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aortic dissections after surgical repair.

197

After type A dissection

repair, patent falselumen in the descending aorta is linked to survival

at 5years. Thus, use of intraoperativeTEE to direct elimination of the

entry tear, not just repairing the ascending aorta, is of great impor-

tance.

c. Partial False Luminal Thrombosis.–Studies have shown that

completely thrombosed false lumens have improved outcomes,

whereas patent falselumens carryan increased risk for aortic expan-

sion and death.

197,210

However, in the IRAD series, partial

thrombosis of the false lumen, deﬁned as the concurrent presence

of both ﬂow and thrombus and present in a third of patients, was

the strongest independent predictor of follow-up mortality, with a

2.7-fold increased risk for death compared to patients with patent

falselumen without thrombus formation.

199

Prospective studies us-

ing CTor MR for assessing the whole aorta are required to conﬁrm

these results.

d. Entry TearSize.–The prognostic value of entrytear size was eval-

uated by Evangelista et al.,

211

who documented that a largeentrytear

is astrong predictor of latemortalityand of theneed foraortic surgical

treatment. An entrytearsize$ 10mmwasanoptimalcutoff valuefor

predicting dissection-related adverse events, with sensitivity of 85%

and speciﬁcityof 87%. TEE and CTare superior to MRI in the assess-

ment of entrytear size and location. Recently it has been shown that

agreement between entryteararea by3DTEE and CTisexcellent.

151

When the entry tear is small, the ﬂow volume that enters the false

lumen is low, and thus the false luminal pressures will be low.

Therefore, the combination of a large entry tear and indirect signs

of high pressure of the false lumen, distinguishable by imaging tech-

niques, should be considered a predictor of aortic enlargement and

adverse events and warrants close follow-up.

e. True Luminal Compression.–True luminal compression is an

indirect sign of high false luminal pressure. However, true luminal

compression assessment may be limited by intimal ﬂap movement

during the cardiac cycle, as wellaslocalfactorssuch asin spiraldissec-

tion, thatmayreducereproducibilityof thisﬁnding. Patientswith clear

overall true luminal compression have a higher risk for rapid false

luminal enlargement and further aortic complications.

10. Follow-Up Strategy. After discharge, follow-up by CTor MRI

is indicated depending on technique availability, preferentialinforma-

tion sought, and patient characteristicssuch as age, renalfunction, and

test tolerance at 3, 6, 12 months and annually thereafter.

C. IMH

1. Introduction. Advances in aortic imaging technology, including

TEE, CT, and MRI, have led to increasing recognition of aortic IMH

among patients with AAS. IMH, generally considered to be a variant

of aortic dissection, accounts for approximately 10% to 25% of AAS

(Table10). IMH was ﬁrst described in 1920 as ‘‘dissection without

intimal tear’’

212

and was believed to result from rupture of the vasa

vasorum, allowing bleeding between the elastic lamina of the aortic

media.

79,212,213

However, recent ﬁndings suggest that at least some

IMHs may be initiated by small intimal tears that are undetectable

by current aortic imaging modalities and are often overlooked on

gross inspection of the aorta at the time of surgery or autopsy.

214-217

IMH is not a single entity but can be associated with several

conditions, including spontaneous (‘‘typical’’) aortic dissection,

penetrating ulcer, aortic trauma, and iatrogenic dissection (cardiac

catheterization, cardiac surgery).

2. Imaging Hallmarks and Features. The imaging hallmarks of

classicaortic dissection—thepresenceof a dissection ﬂapand thepres-

ence of adoublechannelaorta—areboth absent inIMH(Figure38)In

addition, there is usually no reentry site. General imaging features of

IMHarelisted inTable11. Typically, IMHappears as thickening of the

Figure 42 CTA illustrating an IMH. . The arrow points to o the

crescent-shaped thickened walldue tothe IMH. Note the lumen

(L) is preserved.

Figure 41 1 Thisdiagramillustratesthedifferentappearancesofthehemiazygossheath(anormalstructure),atheroscleroticplaque,

and an aneurysm containing a mural thrombus from an IMH.

Journal of the AmericanSociety of Echocardiography

Volume 28 Number2

Goldstein et al 149

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aortic wall > 0.5 cm in a crescentic or concentric pattern (Figures39

and 40).Asmentioned,amobiledissectionﬂapisabsent.Theaortic

lumen’s shape is preserved, and the luminal wall is curvilinear and

usually smooth, as opposed to a rough, irregular border seen with

aortic atherosclerosis and penetrating ulcer, although both may

coexist. There is no Doppler evidence of communication between

the hematoma and the true lumen, but there may be some color

Doppler ﬂow within the hematoma. There may also be areas of

echolucency within the aortic wall hematoma. IMH is generally a

more localized process than classic aortic dissection, which typically

propagates along the entire aorta to the iliac arteries. IMH may

weaken the aortic wall and either progress outward with aortic

expansion and/or rupture or inward with disruption of the

intima-media, resulting in typical aortic dissection. Evangelista et al.

described seven evolution patterns: regression,progression to classical

dissection with longitudinal propagation, progression to localized

dissection, development of fusiform aneurysm, development of

saccular aneurysm, development of pseudoaneurysm, and persis-

tence of IMH. Therefore, serial imaging is necessary to rule out pro-

gression in patients who receive only medical treatment, because

clinical signs and symptoms cannot predict progression. Although

thereareno established guidelines fortheoptimalfrequencyand lon-

gitudinal duration for surveillance imaging of patients with IMH,

Evangelista et al.,

154

on the basis of the signiﬁcant dynamic evolution

of IMH, recommended imaging at 1, 3, 6, 9, and 12months fromthe

time of diagnosis. Once stability has been documented, surveillance

imaging may be annual.

