Jead 024

European Heart Journal - Cardiovascular Imaging (2023) 24, e65–e85 EACVI DOCUMENT
https://doi.org/10.1093/ehjci/jead024
Multimodality imaging in thoracic aortic diseases:

a clinical consensus statement from the European
Association of Cardiovascular Imaging
and the European Society of Cardiology working
Downloaded from https://academic.oup.com/ehjcimaging/article/24/5/e65/7070979 by guest on 22 May 2023

group on aorta and peripheral vascular diseases
Artur Evangelista1*, Marta Sitges 2,3, Guillaume Jondeau 4, Robin Nijveldt 5,
Mauro Pepi 6, Hug Cuellar7,8, Gianluca Pontone 9, Eduardo Bossone 10,
Maarten Groenink11, Marc R. Dweck12, Jolien W. Roos-Hesselink13,14, L. Mazzolai15,
Roland van Kimmenade16, Victor Aboyans 17, and Jose Rodríguez-Palomares1
1
Servei de Cardiologia, Hospital Vall d’Hebron, CIBERCV, Universitat Autonoma de Barcelona, Vall d´Hebron Research Institute, VHIR, Barcelona, Spain; 2Cardiovascular Institute, Hospital
Clinic, University of Barcelona, Villarroel 170, 08036 Barcelona, Spain; 3CIBERCV, Institut Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Villarroel 170, 08036 Barcelona, Spain;
4
Reference Center for Marfan Syndrome and related diseases, Cardiology Department, VASCERN HTAD European Reference Centre, Hopital Bichat, APHP, INSERM U1148, Université de
Paris, France; 5Department of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands; 6Centro Cardiologico Monzino IRCCS, Via Carlo Parea 4, Milano, Italy;
7
Radiology Department, Institut de Diagnòstic per la Imatge, Hospital Vall d’Hebron, Barcelona, Spain; 8Universitat Autònoma de Barcelona, Vall d´Hebron Research Institute (VHIR), Barcelona,
Spain; 9Department of Perioperative Cardiology and Cardiovascular Imaging, Centro Cardiologico Monzino IRCCS, Milan, Italy; 10Cardiology Division, Antonio Cardarelli Hospital, Via Antonio
Cardarelli 9, 80131, Naples, Italy; 11Department of Cardiology and Radiology, Amsterdam University Medical Center, Amsterdam, The Netherlands; 12BHF Centre for Cardiovascular Science,
University of Edinburgh, Chancellors Building, Little France Crescent, Edinburgh EH16 4SB, UK; 13Cardiology Department, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The
Netherlands; 14VASCERN HTAD European Reference Centre; 15Division of Angiology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; 16Department of Cardiology, Radboud
University Medical Center, Nijmegen, The Netherlands; and 17Department of Cardiology, Dupuytren University Hospital, Inserm U1094, Limoges, France
Received 29 January 2023; accepted 30 January 2023; online publish-ahead-of-print 7 March 2023
Imaging techniques play a pivotal role in the diagnosis, follow-up, and management of aortic diseases. Multimodality imaging provides complementary
and essential information for this evaluation. Echocardiography, computed tomography, cardiovascular magnetic resonance, and nuclear imaging
each have strengths and limitations in the assessment of the aorta. This consensus document aims to review the contribution, methodology, and
indications of each technique for an adequate management of patients with thoracic aortic diseases. The abdominal aorta will be addressed else
where. While this document is exclusively focused on imaging, it is of most importance to highlight that regular imaging follow-up in patients
with a diseased aorta is also an opportunity to check the patient’s cardiovascular risk factors and particularly blood pressure control.
...................................................................................................................................................
Keywords imaging • aorta • aortic syndrome • aortic aneurysm
best imaged from the left parasternal long-axis view. To visualize the
Imaging modalities: methodology, mid-distal ascending aorta, it may be necessary to move the transducer
advantages, and limitations to upper intercostal spaces, while right parasternal views might help
visualizing the distal portion of the ascending aorta. The proximal as
Transthoracic echocardiography cending aorta may also be visualized in the apical long-axis (apical three-
Transthoracic echocardiography (TTE) is used to measure the prox chamber) and apical five-chamber views and even in modified subcostal
imal aortic segments in routine clinical practice. However, by using all views (especially in children). The aortic arch and the proximal descend
echocardiographic views, it is possible to visualize most aortic seg ing aorta can be assessed from the suprasternal window. Moreover, the
ments, if image quality is good (Figure 1; see Supplementary data distal portion of the thoracic aorta can be observed in the parasternal
online, Video S1). The aortic root and proximal ascending aorta are long-axis view and in a modified apical two-chamber view while the
* Corresponding author. Tel: +34 932175153. E-mail: [email protected]

© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: [email protected]
e66 A. Evangelista et al.

Figure 1 Transthoracic echocardiography of the aorta: the essential views. (A) standard parasternal long-axis view showing the Ao root and proximal
ascending Ao; (B) adapted long-axis view of the entire ascending Ao from an upper parasternal view; (C ) short-axis view of the aortic root and the aortic
valve; (D) suprasternal view of the arch; (E and F) thoracoabdominal views of the Ao from the subxifoid and adapted 2-chamber view, respectively. Ao,
aorta; BCT, barchiocephalic trunk; Desc Ao, descending Ao; LA, left atrium; LCCA, left common carotid artery; LSA, left subclavian artery; LV, left
ventricle; MV, mitral valve; PA, pulmonary artery; RA, right atrium; RV, right ventricle; TAAo, thoracoabdominal Ao.
abdominal aorta is seen from subcostal and abdominal approaches. thoracoabdominal aorta are its main strengths. It is of utmost import
Finally, in cases with pleural effusion, the descending aorta may be im ance to use an adequate protocol acquisition tailored to the clinical set
aged from the patient’s back.1–3 Three-dimensional (3D) probes allow ting. CT protocols of the aorta typically include a non-contrast phase,
for multiplanar views, potentially yielding more accurate measurements an arterial phase as well as a late scan. The non-contrast phase allows
due to improved alignment of cutting planes.4 for the assessment of aorta calcifications, intramural haematoma
(IMH), and surgical material, the arterial phase describes the lumen,
Transoesophageal echocardiography and the late phase may depict contrast leakage in the context of aortic
dissection (AD) or prior endovascular repair.
The proximity of the oesophagus allows for high-resolution images of
ECG triggering is recommended to avoid motion artefacts of the
the thoracic aorta. The best views of the ascending aorta, aortic root,
aortic root and ascending aorta, which may impair size measurements
and valve are the long-axis (at 120–150°) and short-axis (at 30–60°)
or mimic AD, and allows also for the assessment of the coronary arter
views (Figure 2). With the probe posteriorly oriented, short-axis or
ies. Pre-treatment with beta-blockers or ivabradine might be required
long-axis images of the whole descending thoracic aorta, from the
to ensure adequate heart rates (below 80 bpm for CT angiographic
upper segment to the coeliac trunk and the superior mesenteric artery,
scanning of the aorta), although this is less of a concern for the aorta
can be obtained. Although the take-off of the left subclavian artery is
compared with the coronary arteries when heart rate below 60 bpm
easy to visualize, the left common carotid and brachiocephalic arteries
is recommended. Two main methods of ECG synchronization are cur
can be extremely difficult to image, usually requiring careful clockwise
rently available: (i) ECG-gated spiral acquisition with data acquired
rotation of the probe. The distal portion of the ascending aorta and
throughout the entire cardiac cycle and retrospectively reconstructed,
the proximal part of aortic arch are usually not visible (so-called ‘blind
mainly recommended in emergency cases; (ii) ECG-triggered axial (se
spot’), because of the interposition of the trachea. The deep transgas
quential) acquisition, with lower radiation dose but more sensitive to
tric view can depict the aortic valve (AV) and the ascending aorta.5
heart rate irregularities.6 Newer technologies such as dual-source
and wide-detector CT systems overcome these limitations and can ac
Computed tomography quire the thoracic aorta in one or two heart beats, respectively.
Computed tomography (CT) angiography, particularly with electrocar
diogram (ECG) gating, is an essential tool to diagnose, to risk stratify,
and to plan therapy in patients with thoracic aortic disease. Short acqui Cardiovascular magnetic resonance
sition times, widespread availability, high reproducibility, and the ability Cardiovascular magnetic resonance (CMR) is a reliable and reprodu
to provide simultaneous luminal and mural information of the whole cible imaging modality for aortic diseases, due to its capacity for
Multimodality imaging in thoracic aortic diseases e67
multiplanar and 3D imaging without the use of iodine-containing con assessment of the entire aorta. In addition, CMR offers mor
trast agents or ionizing radiation. It is the ideal technique for compara phologic, functional, and tissue characterization information
tive follow-up studies, especially in younger patients. without radiation exposure. PET is used to diagnose inflamma
Technical aspects are also key when performing CMR to evaluate the tory or infectious disease of the aorta.
aorta and should include ECG gating and acquisition at mid- or end-
diastole to minimize motion artefacts. It is also of utmost importance
to perform 3D imaging. CMR angiography (MRA) of the aorta with How to measure the aorta
contrast-enhanced sequences often shows motion artefacts in the aor
Accurate measurements of the maximal diameter of the aorta are key
tic root and is therefore less clinically useful at this location; however,
to establish a diagnosis of aorta dilation, to assess disease progression,
this approach yields highly reliable measurements of the aorta lumen
and most importantly to guide the need for prophylactic intervention
beyond the aortic root.7 Post-processed 3D volume-rendered images
according to current guidelines.12 Given the known variability in mea
can clarify complex congenital anatomy of the aorta and great vessels.
surements of aorta diameter, mainly related to the different method
3D non-contrast-enhanced MRA sequences can substitute
ologies and imaging modalities used, it is of utmost importance to
contrast-enhanced MRA for morphologic follow-up of the thoracic
follow standardized measurement techniques. One of the main goals

