation
(asterisk). The descending thoracic aortic dilatation was not
noted on TTE.
156 Goldstein et al
Journal of the American Society of Echocardiography
February 2015
aorta and to reconfirm that TTE remains reliable in its measurement
of the ascending aorta. Patients with Marfan syndrome with aneu-
rysmal dilatation of the proximal descending thoracic aorta require
regular CT or MRI to monitor aortic stability, because TTE does not
provide reliable imaging of this region.
TEE is generally not used for the initial diagnosis or follow-up of
aortic dilatation in patients with Marfan syndrome because of its
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