IMHs present a more difﬁcult diagnostic challenge than typical

aortic dissections because of the lack of both ﬂow and an oscillating

dissection ﬂap. Because IMH thickness maybeprogressive, establish-

ingthe diagnosisofIMHmayrequire observation timeand repeat im-

aging. Evangelista et al.

218

demonstrated that the initial imaging test

results were negative in >12% of patients and that a repeat study

wasrequired hours to severaldays later. IMHcan bedifﬁcult to distin-

guish from a thrombosed false lumen of classic aortic dissection,

because they can both appear as a crescent-shaped thickening of

the aortic wall. However, in aortic dissection, the diameter of the

thrombosed false lumen is usually larger than that of most, but not

all, IMHs. Conversely, the circumferential extent of an IMH is usually

larger than that of an aortic dissection. The appearanceof a crescent-

shaped thickening of the aortic wall can be mimicked by a normal

structure, the hemiazygos sheath, which is a periaortic fat pad.

219

This fat pad is typically present on TEE when the tip of the probe is

30 to 35 cm from the incisors. Aortic atherosclerosis results in

thickening of the aortic wall but produces an irregular intraluminal

surface that differentiates it from IMH, which has a smooth luminal

surface. Moreover, the‘‘lumpy-bumpy’’ appearanceof atherosclerosis

tends to vary along each centimeter of the aorta, unlike IMH, which

tends tobesmooth overa greaterlength of theaorta. Muralthrombus

mayappearlining a TAA, most often inthedescending aorta, but typi-

callyhas an irregularluminalsurface, narrowsthelumen and doesnot

extend longitudinally as much as IMH. Figure 41 illustrates the

different features of severalof these entities fromIMH.Aortitiscauses

thickening of the aortic wall that is typically concentric and typically

has normal segments interspersed between the involved sites.

Detection of IMH by CTshows thickening of the aortic wall with

higher attenuation than intraluminal blood (from 40 to 70

Hounsﬁeld units) on contrast-enhanced CT (Figure42). It is vitally

important to perform unenhanced CT as the ﬁrst step in the

computed tomographic imaging evaluation of a suspected AAS,

because contrast material within the lumen may obscure the IMH.

On imaging follow-up of IMH, the appearance of ulcerlike projec-

tions (ULPs) is frequently observed, likely representing intimal rup-

tures that allow communication between the aortic lumen and the

medial wallhematoma.

220

MRIoffersthe possibilityof diagnosing in-

tramural bleeding in the hyperacute phase because the hematoma

shows an isointensesignalon T1-weighted images and a hyperintense

signal on T2-weighted images. From the ﬁrst 24 hours, the change

from oxyhemoglobin to methemoglobin determines a hyperintense

signal on both T1-and T2-weighted images that togetherwith fat sup-

pression is useful fordifferentiating periaortic fat fromIMH. Although

greater availability and shorter examination duration favorthe use of

Table 12 2 IMH:keypoints

 IMHrepresents hemorrhage into medial layerof aorta

 Absence of dissectionﬂap between adouble-channel aorta

 Crescenticorconcentric thickening of aorticwall

 Can progress to localized orfrank dissection or rupture

 IMHthickness and maximal aorticdiameterpredict risk for pro-

gression

 CT appearance is high-attenuationeccentric or concentric wall

thickening on noncontrast image

 Subtle wall thickening can be missed at inexperienced centers

Figure 43 3 Diagramillustratingthecharacteristicfeaturesofa

penetrating atherosclerotic ulcer: presence of severe athero-

sclerosis and penetration of the ulcer or ‘‘outpouching’’ into

the media.

Figure 44 Gross pathology specimen from a a patient with h a

ruptured penetrating atherosclerotic ulcer (small arrow) associ-

atedwithIMHand blood externaltothe aortic wall(large arrow).

150 Goldstein et al

Journal of the American Society of Echocardiography

February 2015

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CTin theacutesetting,MRI maybecomplementaryfor the diagnosis

of IMH. The greater contrast among tissues can allow MRI to detect

even small IMHs that may go unnoticed by CT. In addition, mural

thrombi in TAAs are easier to distinguish from IMH by MRI because

mural thrombus shows a hypo- or isointense signal in both T1- and

T2-weighted sequences.

3. Imaging Algorithm. CT may be considered the ﬁrst-line diag-

nostic imaging modality for IMH, particularly in the acute setting.

Detection is based on the high-attenuation signal of acute bleeding

bynoncontrast enhancement. Whenﬁndings on CTorTEE are equiv-

ocal, MRImaybevaluable, as thehyperintensesignalin theaorticwall

can facilitate a correct diagnosis.

4. Serial Follow-Up of IMH (Choice of Tests). As above, IMH

may evolve by reabsorption, aneurysm formation, or conversion to

classic dissection.