aorta. Nevertheless, contrast-enhanced MRA is recommended for
of the present expert consensus is to provide such standardization.
the whole thoracoabdominal aorta.
Some general rules can be proposed as the basis for measurements
Additionally, 2D turbo spin echo sequences (so-called black blood
of the aorta diameter.
images) may be useful for assessing aorta wall abnormalities, such as
IMH (hyperintense on T1-weighted image and isointense on (a) When using echocardiography, measurements of the aorta diameter
T2-weighted image in the subacute phase), aortitis (hyperintense on should be made with the leading-to-leading edge method (Figure 4).
T2-weighted images), and atherosclerotic plaques and thrombi (isoin Because of the axial resolution of ultrasound, the thickness of the aor
tense on both T1-weighted and T2-weighted images). Cine steady-state tic wall is falsely increased by ∼1–2 additional mm13,14 (the real thick
free precession (SSFP) imaging is recommended for assessing AV
ness of the aortic wall as measured by CT is typically 1 mm).
morphology and function. CMR also allows for the evaluation of aorta
Consequently, measurement of the aortic diameter with echocardi
biomechanical parameters such as stiffness, distensibility, and strain,
ography (using the inner-to-inner convention) systematically underes
used to assess aorta elasticity in patients with Marfan syndrome, bicus
pid AV (BAV), or aorta aneurysms. Aorta stiffness by pulse wave vel timates the aorta diameter measured by CT. This technical artefact
ocity (PWV) defined by phase-contrast sequences has been happens in all segments of the aorta, not only the ascending aorta.
associated with the severity of atherosclerotic disease and the occur All aorta diameter measurements should be made in diastole.
rence of cardiovascular events. In patients with BAV, aortic stiffness Systole is associated with expansion of the aorta of ∼2 mm.15
is similar to that described in the normal population whereas Marfan Several authors have demonstrated optimal agreement using leading
syndrome patients have a stiffer aorta.8 2D phase-contrast velocity edge echo measurement and inner-to-inner CT/CMR measurement
mapping is part of the standard examination and allows flow assess in end-diastole.14,16,17 Additionally, most of the echocardiographic
ment and quantification of associated AV stenosis and regurgitation, studies that demonstrated the benefits of prophylactic surgery were
evaluation of flow patterns in the true and false lumen (FL) of AD, performed using this convention.
and estimation of pressure gradients across a coarctation and its collat (b) When the aorta wall is thickened due to the presence of atheroma,
eral flow.9 IMH, or aortitis, outer-to-outer diameter (including the aorta walls)
In recent years, 3D-cine (time-resolved) phase-contrast CMR with should be also reported.
three-directional velocity encoding (4D-flow) has been developed to (c) In the presence of intraluminal thrombus, the inner-to-inner measure
study intravascular flow10 (Figure 3; see Supplementary data online, ment should exclude the thrombus (Figure 5). If the thrombus is cir
Video S2). It quantifies flow similarly to 2D-cine phase-contrast CMR, cumferential, the outer-to-outer convention must be used instead.
has good scan repeatability, and allows for the analysis of advanced (d) In AD, the inner-to-inner diameter should include both the true and
parameters such as wall shear stress (WSS) or turbulent kinetic energy. FL. If the FL is partially or totally thrombosed, indication in (c). should
In patients with BAV, these sequences have added an understanding of be applied.
the complex flow patterns distal to the AV, shedding light into a poten
tial mechanism underlying associated aortic dilation. Using 4D-flow or Beyond these general recommendations, each segment of the aorta
image-based computational modelling, elevated WSS has been asso has its own individual considerations that are addressed specifically
ciated with aortic wall degradation in ascending thoracic aortic below.
aneurysms. The thoracic aorta consists of four parts, which can be further
subdivided:
Positron emission tomography • The aortic root consists of the annulus, the valvular cusps, and the si
Molecular imaging with positron emission tomography (PET) allows for nuses of Valsalva.
the non-invasive assessments of metabolic activity in the cardiovascular • The tubular segment of the ascending aorta corresponds to the segment
system. While novel tracers targeting calcification, fibrosis, and thrombus from the sinotubular junction to the origin of the brachiocephalic artery.
formation are emerging, most PET studies have focused on inflammatory • The aortic arch is defined from the brachiocephalic artery up to the aor
activity in the aorta of patients with large vessel vasculitis. These provide tic isthmus.
important diagnostic information and the potential ability to track • The descending aorta is from the isthmus to the diaphragm.
changes in disease activity over time and with therapy. Limitations to
the technique include radiation exposure and the relatively high costs The aortic root
and limited availability of cardiovascular PET scanners.11 The most difficult segment to measure is the aortic root, given that it
has a non-circular shape. This applies also to the AV annulus, particular
Key point 1. TTE permits adequate assessment of several aortic ly important for transcatheter AV implantation, but this is beyond the
segments, particularly the aortic root and proximal ascending topic of this expert consensus. Diameters measured using CT/CMR
aorta. However, CT provides rapid, accurate, and reproducible from sinus-to-sinus are generally 2 mm larger on average than those

Figure 2 Transoesopahgeal echocardiography of the aorta: the essential views. (A) short-axis view (30–40ª) of the aortic valve at the level of the great
vessels; (B) long-axis view of the ascending aorta (130–140°) showing the aortic sinuses, the sinotubular junction and the tubular ascending aorta; (C )
longitudinal view of the aortic arch; (D) short-axis view of the descending thoracic aorta (0°); and (E) long-axis view of the descending thoracic aorta
(90°). AA, ascending aorta; AV, aortic valve; D Ao, descending thoracic aorta; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
Figure 3 Systolic 3D streamline representation of 4D-flow CMR data. Two different patients with a bicuspid aortic valve: (A) right-left cusp fusion
and (B) right non-cusp fusion. Notice the difference in the flow direction: in right-left cusp fusion, flow impinges on the outer curvature of the proximal
ascending aorta (arrows), including the root (large arrow). In right non-cusp fusion, flow is posteriorly directed in the proximal aorta (large arrow) and
impinges on the outer wall in the distal ascending aorta (arrows). See also Supplementary data online, Video S2.
measured from sinus-to-commissure (Figure 4). The sinus-to-sinus double obliquity measuring the diameter from sinus to sinus at end-
method has several advantages, including the ease of detecting cusp diastole, using the inner-edge-to-inner-edge convention.
margins in co-axial CT/CMR views, the close agreement with 2D echo By echocardiography, the diameter of the sinus of Valsalva is typically
cardiographic measurements, and its greater feasibility in patients with measured from long-axis parasternal views with specific focus on ob
BAV.14,16,18 Therefore, the consensus is that the maximum aortic root taining a view of the central line of the aorta. Studies comparing the
diameter by CT/CMR must be determined in a transversal plane with maximum aorta root diameter obtained by CT and echocardiography

Figure 4 How to measure the aorta. (A) Parasternal long-axis view by transthoracic echocardiography illustrating measurement of aortic root (1),
sinotubular junction (2) and proximal ascending aorta (3) diameters at end-diastole using the leading edge-to-leading edge method; (B) 3D echocar
diography from the parasternal long-axis view using orthogonal views (biplane or x-views) allows for a better alignment and measurement of the aortic
root diameters (yellow arrows); (C ) aortic root cusp-to-cusp diameters measured by cardiac CT at end-diastole using the inner-to-inner convention
(red lines), in yellow the representation of the direction of ultrasound beam that would be projected by parasternal long-axis view echocardiography.
have concluded that echocardiographic measurements from the left confirmatory examination of the echocardiographic measurements.
parasternal long-axis view using leading-to-leading edge are concordant In case of asymmetric dilation of the aortic root, the recommendation
to sinus-to-sinus inner-to-inner edge measurements taken with CT/ is also to report the three sinus-to-sinus diameters measured for each
CMR.14,16,17 patient particularly during follow-up.
Short axis parasternal echocardiographic views are inappropriate as
perpendicular planes cannot be ensured and the lower lateral reso Tubular segment of ascending aorta
lution of ultrasound limits accurate measurements of the aortic walls
Maximum diameter measurements in the tubular portion of the as
and edges. However, 3D echocardiography may partially overcome
cending aorta and indeed of the rest of the aorta are easier due to its
this limitation as multiplane views [multiplanar reconstruction (MPR)
more typical cylindrical shape. By echocardiography, the tubular as
or biplane views] allow for accurate perpendicular views and true
cending aorta is usually measured from long-axis parasternal views at
short-axis, transversal views of the aortic root.4 Despite initial promise,
end-diastole. As mentioned above, to evaluate optimally the
more validation studies are still needed on the potential role of 3D
medium-upper part of the ascending aorta and to scan it on its central
echocardiography particularly multiplane echocardiography, in asses
axis, it is recommended to move the transducer up one or two inter
sing this asymmetry and improving the accuracy of the measurement
costal spaces. Using CT/CMR, maximum aortic diameter should be
of the largest diameter of the aortic root (Figure 4).
measured from inner-to-inner edges at diastole on double oblique
Of note, it is also important to address the asymmetry of the aortic
images reconstructed at the most dilated level of the aortic segment.
root that might yield different diameters between aortic sinuses. This is
The presence of significant atherosclerosis, IMH, or aortitis may limit
the case for patients with BAV and for those with localized dilation of
accuracy. In these cases, the outer-to-outer diameter should also be re
the non-coronary sinus, the most frequently dilated sinus. Significantly
ported (Figure 5). The most dilated level of the ascending aorta is fre
asymmetric root dilatation, defined with more than 5 mm difference in
quently at the pulmonary trunk, but that is patient and pathology
oblique diameters, can be present in up to >40% of patients with BAV
dependent. Both maximum anteroposterior diameter and a perpen
(particularly in those with fusion of the right coronary and non-
dicular diameter should be measured, but if the aortic shape is circular,
coronary cusps and those without raphe).19,20 In a BAV with two si
they should be identical, and a single value can then be reported
nuses, two orthogonal diameters, the longitudinal and transverse dia
meters, should be measured (Figure 6). Therefore, considering that
significant asymmetry of the aortic root is very prevalent, when aortic Aortic arch and descending thoracic aorta
root dilation is initially diagnosed by echocardiography, a multiplanar In patients with good acoustic window, echocardiography may be use
CT/CMR scan is recommended at least as a baseline reference and ful to screen for aortic arch dilation but has low accuracy and

Figure 5 How to measure the aorta in CT. (A) Sagittal maximum intensity projection of an ECG-gated CT angiography shows an elongated thoracic
aorta in an elderly patient. Measurements in the patient’s axial plane would result in overestimation of the maximum distance. Coloured lines show
alignment of the cutting plane of multiplanar reconstructions for each aortic segment depicted in Panels B–E. (B–E) Double oblique multiplanar recon
structions of the ascending aorta (B), arch (C ), proximal descending (D), and distal/retrocrural aorta (E) with measurements of maximum inner-to-inner
distance and its orthogonal distance. In non-pathologic segments and fusiform dilatation, both distances should be similar and can be averaged. (F)
Double oblique multiplanar reconstruction of a fusiform aneurysm of the descending thoracic aorta, with significant thickening of the aortic wall
due to the combination of an atherosclerotic plaque (black arrows) and a large intraluminal thrombus (white asterisk) at its posterolateral aspect.
The maximum outer-to-outer distance (solid white arrow) is reported, as well as the maximum wall thickness. Periaortic fat and lung collapse (white
stars) should not be included in the measurement. Alternative diameters (dotted white arrows) are marginally shorter. (G) Double oblique multiplanar
reconstruction of the dissected descending thoracic aorta at its most dilated segment. The maximum inner-to-inner distance bisects the centre of the
flap (solid white arrow) and includes the intraluminal thrombus (white asterisk). The longest orthogonal distance (dotted white arrow) can also be
reported. Lung collapse and pleural effusion are excluded (white stars).
reproducibility when taking diameter measurements at this level and aortic diameter by CT/CMR is always mandatory before taking
particularly in the descending aorta. Transoesophageal echocardiog any decision for intervention.
raphy (TOE) may be better for this purpose than TTE; however, it
may be hard to get in to a co-axial transverse plane, and regular follow-
up using this method is uncomfortable. CT and CMR are therefore the
preferred and recommended imaging modalities. Using these imaging
Normal aorta diameter values
modalities, the aortic diameter should be performed using the Reported reference maximum normal aortic diameters values at differ
inner-to-inner edge method. Whenever wall thickening, thrombus or ent segments of the aorta are shown in Figure 7. Factors influencing the
dissection flaps are present, the outer-to-outer edge diameter should aorta size in the normal population include age, gender, ethnicity body
be additionally reported as previously mentioned. In these aorta seg surface area (BSA) and particularly, height.21
ments, it is particularly important to avoid off-axis imaging that can In the specific case of genetic aortopathies where dilation may be a major
overestimate the aortic diameter in patients with tortuous aorta diagnostic criterion, Z-scores should be used to determine if aorta dilation is
courses. Therefore, adequate MPRs with CT/CMR should be present. The Z-score is the number of SDs above or below the predicted
warranted. mean normal diameter. Therefore, an aortic diameter can be considered
normal when the Z-score is ≤2. In infants, aorta dilation must be differen
Key point 2. Maximum aortic diameter should be measured at tiated from aorta growth, proportional to BSA, and within normal paediatric
end-diastole using the leading-to-leading edge convention on reference values. Blood pressure and genetic factors are also determinants,
echocardiography and the inner-to-inner edge convention on even in the normal population. In this regard, the rate of growth in the size of
CT/CMR using transverse planes with double obliquity. the aorta in adults is about 0.9 mm in males and 0.7 mm in females per dec
ade, because of loss of the elastic properties of the media. When body mass
Key point 3. Because the true central axis of the aorta can be index is on the low or the high range, we recommend using nomograms to
sometimes difficult to find when using TTE, assessment of the report the aortic diameter. Supplementary data online, Table S1 summarizes

Figure 6 How to measure the aortic root in bicuspid aortic valve (BAV). SSFP cine CMR sequence at the level of the aortic valve in two different
patients with a BAV: (A) patient with a fused type BAV (right-left type fusion). (B) Two-sinus type (latero-lateral phenotype). The red arrows show the
different conventions used to measure the sinuses of Valsalva: (A) cusp-to-cusp convention, (B) maximum longitudinal diameter and a perpendicular
one.
Figure 7 Maximum diameter reference values for different segments of the aorta according to gender but without normalization by age and body
size.15,21–23 See other references in Supplementary data online.
the most widely used and recommended nomograms that were developed >34 mm in female adults or an indexed diameter/BSA > 22 mm/m2 usu
for echocardiography but are also used for CT and CMR.22,24 ally indicates aorta dilation. The term aneurysm was classically defined as
an aorta diameter >50% the normal value. Since in many cases of ascend
Key point 4. Factors influencing the aorta size in the normal ing aorta dilation, the surgical indication is established before achieving
population include age, gender, ethnicity, BSA, and particularly, this diameter in agreement with the current guidelines,12 we strongly rec
height. In routine clinical practice, a diameter of the aorta ommend the use of significant aorta dilation specifying the diameter value
>40 mm in male and >34 mm in female adults or an indexed while using the term aneurysm when the diameter of the ascending aorta
diameter/BSA > 22 mm/m2 usually indicate aorta dilation. is >45 mm. This cut-off value is arbitrary and based on the fact that it im
plies a significant and clinically relevant aortic dilation. In addition, aorta
dilation may adopt specific geometries altering the shape of the aorta
Aortic dilation that can be classified as fusiform or saccular. These abnormal shapes
are frequently better defined with 3D imaging modalities.
Definition
Although aorta dilation is defined by an aorta diameter >2 SD of the Risk of complications
mean normalized by age, gender, and body size (>2 Z-score), in routine The relationship between the aorta diameter and the risk of dissection/
clinical practice, a diameter of the aorta >40 mm in male adults and rupture is well-established with lower thresholds in genetic diseases
(including some BAV)25 than acquired aortic diseases, as outlined in is partially explained by different conventions used for measurement
more detail in the current guidelines.12 This evidence underscores (how we measure the aorta) and the use of different imaging modalities
the importance of accurate measurements of the maximum aorta and operators; for example, an increase in diameter after changing the
diameter with imaging. The risk of dissection or rupture is also related echocardiographer is more to reflect differences in measurement tech
to the growth rate or progression of the aorta dilation, which warrants nique rather than genuine disease progression.
periodic surveillance with imaging after the initial diagnosis of aorta dila The variability of aorta diameter measurements is typically consid
tion.12 This highlights the importance of the reproducibility of aorta ered to be ≤2mm.14,30 Therefore, a real change in ascending aorta
diameter measurements. Aorta tortuosity might be another parameter diameter, not related to measurement variability, can only be consid
to consider in clinical decision making, in particular in patients with bor ered when greater than 2 mm. In any case, where the growth rate
derline indications for surgery and genetic aortopathies.26 Finally, im may impact clinical decisions, it is important to confirm any enlargement
aging of the aortic branches, looking for dilation/dissections in by direct side by side comparison of measurements performed on the
mid-sized arteries, is particularly recommended in some genetic aorto individual sequential scans (ideally acquired by the same operator in the
pathies [related to variants in the transforming growth factor beta path same centre). The use of new image registration-based semi-automatic
way and possibly FBN1], as these abnormalities appear associated with assessment provides robust 3D mapping of aortic diameters and
poor outcomes.27 Other imaging parameters proposed as predictors