154

In one series, IMH regressed completely in

34%, led to aneurysm in 20% and pseudoaneurysm in 24%, and

progressed to aortic dissection in 12%. Because of their wide ﬁeld

of vision allowing the identiﬁcation of landmarks, MRI and CT are

superior to TEE for deﬁning this dynamic evolution. On surveillance

imaging of IMH, the appearance of ULP is frequently observed, and

such ulcers may rupture and allow communication between the

medial hematoma and the aortic lumen.

221,222

MRI offers the

possibility of diagnosing the intramural bleeding evolution and new

asymptomatic intramural rebleeding episodes.

5. Predictors of Complications. Most of thesepredictorsmaybe

deﬁned by imaging techniques:

1. Maximumaorticdiameterintheacutephase isoneof the majorpredictors

of progression in type B IMH and therefore should be reported. Patients

with maximum aortic diameters > 40 to 50 mm have a higher risk for

dissection,regardlessof thelocationwithinthe descendingaorta.

216,223-229

2. Wall thicknesshas beendescribed as a predictorof progression; however,

not all studieshave supported thisﬁnding. Thethicknessthresholdforpre-

dicting progression has beenvariably reported to be 10, 12, or 15 mm.

228

3. Theincidence of periaortichemorrhageorpleuraleffusionishigherinIMH

than in aortic dissection; in some studies, this incidence reaches to 40%.

Some seriesrelatedpleural effusion to worse prognosis in IMH; however,

this remains controversial. Two mechanismshave beendescribed: leakage

of blood from the aorta through microperforations or a nonhemorrhagic

exudate believed to be inﬂammatory in origin owing to the proximity of

the IMH to the adventitia.

210,230

The likely different prognostic

signiﬁcance of the two pathogenic theories proposed may explain the

discordance inthe medical literature.

Key points related to IMH are listed inTable12.

D. PAU

1. Introduction. The term penetrating aortic ulcer describes the con-

dition in which ulceration of an atherosclerotic lesion penetrates the

aortic internal elastic lamina into the aortic media (Figure 43).

231

Although the clinical presentation of PAU is similar to that of classic

aortic dissection, PAU is considered to be a disease of the intima

(i.e., atherosclerosis), whereas aortic dissection and its variant (IMH)

are fundamentally diseases of the media (degenerative changes of

the elastic ﬁbers and smooth muscle cells are paramount), and most

patients with aortic dissection typically have little atherosclerotic dis-

ease. PAUs may occur anywhere along the length of the aorta but

appear most often in the mid and distal portions of the descending

thoracic aorta, and they are uncommon in the ascending aorta,

arch, and abdominal aorta.

232

PAUs aresometimes multifocal, which

is to be expected because aortic atherosclerosis is a diffuse process.

They mayoccurin an aorta of normalcaliber but are more often pre-

sent in aortas of increased diameter.

232-235

Typically, when an ulcerpenetratesintothemedia, alocalized IMH

develops (Figure44). In most patients, this IMH is localized, but occa-

sionally it can involve the entire descending aorta.

223

Rarely, the

medial hematoma ruptures back into the aortic lumen, resulting in

aclassic-appearingdissection with ﬂowin both lumens.Onceformed,

PAUs may remain quiescent, but the weakened aortic wall may

provide a basis for saccular, fusiform, or false aneurysm

formation.

231,233,224-227

External rupture into the mediastinum or

right or left pleural space may occur but is uncommon.

233,234

Embolization of thrombus or atherosclerotic debris from the ulcer

to the distal arterial circulation may also occur.

2. Imaging Features. The diagnosis of PAU requires demonstra-

tion of an ‘‘ulcerlike’’ or ‘‘craterlike’’ out-pouching in the aortic wall

(the internal elastic lamina is not visible on imaging studies), as seen

inFigure45. Because protrusion through the internal elastic lamina

cannot beidentiﬁed, PAUs can bedetected only when they protrude

outsidethe contour of theaortic lumen. Atheromatous ulcers that do

not enter themediamaybehard to distinguish fromPAUs. Therefore,

adiagnosis of PAU must be made with caution, especially if the sus-

pected aortic defect has been detected incidentally.

Another entity that may be mistaken for a PAU is a ULP that

may evolve from an IMH, as described above. These are localized,

Figure 45 5 Transesophageal echocardiogram m from a patient

witha penetrating atherosclerotic ulcer (arrow). Note the prom-

inent aortic atheroma (not labeled).

Table 13 3 PAUs:imagingparameterstoreport

Lesion Location

Lesion width,length, depth

Aorticdiameterat the level of thelesion

Presence/absence/extent of IMH

Contrast extension beyond/outside aortic wall

Mediastinal hematoma

Pleural effusion

Presence and length of falselumen

Journal of the AmericanSociety of Echocardiography

Volume 28 Number2

Goldstein et al 151

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blood-ﬁlled pouches that protrude into the IMH, with a wide

communicating oriﬁce of >3 mm. ULPs are felt to be the conse-

quence of a focal dissection and a short intimal ﬂap resulting in

asmall pseudoaneurysm. Differentiation from a PAU may be difﬁ-

cult. Generally, a PAU has jagged edges, is accompanied by multi-

ple irregularities in the intimal layer, and may be accompanied by

localized hematoma.