growth rates that go beyond the current established manual analysis
of rapid aorta enlargement or events include effacement of the sinotub and may reduce variability.31
ular junction, aorta wall stiffness, molecular assessments of disease ac Of note, any increase >3 mm by TTE should be validated by CT/
tivity, and blood flow pattern. However, data supporting their CMR and compared with baseline data When follow-up is based on
predictive value are much more scarce and still controversial. CT, such as the case of aortic arch and descending thoracic aorta in
volvement, a second modality is not necessary to confirm dilation pro
gression. Moreover, when surgery is indicated, CT should be always
Follow-up: how and when performed to confirm the maximum aorta diameter as well as evaluat
The ideal imaging modality used for the follow-up of adults with aorta ing the coronary ostia when the aortic root is involved. At the time of
dilation should be as reproducible, accurate and widely available as pos surgery, TOE can also provide useful information on AV anatomy and
sible because repeated exams are required over time. To reduce vari the feasibility of AV sparing or repair surgery if aortic valvular regurgi
ability, the same imaging modality should always be used over time. In tation is present.
this sense, TTE stands as the preferred imaging modality to follow-up
patients with dilation of the aortic root or ascending aorta, where these Post-operative aortic imaging
segments are well visualized on echocardiographic windows. In patients
After any intervention on the thoracic aorta either with open or endo
with a dilated aorta, baseline aorta diameters at the time of diagnosis
vascular surgery, early TTE and CT or CMR are generally performed to
should be validated with CT or CMR. Once the agreement between
establish a baseline reference and discard potential postoperative com
the aorta dimensions measured by TTE and CT/CMR has been con
plications. Thereafter, annual aortic imaging is generally recommended;
firmed, TTE can be used for serial imaging of the dilated aortic root
however, the frequency is individualized depending on patient charac
and the proximal ascending aorta. Conversely, if aorta diameters ob
teristics and the type of operation performed.
tained from TTE and CT/CMR differ by more than 3 mm from echocar
diographic diameters, follow-up should be based on CT/CMR. When
dilation involves the distal (upper) portion of the ascending aorta, the Screening
arch, or the descending thoracic aorta, CT and CMR are the recom About 20% of patients with thoracic aortic aneurysm or dissection have
mended imaging modalities for follow-up (Figure 8). a first-degree relative with a similar disease. In familial forms, defined as
Follow-up intervals between imaging exams will be slightly different more than one family member having an aortic aneurysm or dissection,
depending on the predicted growth rate of the dilated aorta. aortic imaging is recommended particularly in first-degree relatives if
Generally, when baseline aorta diameter is >45 mm, a second exam none of the known disease-causing genes are identified.32 If initial
is recommended 6 months after the initial diagnosis to confirm stabil screening is normal, it should be repeated every 5 years until the age
ity/progression of the aorta dilation. Thereafter, serial exams can pro of 65. TTE is the primary imaging tool for screening of family members
ceed on a yearly basis.28 If a real increase in aortic diameter or in the although CT or CMR is recommended at initial evaluation, to exclude
annual growth rate is confirmed, the time intervals for follow-up should the presence of aneurysms at areas poorly visualized by TTE. In spor
be reduced. Conversely, stability in aortic diameters measured over a adic cases with a high risk of genetic predisposition (<50-year-old, no
period of years might eventually lengthen these follow-up intervals. In hypertension in patients <60-year-old) a single screening scan in first-
particular, patients with BAV-related aortopathy may show stable dila degree relatives may be considered. However, there are no available
tion of the ascending aorta over several years and, therefore, the fre data on the cost-effectiveness of such an imaging screening strategy
quency of repeated imaging follow-up should be individualized. On in these individuals. If the genetic cause is known, aortic imaging is per
the other hand, patients with BAV and no overt aortopathy should formed only in carriers of the mutant gene. In the first-degree relatives
be screened every 3 years with TTE to check for aorta dilation and add of BAV patients, the prevalence of ascending aorta dilation diagnosed by
itionally with repeated CT or CMR every 5 years to reconfirm TTE TTE is around 10%; therefore, screening by TTE should be offered.33
measurements of the aortic root and ascending aorta, as well as to re
assess the arch and the descending thoracic aorta.28 Key point 5. When aortic root or ascending aorta dilation is ini
The annual growth rate of aortic dilatation segments are significantly tially diagnosed by TTE, a multiplanar CT/CMR scan is recom
greater in the descending thoracic aorta, ∼1.9 mm/year, than the ascend mended to confirm TTE measurements, to rule out aortic
ing aorta, ∼0.5 mm/year.5 According to the 2014 European Society of asymmetry, and to have a baseline reference in the follow-up.
Cardiology (ESC) guidelines, an increase >3 mm/year, confirmed by When a baseline aorta diameter is >45 mm, a second exam is
two imaging modalities, is a risk factor prompting consideration of surgical recommended at 6 months to confirm stability of aorta dilation,
intervention in patients with a maximum diameter of 45–50 mm.12 with serial exams performed on a yearly basis thereafter.
The reproducibility of diameter measurements remains imperfect.
Indeed, different studies have shown large inter-centre, inter-observer, Key point 6. A genuine change in ascending aorta diameter, not
and intra-observer variability for these measurements.29 This variability related to measurement variability, can be only considered

Figure 8 (A) Volume rendering of an ECG-gated contrast-enhanced CT angiography acquired during the diastolic phase shows a fusiform aneurysm
of the ascending aorta and a normal arch. Note absence of pulse wave artefacts and motionless depiction of the coronary arteries. (B and C) Alignment
of the transverse aortic plane is simultaneously performed at the most dilated location in coronal (B) and sagittal (C ) multiplanar reconstructions of the
ascending aorta. (D) The corresponding double oblique transverse multiplanar reconstruction of the ascending aorta allows for reliable and reprodu
cible measurement of the maximum aortic distance.
when larger than 2 mm. Any increase ≥3 mm by TTE should be echo-technology and contrast enhancement, the sensitivity of TTE in
always validated by CT/CMR and compared with baseline data. the visualization of the intimal flap has improved up to ∼75–85%.35,36
Important features of AD typically include flap oscillation or motion
that is independent of the aortic wall and visualized in more than one
Acute aortic syndromes view. These features allow distinction from artefact due to reverbera
tions from other structures. In addition, TTE provides assessment of
Acute aortic syndromes (AAS) comprise a range of interrelated condi
left ventricular function, pericardial effusion, AV function, right ven
tions caused by disruption of the medial layer of the aortic wall, includ
tricular size and function, and pulmonary artery pressure (Figure 11;
ing AD, IMH, penetrating atherosclerotic ulcer (PAU) and contained or
see Supplementary data online, Videos S3 and S4).
not contained aortic aneurysm rupture. AAS are potentially life-
Although TTE has a lower sensitivity to diagnose type B AD, particu
threatening: prompt, accurate diagnosis is crucial. These clinical entities
larly in the thoracic aorta segments, the intimal flap can often be visua
are classified as type A and type B AAS depending, respectively, on the
lized in the abdominal aorta, particularly if the Nyquist level in the
involvement or not of the ascending aorta regardless of the site of origin
colour Doppler scale is lowered or contrast used. In addition, when a
(Stanford classification).2,3,5,12,34 Figures 9 and 10 show examples of the
pleural effusion is present views from the patient’s back may also be
Stanford classification.
useful in identifying the flap. The low negative predictive value of TTE
does not rule out AD, and further tests are required if clinical suspicion
Aortic dissection is high and the TTE examination is negative.
AD is defined as a disruption of the medial layer leading to the
formation of two lumens separated by an intimomedial flap. Transoesophageal echocardiography
Echocardiography, CT, and CMR can be used to diagnose AD yielding
complimentary information. The sensitivity of TOE for the diagnosis of AD reaches 99%, with a spe
cificity of 89%.2,3,5 Linear artefacts within the thoracic aorta on TTE and
TOE can be reverberations or sidelobe artefacts. Assessing location and
Transthoracic echocardiography mobility patterns of these lineal images suggestive of reverberations of
TTE often provides adequate assessment of AD in the aortic root and structures nearest to the transducer by M-mode is indeed key for cor
proximal ascending aorta. While TTE can visualize most segments of rect interpretation, but also applying colour flow Doppler and confirm
the rest of the aorta (using left and right parasternal long-axis, supras ation in alternative imaging windows. Alternative modalities (CT/MR)
ternal, apical two-chamber and subcostal scanning planes), the use of are sometimes necessary due to dubious ultrasound artefacts, again
other imaging modalities is usually required in these areas. With current underscoring the value of multimodality imaging in AAS. TOE is also

Figure 9 Classic Stanford type A aortic dissection. (A and B) Volume rendering (A) and oblique coronal reconstructions (B) show the intimal flap
(arrows) extending from the sino-tubular junction to the aortic arch and the descending aorta, dissecting the brachiocephalic artery (asterisk) and
the left common carotid artery (arrowhead). (C and D) Axial CT images show the intimal flap dividing true (T ) and false (F ) lumina at the level of
the main pulmonary artery (C) and of the aortic arch (D). In type A aortic dissection, the false lumen is usually located along the right anterolateral
wall of the ascending aorta and extends distally in a spiral fashion along the left posterolateral wall of the descending aorta.
Figure 10 Acute type B aortic dissection. (A and B) Sagittal (A) and coronal (B) maximum intensity projections of an arterial phase CT angiography of
an acute Stanford type B aortic dissection show the extent of the intimomedial flap from the aortic isthmus to the proximal right common iliac artery.
Note the large size of the false lumen (FL) and absence of a distal entry tear (white arrow). (C–E) CT images show an isthmic entry tear (C ), circum
ferential involvement at the retropulmonary descending thoracic aorta (D) with a centrally located true lumen (black asterisk) and abdominal dynamic
ischemic configuration of the flap with compression of the true lumen at the ostium of the coeliac trunk (E) and a non-enhancing ischemic left kidney. (F)
Multiplanar reconstructions show involvement of the coeliac trunk and the superior mesenteric artery with ischemic configuration. The flap extends
into the proximal segments of both arteries, but the lack of a distal tear results in a cul-de-sac of the false lumen of the coeliac trunk (small arrow) and
toral thrombosis of the FL mesenteric artery that compresses the true lumen of the visceral arteries.
very useful for locating and measuring the size of the primary entry tear moves towards the FL at the start of systole by expansion of the TL
and for visualizing secondary communications by colour Doppler as well (Figure 12; see Supplementary data online, Videos S5–S10).
as the presence of thrombus in the FL. The FL is usually larger and has less TOE is also the best technique for defining the mechanisms under
flow than the true lumen (TL). M-mode TOE shows how the intima lying any associated aortic valvular regurgitation. These mechanisms

Figure 11 Aortic dissection by TTE. (A) Type A aortic dissection with intimal flap (arrow) in the aortic root protruding into the opening of the aortic
valve by parasternal long-axis view; (B) type B aortic dissection with entry tear in proximal descending aorta from the suprasternal view. Arrow shows
the intimal flap between false and true lumen; (C) abdominal aorta dissection, arrow shows the intimal flap; (D) pericardial tamponade (large arrow) with
right ventricular (*) compression. Small arrow shows the intimal flap; (E and F ) severe aortic regurgitation secondary to intimal flap prolapse into the
aortic valve (arrow). AA, ascending aorta; DAo, descending aorta; FL, false lumen; LA, left atrium; LV, left ventricle; TL, true lumen. See also
Supplementary data online, Videos S3 and S4.
potentially include normal AV anatomy with flap invagination causing excellent sensitivity (95% for AD) providing a fast evaluation on the en
interference with valve closure, dilatation of the ascending aorta with sec tire aorta and branches. Sensitivity and specificity for diagnosing arterial
ondary functional regurgitation, or intrinsic AV disease. TOE can differen vessel involvement are 93% and 98%, respectively, with an overall ac
tiate two mechanisms of decreased flow in the arterial trunks in AD: curacy of 96%.2,3,12 CT can also rule out alternative causes of acute
proper dissection of the arterial branch, also called ‘static obstruction’, chest pain, including pulmonary embolism and coronary artery disease
or alternatively, compression of the vessel ostium by the aortic intimal (Figure 11).
flap, known as ‘dynamic obstruction’. Visualization of the upper abdominal As previously described, modern CT acquisition protocols including
aorta segment and the origins of the proximal coeliac trunk and the super ECG gating eliminate aortic pulsation artefacts and pseudoflaps. These
ior mesenteric artery should be included during the TOE assessment.37 protocols typically begin with a low-dose non-contrast CT to help in
3D TOE may provide additional information beyond 2D TOE allow the detection of hyperdense IMH, followed by contrast-enhanced CT
ing better morphologic and dynamic evaluation of the entry tear in AD angiography. In AD, the major role of CT is to confirm the diagnosis
by multiple simultaneous view.38 In addition, contrast TOE is also very and provide measurements of the diameter and extent of dissection,
useful to better define the TL and FL, yielding a comprehensive assess TL and FL description, involvement of organ vasculature and arterial
ment of FL flow dynamics. trunks, and distance from the intimal tear to the organ arterial branches
The main limitation of TOE is the blind spot between the distal ascending (see Supplementary data online, Figure S1). CT is also useful to recog
aorta and the mid segment of the arch. Furthermore, TOE may induce gag nize the different potential configurations of the flap when a visceral ar
ging thereby increasing the systemic blood pressure of the patient. Adequate tery is involved including if the branch originates from the TL or FL, if
sedation is mandatory to avoid such reactive hypertension. Notwithstanding there is flap prolapse into a branch (dynamic obstruction), or if there
this precaution, we think that the systematic use of TOE to diagnose AAS is intimal dissection stopping at a bifurcation (fixed obstruction)
should be avoided and only indicated in cases where marked haemodynamic (Figure 12). Finally, it is also useful to diagnose visceral ischaemia, peri
instability precludes the safe transfer of the patient to the CT scanner or cardial effusion and periaortic haematoma.39 A late thoracoabdominal
when specific information from TOE is essential. If that is the case, TOE scan (1 min after bolus injection) distinguishes slow flow in the FL from
should be always performed by an expert echocardiographer in a patient un thrombosis or IMH, improves the detection of impaired visceral perfu
der adequate sedation or preferably a general anaesthetic. Nonetheless, sion, and frequently allows for alternative diagnoses in low- and
TOE should be performed in the operating room in all patients during repair intermediate-risk patients with negative AAS findings.
of type A AD. Similarly, TOE is essential to guide transcatheter endoluminal
aortic repair procedures, showing the location of entry tears, secondary Cardiovascular magnetic resonance
communications, and changes of FL flow and possible leaks after stent im
CMR can address all issues and details of AD noninvasively with high
plantation by colour Doppler or contrast enhancement.
spatial resolution and functional assessment with high sensitivity
(>97%) and specificity (>94%) for diagnosing AD. However, scan times
Computed tomography are significantly longer than for CT angiography or TOE, and monitor
CT is the most used imaging technique for the evaluation of AAS, par ing of the patient during study acquisition is cumbersome. Therefore, its
ticularly for AD because of its accuracy, widespread availability, and use in the acute phase of AAS is limited to selected cases.

Figure 12 Transoesophageal echocardiography (TOE) in aortic dissection. (A) Entry tear located in proximal descending aorta by 2D TOE; (B) large
entry tear (arrow) by 3D-TOE; (C) by colour Doppler, the flow of the entry tear from true to false lumen is visualized (arrow); (D and E) severe aortic
regurgitation secondary to the prolapse of the flap in the left ventricular outflow tract; (F ) dissection of the coeliac trunk with turbulent flow in the true
lumen. AA, ascending aorta; DAo, descending aorta; LA, left atrium; LV, left ventricle. See also Supplementary data online, Videos S5–S10.
Diagnostic workup 85%. Special attention should be made during the TTE exam to
The diagnostic workup to confirm or to rule out AD is highly dependent aortic root dilatation, aortic regurgitation, and/or pericardial ef
on the a priori risk of this condition based on three groups of variables: fusion, since these findings should raise the suspicion of AAS.
predisposing factors, pain characteristics, and clinical examination that
are included in several proposed risk scores (Table 1). Figure 13 illustrates Key point 8. TOE is a reference technique in the diagnosis and
a comprehensive diagnostic pathway. Currently, TTE is largely per assessment of thoracic AAS but, in this setting, requires ad
formed in patients with chest pain in the emergency room and maybe equate sedation to avoid reactive systemic arterial hyperten
useful to rule out alternative diagnoses such as myocardial infarction or sion. When a diagnosis is definitively established using other
to detect an aortic intimal flap. Moreover, it may identify imaging signs imaging techniques, TOE should be performed preoperatively,
suggestive of AAS despite not visualizing an intimal flap (pericardial effu in the operating theatre under general anaesthesia for comple
sion, aortic valvular regurgitation, aortic dilation, or aortic wall thicken mentary information including entry tear location and size, the
ing).2–5,12,39 Indeed, one of the novelties of this flow chart in mechanism underlying associated aortic regurgitation, and
comparison with previous algorithms is the suggested early implementa other associated features.
tion of TTE. A CT scan of the entire aorta should be performed in all pa
tients with a dissection risk score >1 and increased levels of D-dimers, Key point 9. CT is the imaging technique of choice in the evalu
particularly when the troponin value is normal and there are no ECG ation of AAS because of its accuracy, fast evaluation of the en
changes suggesting myocardial ischaemia as the cause of chest pain. tire aorta and branches, and widespread availability. CT is very
The exception is in patients with haemodynamic instability with high clin useful in the assessment of visceral organ involvement and for
ical suspicion or a confirmed AD on TTE who cannot be transferred to planning optimal therapy. The best imaging strategy for appro
the CT scanner. In these patients, TOE should be performed under deep priately diagnosing AAS and its complications is a combination
sedation or preferably, general anaesthesia prior to surgery. of a bedside TTE and CT.
Key point 7. TTE is currently largely performed in patients with

chest pain in the emergency room and maybe useful to rule out
alternative diagnoses or to detect an aortic intimal flap, particu
Intramural haematoma
larly in the aortic root or abdominal aorta. Visualization of the IMH accounts for ∼10–20% of AAS. Typically, IMH appears as thicken
intimal flap has improved with a diagnostic accuracy of ∼75– ing of the aortic wall in a crescentic or concentric pattern. The aorta
CT without contrast is crucial for the diagnosis of IMH. A high-