TEE, CT, and MRI mayalldetect PAU and its complications. Once

identiﬁed, attention should be directed to assessing (1)the maximum

depth of penetration of the ulcer, measured from the aortic lumen;

(2) its maximum width at the entry site; and (3) the axial length of

the associated medial hematoma. Observations that should be re-

ported are listed inTable13.

3. Imaging Modalities. a. CT.–Thetypicalcomputed tomographic

ﬁnding of a PAU is a localized contrastlike out-pouching of the aortic

wall communicating with the lumen. Its appearance has been likened

toa‘‘collarbutton.’’

228

Asmentionedabove, PAUsaremostoftenfound

in themid ordistaldescendingthoracic aorta.

226

Thickening(enhance-

ment) of theaortic wall externalto sites of intimalcalciﬁcation suggests

localized IMH. These ﬁndings are usually in conjunction with severe

atherosclerosis. CT has certain advantages over TEE. It can examine

areas of theaorta not covered byTEE, allowing morecomplete identi-

ﬁcation of theout-pouching produced by PAUs. Moreover, it can also

identify calciﬁed atherosclerotic plaques surrounding the ulcer.

Computed tomographic angiography is also more likely than TEE to

demonstrate extraluminal abnormalities, including pseudoaneurysm

or ﬂuid in themediastinum or pleural space.

b. MRI.–MRI is excellent for detecting focal or extensive IMHs,

which appear as areas of high signal intensity within the aortic wall

on T1-weighted images.

79,229

Yucel et al.

236

demonstrated that

MRI is superior to conventional CT for differentiating acute IMH

from atherosclerotic plaque and chronic intraluminal thrombus.

MRI has the additional advantage of providing multiplane images

without the use of contrast material.

c. TEE.–TEE hasbeen lesswellstudied than CTandMRI forthediag-

nosis of PAU but maybeof valuewhen the results of CTand MRI are

inconclusive. The characteristic ﬁnding, similar to what is seen on CT

and MRI, isa craterlikeout-pouchingof theaortic wall, often with jag-

ged edges, usually associated with extensive aortic atheroma.

Although uncommon, a localized aortic dissection may occur, but

the dissection ﬂap, if present, tends to be thick, irregular, nonoscillat-

ing, and usuallyof limited length.

237

Thereason for thelimited length

of the dissection may be that the dissection plane is lost because of

scarring oratrophyof themedia and secondaryto theatherosclerotic

process.

d. Aortography.–Catheter-based aortography is rarely performed

to diagnose PAU because of the superiority of current axial imaging

modalities and the high deﬁnition of TEE. These modalities also

Table 14 4 RecommendationsforchoiceofimagingmodalityforPAU

Modality

Recommendation

Advantages

Disadvantages

First-line

 Superior to TEEfor detecting PAU, especially small PAUs

 Permits assessment of entire aortaand other thoracic

structures

 Detects extraluminal abnormalities betterthan TEE (e.g.,

pseudoaneurysm, mediastinal ﬂuid)

 Follow-up by CT recommended

 Ionizing radiation exposure and iodinated

contrast material

MRI

Second-line

 Provides multipleimages without contrast

 Excellent fordetecting associated IMH complicating PAU

 Excellent fordifferentiating primary IMHfrom atheroscle-

rotic plaque and intraluminal thrombus

 Less widely available than CT

 Operator dependent

TEE

Third-line

 Differential diagnosis between PAUand ULP

 Less well studied than CT orMRI

 Semi-invasive

 Operator dependent

Table 15 5 EtiologiesofTAAs

1. Marfan syndrome

2. BAV-related aortopathy

3. Familial TAA syndrome

4. Ehlers-Danlos syndrome typeIV(vascular type)

5. Loeys-Dietz syndrome

6. Turner syndrome

7. Shprintzen-Goldberg (marfanoid-craniosynostosis) syndrome

8. Noninfectious aortitis (e.g., GCA, TA, nonspeciﬁc arteritis)

9. Infectious aortitis (mycotic syndrome)

10. Syphilitic aortitis

11. Trauma

12. Idiopathic

Figure 46 Diagram illustrating the two o morphologic typesof

aortic aneurysms: saccular and fusiform.

152 Goldstein et al

Journal of the American Society of Echocardiography

February 2015

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provide superior deﬁnition of the surrounding wall, making identiﬁ-

cation of associated IMH easier. The characteristic aortography

ﬁnding, a contrast medium–ﬁlled out-pouching resembling an ulcer

of the gastrointestinal tract, is typically associated with ‘‘cobbleston-

ing’’ of the aortic wall in the region of the ulcer consistent with

diffuse atherosclerosis, in the absence of a dissection ﬂap or false

lumen.

4. Imaging Algorithm. CTA is considered the ﬁrst-line diagnostic

imaging modality.

226,234,238-240

It is widely available, permits

assessment of other thoracic structures, and provides 3D

reconstructed images that are essential in planning surgery or

TEVAR. Moreover, CT is superior to TEE for detecting small ulcers.

It is also efﬁcient for the evaluation of the degree of ulcer

penetration and bleeding into or outside the aortic wall. MRI is

excellent for differentiating PAUs from IMH, atherosclerotic plaque,

and intraluminal chronic mural thrombus.

241,242

However, MRI is

less widely available than CT and is unable to detect displacement

of intimal calciﬁcation, which frequently accompanies PAU.

Recommendations for choice of imaging modalities for PAUs are

summarized inTable14.

Despite differences in opinion regarding the natural history and

management of PAUs, there is agreement that all PAUs, even those

found incidentally, warrant close clinical and imaging follow-up, usu-

ally by CTA. Findings concerning for progression include an increase

in aortic diameter or wall thickening or the appearance of a thin-

walled saccular aneurysm. Rupture is indicated by the presence of

extra-aortic blood.

5. Serial Follow-Up of PAU (Choice of Tests). The natural

history of PAU is unknown. As with IMH, several outcomes have

been described. Many patients with PAUs do not need immediate

aortic repair but do requireclosefollow-upwithserialimagingstudies,

by CT or MRI, to document disease progression. Although many

authors have documented the propensity foraortic ulcers to develop

progressive aneurysmal dilatation, the progression is usually slow.

Spontaneous complete aortic rupture is uncommon but may occur.

SomePAUs arefound incidentally, in which casesizeand progressive

enlargement are the only predictors of complications. Both CT and

MRI provide superior assessment to TEE in the follow-up of PAU.

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 themedia) occurwithout preceding aneurysms. Truean-

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

aorticsegments (theaorticroot, ascending aorta, arch, or descending

thoracic aorta). Sixty percent of TAAs involvetheaortic 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

bythe2010American Collegeof 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 forthediagnosis and man-

agement of patients with thoracic aortic disease.

Figure47 Diagramofsomeofthevariousshapesofaorticrootandascendingaorticaneurysms.(A)Normal.(B)Characteristic‘‘mar-

fanoid’’ or ‘‘pear-shaped’’ aortic root withdilatation localized tothe annulus and sinuses of Valsalva. (C, D) Twopatterns of dilatation

involvingthe annulus, sinusesof Valsalva, andascending aorta. (E) Dilatation beginningatthe STJ,butsparingthe aortic annulus and

sinuses of Valsalva.

Table 16 6 GoalofimagingofTAAs

1 Conﬁrm diagnosis

2 Measure maximal diameterof theaneurysm

3 Deﬁne longitudinal extent of the aneurysm

4 Measure the diameters of the proximal and distal margins of the

aneurysm

5 Determine involvement of theaortic valve

6 Determine involvement of thearch vessel(s)

7 Detect periaortic hematomaor othersign of leakage

8 Differentiate from aorticdissection

9 Detect mural thrombus

Journal of the AmericanSociety of Echocardiography

Volume 28 Number2

Goldstein et al 153

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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 areincluded in the 2010guidelines

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 beclassiﬁed according to morphology, location

(as above), and etiology. The etiologies of TAAs are listed inTable15.

These are discussed in other sections of this document.

C. Morphology

Aneurysms of theaorta can beclassiﬁed 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 (Figure47).

The major goals of imaging TAAs are listed in n 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 7 RecommendationsforchoiceofimagingmodalityforTAA

Modality

Recommendation

Advantages

Disadvantages

First-line

 First-line technique forstaging, surveillance

 Contrast: enhanced CT and MRI very accurate for

measuring size of all TAAs (superiorto echocardi-

ography for distal ascending aorta, arch, and de-

scending aorta)

 All segments of aortaand aortic branches well visu-

alized

 Use of ionizing radiation and ICM

 Cardiacmotion can cause imaging artifacts

MRI

Second-line

 Ideal technique forcomparativefollow-up studies

 Excellent modality in stablepatients

 Preferred for follow-up for youngerpatients

 Avoids ionizing radiation

 Can imageentire aorta

 Examination times longer than CT

 Beneﬁts from patient cooperation (breath hold)

 Limited in emergency situations in unstable patients

and patients with implantablemetallic devices

 Beneﬁts from gadolinium

TTE

Second-line

 Usually diagnostic foraneurysms effecting aortic

root

 Useful for family screening

 Useful for following aortic root disease

 Excellent reproducibility of measurements

 Excellent for AR, LVfunction

 Distal ascending aorta, arch, and descending aorta

not reliably imaged

TEE

Third-line

 Excellent for assessment of AR mechanisms

 Excellent images of aorticroot, ascending aorta,

arch, and descending thoracicaorta

 Less valuable forroutine screening or serial follow-

up (semi-invasive)

 Distal ascending aorta may be poorly imaged

 Does not permit full visualizationof archvessels

 Limited landmarks forserial examinations

Aortography Third-line

 Reserved for therapeuticintervention

 Useful to guide endovascularprocedures

 Invasive; riskfor 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

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diameter.

250

Therate 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 clinicalimportance of the maximum aortic diameter for deter-

mining thetiming of prophylacticsurgicalrepairimpliesmakesitcritical

that measurements be made as accurately as possible. It is essentialfor

the same observer to compare measurements side by side using the

sameanatomic references. Tomographic scans in a situation for which

theaorta doesnot lieperpendicularto theplaneof thescan producean

ellipticalimage with major (maximum)and minor (minimum) diame-

ters. Because the major diameter is typically an overestimate, in most

naturalhistory studies of aneurysm expansion, theminimumdiameter

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 theSTJ, TTE does not always adequatelyvisualizethe affected

segment, in which caseCTor MRI should beperformed. TEE maybe

warranted when the type of surgical treatment (repair or valve

replacement) is being considered. Both TTE and TEE have limitations

for adequate measurement of distalascending aorta, aortic arch, and

descending aorta diameters. In addition, if theaorta 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 techniquefor in-

terval surveillance of both thoracic and abdominal aortic aneurysms.