Table 1 Comparison of diagnostic accuracy and pros attenuation crescentic thickening of the aortic wall, extending in a lon
and cons of the different imaging modalities in acute gitudinal, non-spiral fashion, is the hallmark of this entity. In contrast to
aortic syndrome
AD, the aortic lumen is rarely compromised in IMH, and no intimal flap
TTE TOE CT CMR or enhancement of the aortic wall is seen after contrast administration.
.................................................................. Using CT, the combination of an unenhanced acquisition followed by a
Diagnostic accuracy contrast-enhanced acquisition yields a sensitivity as high as 96% for the
detection of IMH. However, in some cases, the differentiation of IMH
Ascending aorta dissection ++ +++ +++ +++
from atherosclerotic thickening of the aorta, thrombus, or thrombosed
Aortic arch dissection + ++ +++ +++ dissection and aortitis may be difficult using CT. In those circumstances,
Descending aorta dissection + +++ +++ +++ magnetic resonance imaging (MRI) can be useful to diagnose IMH, as it
Intramural haematoma + +++ +++ +++ offers the possibility of diagnosing intramural bleeding in the hyperacute
phase because the haematoma shows an isointense signal on
Penetretating aortic ulcer + ++ +++ +++
T1-weighted images and a hyperintense signal on T2-weighted images.
Aortic valve morphology and function +++ +++ ++ +++

Beyond the first 24 h, the change from oxyhaemoglobin to methaemo
Dynamic visualization of flap + +++ ++ ++ globin determines a hyperintense signal on both T1-weighted and
Aortic lumen dimensions ++ +++ +++ +++ T2-weighted images. The differential diagnosis with mural thrombus
Thrombosis of the false lumen + +++ +++ +++ is easier using CMR than CT or TOE, because a thrombus appears as
a hypointense or isointense signal in both T1-weighted and
LV, RV functional data +++ +++ + +++
T2-weighted sequences. Focal intimal disruption, yielding the morpho
Non-invasive haemodynamic data +++ ++ — + logic image of ulcer-like projection of the aorta, may appear within the
Coronary anatomy involvement + ++ +++ ++ first days (10–30% of cases) or in the first 6 months after the acute on
Involvement of aortic branches + ++ +++ +++ set of symptoms (30% of cases). Imaging predictors of complication can
be also identified in the acute phase including ascending aorta involve
Pros and cons
ment and maximum aortic diameter >50 mm, development of focal in
Ease of use +++ ++ +++ + timal disruption, progressive maximum wall thickness >11 mm, and
Portability +++ +++ — — haemomediastinum or progressive pleural effusion.40
Time requirement + ++ ++ +++
Key point 10. IMH is diagnosed on the basis of a crescentic or
Radiation — — +++ —
circular wall thickness >5 mmm, in the clinical context of
Nephrotoxicity — — ++ + AAS. Non-contrast CT is very useful showing a high-attenuation
Need for sedation — +++ — — thickening of the aortic wall. Differential diagnosis should be
made with severe atherosclerosis, total thrombosis of the FL
or aortitis. In doubtful cases, CMR is the technique of choice.
The dynamic evolution of IMH in the acute and subacute phase
lumen shape is generally preserved, and the luminal wall is curvilinear requires close imaging surveillance by CT/CMR.
and usually smooth. This may differentiate aortic atherosclerosis and in
traluminal thrombus that present more frequently with a rough, irregu
lar border. IMH is generally a more localized process than classic AD,
which typically propagates along the entire aorta towards the iliac ar
Penetrating atherosclerotic ulcer
teries. Type A IMH may account for 30–40% of cases, whereas type A PAU is defined as an ulceration of an aortic atherosclerotic plaque
B occurs in 60–70% of cases.2,3,12 penetrating through the internal elastic lamina into the media. PAU re
The diagnosis of IMH is challenging and more difficult than AD. Initial present 2–7% of all AAS.2,3 A real PAU must be distinguished from
imaging test results may be negative in >12% of patients; as IMH thick other ‘ulcer-like’ images that have been grouped under the term pene
ening may be progressive, establishing the diagnosis of IMH may require trating aortic ulcer (this term can lead to confusion given its similarity
observation and repeat imaging hours or several days after the clinical and because it can be abbreviated to the same acronym). The terms
onset. IMH can be difficult to distinguish from a total thrombosed FL, penetrating aortic ulcer or ulcer-like projections relate to an imaging
because they can both appear as a crescent-shaped thickening of the morphologic concept, which include several entities of very different
aorta wall. However, total thrombosis of a FL is uncommon in an initial origin and prognosis, each of which requires diagnostic distinction
diagnostic test and dynamic evolution is more characteristic of IMH. from PAU.41 Figure 15 illustrates TOE and CT examples of PAU.
For the detection of an acute IMH, TTE is generally inadequate be PAU may occur anywhere along the length of the aorta but appears
cause of low sensitivity. However, in the presence of suspicion for most often in the mid and distal portions of the descending thoracic
AAS with normal TTE, other imaging modalities are mandatory, and aorta. Propagation of the ulcerative process may either lead to IMH,
TOE, CT, and CMR are the main recommended modalities for the diag pseudoaneurysm, or even aortic rupture.
nosis of IMH. CT is the ideal diagnostic imaging modality for PAU diagnosis as it al
The diagnostic criteria of IMH in TOE includes a wall thickness lows comprehensive visualization of the whole aorta, can identify calcified
>5 mm, although in patients with severe atherosclerosis, a cut-off va atherosclerotic plaques surrounding the ulcer, and detects extraluminal
lue >7 mm is more specific. Key features in distinguishing IMH from abnormalities, including pseudoaneurysms or fluid in the mediastinum
other pathological aortic conditions include adequate identification or pleural space. CMR is excellent for differentiating PAU from IMH, ath
of the intima, which often has a bright echo-dense appearance due erosclerotic plaque, and intraluminal chronic mural thrombus.
to calcification. Moreover, an intraparietal echo-lucent zone has Once developed, PAU may remain quiescent, but the weakened aor
been reported in 70–80% of patients with IMH. Additional signs, tic wall may also evolve into a focal and typically saccular area of dilata
such as periaortic, pericardial or pleural effusion, or mediastinal tion or even to an aortic pseudoaneurysm. External rupture into the
haematoma, support the diagnosis of IMH and indicate overt compli mediastinum or the pleural spaces may rarely occur. Imaging predictors
cations in evolution (Figure 14). of complications in the acute phase are aortic maximum diameter,

Figure 13 Diagnostic algorithm for patients with suspected acute aortic syndrome. See score description in Table 2. Focus TTE*: focus TTE oriented
to rule out acute aortic disease, abnormal LV motion or pericardial effusion. TTE**: comprehensive TTE including evaluation of aortic valve, ventricular
function, and pericardial effusion if NOT performed in Step II. ^TOE indicated only if experts in TOE are available or in patients under artificial ven
tilation and deep sedation/general anaesthesia.
Figure 14 Intramural haematoma in ascending aorta. (A) Echocardiographic orthogonal views in TOE showing circumferential thickening of the aor
tic wall mainly in the anterior wall (arrows); (B) semilunar hyperattenuation (arrow) by non-contrast CT; (C) increased signal intensity of the aortic wall
(arrow) by axial T1-weighted black-blood image in CMR.
periaortic haematoma, pleural effusion, and ulcer size. A maximum Aortic rupture
diameter >12.5 mm or an ulcer depth >9.5 mm have been reported Rupture of the aorta is the last episode in the evolution of an aortic an
as predictors of complications.42 eurysm or an AAS and is characterized by an acute, devastating clinical
presentation that requires emergent repair whenever possible.
Key point 11. The term penetrating aortic ulcer or ulcer-like pro Contained rupture commonly manifests as an aortic pseudoaneurysm
jection relate to an imaging morphologic concept that includes (false aneurysm) defined as a dilation of the aorta due to disruption of all
several entities of very different origin and prognosis and that re wall layers, which is only contained by the periaortic connective tissue.
quires diagnostic distinction from a PAU. CT is the preferred im Rupture is typically diagnosed by CT with periaortic haematoma and, in
aging modality to depict it and differentiate these entities. some cases, identification of a discontinuity in the aortic wall with or
most common location of aorta traumatic injury is at the aortic isthmus