Measurements must adhere to a strict protocolthat permits compar-

ison between different imaging techniquesas 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-

nalplane of theaortic segment.Whentheaxialdataarereconstructed

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 inﬂammatorychanges in

theaorticwalland adjacent fat, secondarytobacterialinfection. Thein-

formationprovidedbyMRAin aortic aneurysmassessment issimilarto

thatoffered bycurrent MDCT.Both methodspermit accurate determi-

nations of aortic diameters in sagittal plane. Furthermore, postprocess-

ing techniques(MIP, multiplanereconstruction, and volumerendering)

facilitate visualization of the aorta in its entirety, together with therela-

tionship of its principal branches, and are highly usefulwhen 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.Algorithmfor Follow-Up. TTE can beused forserialimaging of

thedilated aortic root and proximalascending aorta when agreement

between the dimensions measured by TTE and CTor 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 annualsurveillanceMDCThas 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, historyof complications among familymembers, 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-

perativeTEE to conﬁrm the prior imaging ﬁndings. Theinitial 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,

oneshould determinethepresenceand severityof associatedvalvular

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 BAVrepair. One should

also assess the degree of leaﬂet thickening, calciﬁcation, and restric-

tion, becausein thesetting of signiﬁcant valve dysfunction, theseﬁnd-

ingsmayinﬂuencethesurgeon’s decision regardingtheneed forvalve

repair or replacement.

Even when theaortic valveis tricuspid with otherwisenormalleaf-

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 theaorta at that level maytether the leaﬂets, leaving

insufﬁcient slackfor thethreeleaﬂets to coapt properlyin themiddle,

resulting in a jetof centralAR.

32,194,251

AR dueto leaﬂet tethering can

occur with aneurysms of both the aorticroot and theascending 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

beinspected forthepresenceof associatedpathology, such asanunrec-

ognized aortic dissection, IMH, PAU, or protruding atheromas. Large

atheromas in the ascending aorta or arch mayprompt additionalimag-

ing of theaorta using intraoperativeepiaortic echocardiographyand in-

ﬂuencedecisions regarding thesiteof aorticcannulation and perfusion.

Postoperative TEE should begin as soon as the patient comes off

cardiopulmonary bypass. The examination should begin with inspec-

tion of theaortic valve, as unanticipated valvedysfunction mayneces-

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 ARafterrepair. If repairofbicuspidvalve

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 AmericanSociety of Echocardiography

Volume 28 Number2

Goldstein et al 155

aortic valvehad been replaced,oneshouldconﬁrmthat theprosthetic

leaﬂet ordisk motion is normal, that thereisnomorethan physiologic

AR, and that there are no paravalvular leaks.

Afteraorticvalvereplacement oraorticrootreplacement, it istypical

to see focalthickening around the aortic root. It is important to docu-

ment thisso asnottoconfusethis ﬁndingwithpathologyon 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 disorderof

connective tissue that occurs as a result of a mutation in the FBN1

gene, which encodes ﬁbrillin. Oneof thehallmark features of this dis-

orderis dilatationordissection of theproximalascending 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 isgenerallytheinitialimaging toolused fortheidentiﬁca-

tion and serial follow-up of ascending aortic enlargement in patients

with known or suspected Marfan syndrome, because of its availability,

noninvasivenature,reliability,and lackof need for radiationorcontrast

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 (Figure48). Normative values are used

to determinethepresenceand extent of aortic enlargement on theba-

sis of age and BSA.

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

Zscore.

255

Although there is dispute regarding the best echocardio-

graphic aortic measurement method, the most important concept is

that serial measurements foreach individualpatient areperformed us-

ing thesamemethod to determineaorticdimension changeovertime.

Some patients with Marfan syndrome have suboptimal transthoracic

echocardiographic images, and in these patients, serial CTor MRI is

required to monitor aortic diameter.

At the time of initial diagnosis of aortopathy in Marfan syndrome

by TTE, additional imaging with CTor MRI is generally recommen-

ded to conﬁrmthat thesize of theaorta measured by TTE is accurate

and correlateswith the computedtomographic orMRImeasurement

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 (Figure49).

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

thereis no family or personalhistoryof 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 thedimensions measured

by TTE and CTor 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 8 Transthoracic long-axis s image e demonstrating

marked dilatation of the aortic root (sinuses) in a patient with

Marfan syndrome. Note the normal dimension at the STJ.