Table 2 Risk score for acute aortic syndrome (adapted just distal to the left subclavian artery. The second most common loca
from Ohle et al.a) tion is the supravalvular portion of the ascending aorta.
Point score Score Contrast-enhanced CT is currently the preferred first-line imaging
.................................................................. technique for blunt aortic injury, especially for patients with multiple in
Clinical history 0: Low-risk probability juries.43 TOE and aortography might be of help when CT findings are
(< 0.5%)
equivocal. In some haemodynamically unstable patients, TOE may be
• Marfan syndrome No risk factors: 0; Any
a first-line test, especially if CT requires transportation to a remote
(or other non-aneurysmal risk 1: Moderate-risk
area.
connective tissue factors: 1; aortic probability (0.5–5%) Iatrogenic injury to the thoracic aorta may occur in the setting of
diseases) aneurysm: 2 ≥2: High-risk catheter-based interventions, during surgery or endovascular treat
• Family history of probability (>5%) ment. Usually, the diagnosis of this lesion is straightforward during angi
aortic disease ography, characterized by stagnation of contrast at the level of the
• Known aortic aortic root or ascending aorta or by the development of IMH. In this

setting, IMH diagnosis requires an additional TOE or CT (see
valve disease
Supplementary data online, Figure S3 and Supplementary data online,
• Known thoracic Videos S11 and S12).
aortic aneurysm Advantages, limitations, and comparison among the different imaging
• Previous aortic modalities in the evaluation of AAS are summarized in Table 2. Because
manipulation of the importance of prompt recognition to their successful treatment,
(including cardiac this table not only emphasizes the diagnostic power of each technique
surgery) but also suggests that not any single modality is preferred for all patients
and that the choice of imaging modality depends on the patient’s clinical
Symptoms
condition and local institutional factors such as expertise and availability.
• Chest, back, or No high-risk pain
abdominal pain features: 0; 1 or 2
described as any of high-risk pain Imaging follow-up after AAS
the following: features: 1; 3 or As patients are still at risk of complications after an AAS, follow-up by
CT or CMR is indicated depending on availability and patient character
- abrupt onset more high-risk pain
istics at 1–3, 6, 12 months, and annually thereafter. After an ascending
- severe intensity features: 2
AD repair, complications such as local bleeding, graft infection, and
- ripping or tearing pseudoaneurysm can be present. Slight fibrotic peri-graft thickening is
Clinical common following surgery; however, large or asymmetrical thickening
examination may represent localized haematoma caused by anastomotic leakage, of
(signs) ten observed at the site of the reimplanted coronaries.
Various poor prognostic imaging signs should be considered after the
- Evidence of No high-risk physical
acute phase of AD. A persistent patent FL in the descending thoracic
perfusion deficit: examination aorta and a maximum aorta diameter ≥45 mm have been related to
- systolic blood findings: 0; any an increased growth rate2 with a high risk of rupture if the maximum
pressure high-risk physical diameter is >60 mm or annual growth >5 mm.12 A large entry tear
difference examination (diameter >10 mm) in the proximal descending aorta is another estab
- focal neurological findings: 2 lished predictor of adverse events.44 An absolute tear area difference
deficit (in (proximal vs. distal tears) >1.2 cm2 has been also considered as an im
portant risk factor.45 However, it may be difficult to identify the distal
conjunction with
re-entry communication; thus, in the presence of a large entry tear, in
pain) direct signs such as TL compression or partial FL thrombosis should be
- pulse deficit considered.2 High systolic antegrade flow in the FL with significant
- aortic diastolic retrograde diastolic flow assessed by 2D phase-contrast MRI identifies
murmur (new and patients with a higher risk of complications.46 2D phase-contrast and
with pain) 4D-flow MRI also hold promise in the functional assessment of FL
- Hypotension or flow after AD (Figure 16).
IMH evolution is very dynamic and may result in complete resorption
shock
with or without aorta dilation, focal intimal disruption leading to ulcer-
a like images, or less frequently classical AD.47 Given their wider field of
Ohle R, Yan JW, Yadav K, Cournoyer A, Savage DW, Jetty P, et al. Diagnosing acute
aortic syndrome: A Canadian clinical practice guideline. CMAJ 2020 Jul 20; 192:
view, CMR and CT are better than TOE at defining this dynamic evo
E832-E843. lution. CMR allows monitoring of the evolution of intramural bleeding
and can depict episodes of new asymptomatic intramural re-bleeding.
An aorta diameter >50 mm and enlargement of focal intimal disrup
without contrast extravasation (see Supplementary data online, tions have been considered risk factors for adverse outcomes, particu
Figure S2). larly in the ascending aorta.2,12
Many patients with PAU do not need immediate aortic repair but do
require close follow-up with serial imaging studies (by CT or CMR) to
Traumatic and iatrogenic injury to the thoracic aorta detect disease progression. In these cases, size and enlargement are the
A variety of aortic lesions can result from blunt aortic trauma such as only predictors of complications and both CT and CMR are the pre
aortic transection, pseudoaneurysm formation, IMH, or AD. The ferred imaging modalities for follow-up.48

Figure 15 Different types of aortic ulcers by TOE (upper panel) and CT (lower panel). (A and B) Atherosclerotic ulcerated plaque protruding into
the aorta lumen; (C and D) focal intimal disruptions (ulcer-like image) with the contrast-filled outpouching that protude from the aortic lumen into the
intramural haematoma (arrows); (E) tiny ulcers (≤3 mm); TOE with colour Doppler may be superior to any other imaging technique for demonstrating
small communications usually associated with intercostal or lumbar artery ostia (white arrows). (F ) contrast CT may also demonstrate these small
communications (black arrow); (G and H ) penetrating atherosclerotic ulcer. Atherosclerotic ulcerated lesion penetrating through the aortic intima
into the aortic wall with a pouch-like protrusion into the aortic wall (arrows).
Key point 12. After an AAS, follow-up by CT or CMR is indicated determine the velocity before the stenosis and the length of the nar
depending on availability and patient characteristics at 1–3, 6, rowed segment. When the coarctation is long or there is extensive col
12 months, and annually thereafter. Imaging signs of poor out lateral circulation, gradients might be less accurate.49 It is important to
come after AD include the following: a persistent patent FL in also look for the Doppler sign of a diastolic tail in the descending thor
the descending thoracic aorta, maximum aorta diameter acic aorta or the typical saw-tooth pattern of anterograde diastolic flow
≥45 mm, large entry tear (diameter >10 mm) in the proximal in the abdominal aorta because these signs indicate significant haemo
descending aorta, and a CMR FL pattern of high systolic ante dynamic impairment of flow. The aortic diameter is difficult to measure
grade flow with significant diastolic retrograde flow. and often not reliable using TTE.
CT or CMR is recommended at the time of initial evaluation to deter
Key point 13. CMR is useful for monitoring the evolution of mine the site and degree of obstruction, to assess all aorta segments and
intramural bleeding and to detect new asymptomatic intra the extent of collateral circulation (Figure 17). Pseudocoarctation can be
mural re-bleeding episodes. Chronic and stable PAU requires differentiated from true coarctation by identifying a high, elongated arch,
close follow-up with serial imaging studies (by CT or CMR) to kinking that lacks luminal narrowing, and the absence of enlarged collateral
detect disease progression. arteries. Periodic follow-up by CT or CMR is also recommended after
intervention to identify restenosis or progressive aorta dilation. TTE might
overestimate restenosis as gradients might be high even in the absence of
Aortic coarctation significant narrowing, due to decreased aorta compliance in these patients.
Coarctation is a local narrowing of the aorta, presenting as a discrete Key point 14. In aorta coarctation, CT or CMR is recommended
stenosis or as a long, hypoplastic segment typically located in the area at the time of initial evaluation to determine the site and degree
where the ductus arteriosus inserts, just distal to the subclavian artery. of obstruction and to assess all aorta segments and the extent of
TTE can usually confirm the diagnosis of aortic coarctation and is used collateral circulation. Patients with mild degrees of coarctation
to identify associated cardiovascular disorders such as a BAV (present who do not require intervention should undergo periodic TTE
in >50% of patients with aortic coarctation) and aorta dilatation. (every 1–2 years) and CT or MRI (every 3–5 years) to monitor
Indeed, it should be always ruled out with TTE in patients with BAV disease progression.
or Turner syndrome due to its relatively high associated prevalence.
TTE evaluation for coarctation is best done via suprasternal windows
and should include colour and CW Doppler assessments of the distal
arch and isthmus. Maximal velocity measurement across the coarcta
Aortic atherosclerosis
tion by CW Doppler provides information on the severity of the sten Atherosclerosis is characterized by the accumulation of lipids, inflam
osis but is not the only parameter to check. It is also important to matory cells, and connective tissue cells within the arterial wall. The

Figure 16 Outcome and predictors of type B aortic dissection. (A) CT images of the proximal descending thoracic aorta in the acute and chronic
phases of a Stanford type B aortic dissection. Note aortic expansion caused by dilation of the hypoenhancing false lumen (FL), with the presence of
partial peripheral thrombosis, indirect signs of slow flow; (B) descending aorta dissection with a large entry tear (arrow) by CMR; (C) phase-contrast
gradient-echo CMR sequence for measuring the true and FL aorta flow at the inferior pulmonary level; (D) instantaneous flow-time curves throughout
the cardiac cycle. Dotted line defines end-systole. Diastolic retrograde (arrow) flow in the FL (in yellow) is a predictor of complications; (E)
volume-rendered image shows the characteristic hypoenhancement of the FL (arrows) and the location of the maximum aortic distance at the isthmus,
close to the large entry tear.
Figure 17 3D-CMR angiography of the thoracic aorta in a patient with severe aortic coarctation (arrow), showing extensive collateral vessels.