Figure 49 9 Computedtomographic3Dvolume-renderedrecon-

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 CTor 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

semi-invasive nature and thedifﬁcultydirectlycomparing dimensions

over time.

b. Postoperative Aortic Imaging in MarfanSyndrome.–Afterelec-

tive aortic root replacement, dismissalorearly (within 6months) TTE

and CTor MRI are generallyperformed to establish a baseline aortic

assessment for patients with Marfan syndrome. Annual TTE and CT

or MRI of the aorta are generally recommended after aortic root

replacement. The frequency of aortic imaging is individualized

depending on patient characteristics, such as the type of operation

performed and the extent of aortic dilatation elsewhere. Serial post-

operative follow-up imaging should focus on progression of disease

affecting the native aorta, and common postoperative complications,

including thedevelopment of pseudoaneurysm and coronaryanasto-

motic aneurysms.

c. Postdissection Aortic Imaging in Marfan Syndrome.–Patients

with Marfan syndrome with repaired type A aortic dissection should

undergo serial aortic imaging with CTor MRI; theimaging frequency

depends on the extent of aortic dissection and the type of repair.

Patients with Marfan syndrome with type B aortic dissection that

has not been repaired require regular follow-up imaging (see section

III.B, ‘‘Aortic Dissection’’).

d. FamilyScreening.–Marfan syndromeis inherited in an autosomal-

dominant fashion, so transthoracic echocardiographic screening is rec-

ommended fortheﬁrst-degreerelatives of an affected individualunless

agenemutation has been identiﬁed and genetic testing can be used to

identifyaffected familymembers.

Allaffected familymembers should

undergo regular aortic imaging. Although there are no speciﬁc guide-

line recommendations for ‘‘regular’’ imaging, serial imaging should

depend on the age and speciﬁc features of a given individual.

2. OtherGeneticDiseases ofthe Aortain Adults. Two genetic

conditions associated with thoracic aortic disease, BAV and Marfan

syndrome, are relatively common and are discussed elsewhere in

this review. Many additional predisposing conditions for aortic aneu-

rysm formation and dissection arelisted inTable18. Thescope of this

document doesnot permit a detailed discussionof theselesscommon

entities, but a few pertinent details are mentioned. Importantly,

awareness of these disorders and their potential risk is critical not

only to presenting patients but also to their close relatives.

a. Turner Syndrome.–Women with Turner syndrome are at risk for

BAV, aortic coarctation, andaorticdilatation ordissection. Aortic dila-

tation in patients with Turner syndromehas beenreported to occurin

up to40%of cases. Imaging of theaorta inthesepatientsmust include

theascending aorta, aortic arch, and proximal descending aorta. Asin

other cases, aortic dilatation coexists with a BAV and/or coarcta-

tion.

256-258

Patients with Turner syndrome have a small stature

compared with same-age individuals in the general population, so

allaorticmeasurements should beindexed to BSA. An indexed aortic

diameter of >2 cm/m

in the ascending aorta should be followed

annually, as the risk for aortic dissection is increased.

258

b. Loeys-Dietz Syndrome.–Loeys-Dietz syndrome results from a

gene mutation of transforming growth factor b receptor 1 or 2 and

is inherited in an autosomal-dominant fashion.

259

Aortic root aneu-

rysms are present in the majority of patients with Loeys-Dietz syn-

drome. Involvement of other aortic segments and smaller arteries in

the form of aneurysms or marked tortuosity are characteristic in

this population.

259,260

Dissection can occur at dimensions smaller

than in other inherited disorders of connective tissue. Although an

annual comprehensive arterial imaging protocol with MDCT or

MRI has been recommended,

261

the strategy for follow-up is not

well established and should beindividualized from annually to every

2to 3 years depending on abnormalities present, family risk of com-

plications, and degree of evolution. Interpretation should include in-

formation about the caliber of the aortic root, the ascending and

descending aorta, and thepulmonaryartery(pulmonaryarterydilata-

tion may also occur). If progression of aortic disease has occurred, it

should be monitored every 6 months (every 12 months for other ar-

teries) given the markedly increased risk for dissection or rupture.

c. Familial TAAs.–Different gene mutations have been identiﬁed in

familial TAAs, predominantly with an autosomal-dominant inheri-

tance. Aneurysms in relatives may be seen in the thoracic aorta, the

abdominal aorta, or the cerebral circulation. Therefore, comprehen-

sive imaging for screening of ﬁrst-order relatives of probands with

TAA is advisable.

262

Thefrequencyand modalityof vascular imaging

inaffected persons issimilar to that outlined forMarfan syndromebut

should be individualized.

263

d. Ehlers-Danlos Syndrome.–The vascular form of Ehlers-Danlos

syndrome (type IV) is a rare autosomal-dominant disorder with

vascular involvement characterized byarterial dilatation and rupture.

The role of aortic imaging in this population is less clear. Elective sur-

gical repair of aortic aneurysms or other vascular involvement carries

ahigh risk because of due to tissue fragility, so the impact of serial

aortic imaging is unclear.

264

3. BAV-Related Aortopathy. a. Bicuspid Valve–Related Aort-

opathy.–BAVs affect 1% to 2% of thepopulation and are often associ-

ated with aortopathy.

265-269

Nearly 50% of patients with BAVs have

dilatation of either the aortic root or ascending aorta.

247,268,270

Dilatation of the aortic arch and descending thoracic aorta can also

occur but is less common. Recently, it has been reported that patients

with BAVs also are at increased risk for intracranial aneurysms

compared with the normal population,

271

although the clinical signiﬁ-

canceof this is unknown. Progressive dilatation of theaorta may occur

irrespective of the functional status of the BAV and places patients at

increased risk for aortic dissection or rupture.