Figure 18 Atherosclerosis in descending aorta. (A) Atheroma with thickness >5 mm (arrow) but without any mobile element by TOE; (B) complex
atheroma with mobile components (arrow); (C ) large mobile thrombus defined by TOE in descending aorta (arrow); (D) CT angiography with diffuse
atherosclerosis (arrows). See also Supplementary data online, Videos S13 and S14.
Figure 19 18F-Fluorodeoxyglucose (18F-FDG) PET/CT scan of a patient with large vessel vasculitis. High-intensity signal (arrows) is observed cir
cumferentially around the thoracic aorta. Unpublished images provided by Jason Tarkin, Cambridge.
accumulation of fat-laden macrophages leads to thickening of the in used as an alternative although neither technique provides information
timal layer with further progression into a mature atherosclerotic pla on current disease activity.
que. The location and characteristics of the aortic atherosclerotic 18F-FDG PET/CT provides an assessment of inflammatory activity in
burden can be partially described with TTE, but particularly TOE. the aorta and improves the detection of aortitis beyond CT at the seg
TOE is the reference echocardiographic method for the evaluation of ment level, detecting inflamed sections that look normal on CT and
thoracic aortic atherosclerosis depicting its location (descending, providing prognostic information.51 In aortitis, circumferential high-
arch, ascending aorta) and severity. Several classifications have been intensity 18F-FDG activity is observed that can be differentiated from
proposed to quantify severity of aortic atherosclerosis. One of the the more regional and lower intensity 18F-FDG uptake observed in ath
most accepted ones and recommended by this expert panel is to con erosclerosis (Figure 19). 18F-FDG PET also holds promise in tracking
sidered mild atherosclerosis when intimal thickening (focal or diffuse) is disease activity with time and assessing the efficacy of glucocorticoid
2–3 mm (grade I), moderate when the atheroma thickness is <4 mm and immunosuppressant therapy with further research in this area
(grade II), severe when it is >4 mm (grade III), and complex when required.
any grade has associated mobile or ulcerated components (grade IV).
Mobile lesions can be (i) discrete: 1–2 mm mobile lesions, (ii) long, slen Key point 16. Circumferential thickening of the aortic wall on CT

der lesions that moves freely in the pulsatile flow of aorta, and (iii) a or CMR is a marker of aortitis. 18F-FDG PET provides an assess
large mass that rocks with aortic blood flow. The identification of pro ment of inflammation that can help establish an early diagnosis
truding atheroma ≥4 mm has been associated with stroke (Figure 18; of aortitis allow monitoring of disease progression and treatment
see Supplementary data online, Videos S13 and S14). However, TOE and evaluation of vascular complications and relapse.
is less accurate in distinguish thickening, fibrosis, or calcification in the
thoracic aorta than CT/CMR. Large aortic thrombi may lead to a false-
positive diagnosis of dissection, particularly in the context of acute per Conclusion
ipheral ischaemia. TEE allows accurate diagnosis and monitoring of
thrombus size with time and response to anticoagulant therapy (see Multimodality imaging plays a pivotal role in the diagnosis and manage
Supplementary data online, Video S15). ment of thoracic aortic diseases. Since maximum aortic diameter is a
CT is a useful imaging modality to detect the atherosclerotic burden cornerstone parameter to define evolution, prognosis and timing of
of thoracic aorta (Figure 18). Non-contrast CT is used to assess aortic intervention, the use of the recommended conventions to measure it
calcification as a surrogate marker of the total atherosclerotic plaque is required to improve accuracy and reproducibility among imaging
burden. Contrast CT angiography allows assessment of arterial wall techniques. During follow-up, direct comparison of images is important
thickness and the burden of both calcific and non-calcific atherosclerot to assess and detect progression of disease and evolutive changes.
ic plaque with high specificity for detecting aortic arch atheromas.50 Measurement by echocardiography using the leading-to-leading con
Fluorodeoxyglucose (FDG)-PET uptake provides a measure of meta vention and by CT/CMR using the inner-to-inner convention at end-
bolic activity and inflammation in aortic atheroma. Evaluation of thor diastole should be always adopted. In AAS, the combination of CT
acic aorta calcification by CT is particularly important in elderly with TTE is the preferred and most efficient imaging strategy, while
patients undergoing cardiac surgery, in patients with previous history CT and CMR are the recommended ones for follow-up. Each imaging
of radiotherapy or in in patients with previous CABG and a plan for re technique has strengths and limitations that should be considered be
peat cardiac surgery. fore indication of a test in different clinical scenarios to answer the right
clinical questions and provide essential or complementary information
Key point 15. Although the suprasternal window by TTE may al to apply the best care to patients with a potentially life threatening con
low identification of atherosclerotic plaques in the aortic arch, dition in the acute and chronic thoracic aortic diseases.
TOE permits the visualization of most thoracic aorta segments
and the accurate measurement complex mobile plaques and is
therefore the reference imaging modality. The severity and lo Supplementary material
cation of the most severe atherosclerotic plaques should be re Supplementary materials are available at European Heart Journal -
ported. CT and PET are both also useful imaging modalities to Cardiovascular Imaging online.
detect atherosclerotic burden and disease activity respectively
in the thoracic aorta. Funding
None declared.
Conflict of interest: A.E.: Nothing to be declared. M.S.: Speaker and
Aortitis consultancy fees from Abbott (Heart Valve Disease), Edwards
Lifesciences (Heart Valve Disease), Medtronic (Heart Valve Disease).
Aortitis includes all conditions leading to inflammation of the aortic Research funding from Bristol Myers Squibb (Atrial Fibrillation),
wall. Imaging features include mural (inflammation, oedema, and fibro Menarini (Education on Cardiovascular Diseases), Novartis (Heart
sis) and luminal alterations (dilatation/dissection, stenosis, and throm Failure), Rovi (Heart Failure), Amgen (Hypercholesterolemia), Merck
bosis). Ultimately, vessel wall hypertrophy can cause arterial stenosis Sharp & Dohme (Hypercholesterolemia). V.A.: Speaker and consultancy
and vessel occlusion. Hence, the early detection of aortitis is pivotal, fa fees from Bayer Healthcare (Antithrombotic therapies), Novo-Nordisk
cilitating identification and monitoring of the disease and the direction (Diabetes), Astrazeneca (Diabetes), Boehringer Ingelheim (Diabetes).
of appropriate therapy. E.B.: Nothing to be declared. H.C.: Nothing to be declared. M.R.D.:
CMR, CT, and echocardiography can demonstrate homogeneous Speaker and consultancy fees from Beren, Silence Therapeutics (aortic
circumferential thickening of the aortic wall with a uniform smooth in stenosis), Jupiter Bioventures (coronary atherosclerosis), Novartis (aor
ternal surface, which is different from the appearance of atherosclerosis tic stenosis, coronary atherosclerosis), Pfizer (cardiac amyloidosis).
but may be misdiagnosed as IMH. CT detects the characteristic aortic M.G.: Nothing to be declared. G.J.: Nothing to be declared. L.M.: Speaker
wall thickening observed in large vessel vasculitis as well as the vascular and consultancy fees from Bayer (anticoagulants), Daiichi Sankyo (lipid low
complications, providing a highly sensitive diagnostic technique. ering), Novartis (lipid lowering), Sanofi Aventis (lipid lowering). Research
Black-blood CMR sequences provide similar information and may be funding from Bayer (anticoagulants), Daiichi Sankyo (lipid lowering),
Novartis (lipid lowering), Sanofi Aventis (lipid lowering). R.N.: Speaker and 17. Bons LR, Duijnhouwer AL, Boccalini S, van den Hoven AT, van der Vlugt MJ, Chelu RG
consultancy fees from Bayer and Sanofi Genzyme. Research funding from et al. Intermodality variation of aortic dimensions: how, where and when to measure the
ascending aorta. Int J Cardiol 2019;276:230–5.
Biotronik and Philips Volcano. M.P.: Nothing to be declared. G.P.:
18. van Hout MJ, Scholte AJ, Juffermans JF, Westenberg JJ, Zhong L, Zhou X et al. How to
Nothing to be declared. J.R.-P.: Speaker and consultancy fees from Pfizer measure the aorta using MRI: a practical guide. J Magn Reson Imaging 2020;52:971–7.
(Amyloidosis), Takeda Pharmaceuticals (Fabry disease), Amicus (Fabry dis 19. Vis JC, Rodríguez-Palomares JF, Teixidó-Tura G, Galian-Gay L, Granato C, Guala A et al.
ease). Research funding from General Electric and Circle Cardiovascular Implications of asymmetry and valvular morphotype on echocardiographic measure
Imaging, Inc (Advanced cardiac imaging). ments of the aortic root in bicuspid aortic valve. J Am Soc Echocardiogr 2019;32:105–12.
J.W.R.-H.: Nothing to be declared. R.v.K.: Speaker and consultancy fees 20. Plonek T, Berezowski M, Bochenek M, Filip G, Rylski B, Golesworthy T et al. A compari
from Novartis (heart failure), Sanofi Aventis (heart failure), Bayer son of aortic root measurements by echocardiography and computed tomography. J
Thorac Cardiovasc Surg 2019;157:479–86.
Netherlands (heart failure). Research funding from Novartis (heart failure).
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Reference values for echocardiographic assessment of the diameter of the aortic root
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a review consensus paper, and the data presented are related to the corre in the Copenhagen General Population Study. Eur Heart J CardiovascImaging 2019;20:

sponding references inserted throughout the text. 939–48.
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European Heart Journal - Cardiovascular Imaging (2023) 24, e65–e85 EACVI DOCUMENT

Multimodality imaging in thoracic aortic diseases:

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* Corresponding author. Tel: +34 932175153. E-mail: [email protected]

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Key point 7. TTE is currently largely performed in patients with

CT without contrast is crucial for the diagnosis of IMH. A high-

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most common location of aorta traumatic injury is at the aortic isthmus

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