272,273

Patients with

BAVs may also have coexisting coronary artery anomalies, including

reversal of dominance, short left main coronary artery (<10 mm), and

anomalous origin of the left circumﬂex artery from the right coronary

Table 18 8 Geneticconditionsassociatedwithaorticdisease

BAV

Marfan syndrome

Loeys-Dietz syndrome

Turner syndrome

Ehlers-Danlos vascular type (type IV)

Familial TAA

Shprintzen-Goldberg (craniosynostosis) syndrome

Journal of the AmericanSociety of Echocardiography

Volume 28 Number2

Goldstein et al 157

cusp.

250,270,274-278

Failure to recognize these anomalies may result in

riskfor coronary arteryinjury during aortic valve repair or replacement.

b. ImagingoftheAortainPatientswithUnoperatedBAVs.–TTE is

the primary imaging tool for the initial diagnosis and screening as

well as serial follow-up of patients with known or suspected BAVs

with or without aortopathy. The aortic root or ascending aorta

may be dilated. The pattern of dilatation may be associated with

BAV morphology.

269

At the time of initial diagnosis of aortopathy

in patients with BAVs, imaging with CTor MRI is generally recom-

mended to conﬁrmthat thesize of the aorta measured byTTE is ac-

curate. Eccentric dilatation of the aortic sinus adjacent to the

conjoined cusp increases the chance of underestimation of the

aortic root measurement by TTE, particularly when the measure-

ment is obtained only in the long-axis format. CTand MRI also pro-

vide important information about the size of the aortic arch and

descending aorticsegments,which areoftenincompletely visualized

by TTE (Figure49). Although BAVoccurs in >50% of patients with

coarctation, coarctation is noted in <10% of patients with BAVs.

Nevertheless, whenever a BAV is detected, coarctation should al-

ways be sought.

c. Follow-Up Imaging of the Aorta in Patients with Unoperated

BAVs.–All patients with BAVs and associated aortopathy should un-

dergo annualsurveillanceimaging of theascending aorta to monitor

growth over time. TTE can be used to monitor the aortic root and

ascending aorta when correlation between the dimensions

measured by TTE and CT or MRI has been conﬁrmed. After the

identiﬁcation of ascending aortic enlargement in a patient with

BAV, repeat imaging after 6months is recommended. If theaorta re-

mains stable at 6-month follow-up and is <45 mm in size, and there

is no familyor personal historyof aortic dissection, annualaortic im-

aging is recommended. Patients who do not meet these criteria

should undergo repeat aortic imaging using TTE every 6 months.

Occasionally, patients with BAV-related aortopathy have demon-

strated stable dilatation of the ascending aorta over several years;

thefrequencyof aorticfollow-up in thesepatients should beindivid-

ualized. Patients with BAVs and no demonstrableaortopathyshould

be screened every 3 to 5 years with TTE for the development of

aortic enlargement.

Repeat CTorMRIis suggested at least every3to 5years to reassess

theaorticarch and descending aorta and reconﬁrmthat transthoracic

echocardiographic measurements of the aortic root remain reliable

for serial measurements.

TEE isgenerallynot used forinitialdiagnosis andfollow-up of BAV-

related aortopathy, because of its semi-invasive nature and difﬁculty

comparing dimensions over time.

Patients with BAVs with aneurysmal dilatation of theaortic arch or

descending thoracic aorta or those with remote histories of type B

aortic dissection requireregular computed tomographic angiography

or MRI to monitor aortic stability. In such cases, imaging should be

repeated annually. TTE does not provide reliable imaging for serial

follow-up of the dimensions of these portions of the aorta.

d. Postoperative Aortic Imaging in Patients with BAV-Related

Aortopathy.–After elective aortic root replacement, early (dismissal

or within 6 months) TTE and CTor MRI are generally performed to

establish a baseline aortic and valve assessment. During the imaging

study, it is critical to know what surgical procedure was performed to

identify potentialresidua or sequelae (Figure50). Annualaortic imag-

ing isgenerallyrecommended after aorticroot replacement orreplace-

ment of theaorta abovethecoronaryarteries; however, thefrequency

isindividualized dependingon patient characteristics, typeofoperation

performed, and duration of follow-up. Dilatation of the remaining

ascending aorta, aortic arch,ordescending thoracic aortamay continue

after the ascending aorta has been replaced. Serial postoperative

follow-upimaging shouldalso focusoncommonpostoperativecompli-

cations, including the development of coronary button pseudoaneur-

ysm formation, anastomotic site pseudoaneurysm formation, and

progressive dilatation of other aortic segments.

Figure50 (A)Computedtomographic3Dvolume-renderedreconstructionofthethoracicaortainapatientwithprioraorticvalveand

ascending aorta replacement due to bicuspid valve–related aortopathy; the image demonstrates aortic arch dilatation(asterisk). (B)

Computed tomographic 3D volume-rendered reconstruction of the thoracic aorta in a patient with BAV and aortopathy with prior

aortic valve and supracoronary aortic replacement; the image demonstrates features of asymmetric aortic root dilatation (asterisk).

158 Goldstein et al

Journal of the American Society of Echocardiography

February 2015

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