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European Heart Journal - Cardiovascular Imaging Advance Access published October 29, 2012

European Heart Journal – Cardiovascular Imaging REVIEW


doi:10.1093/ehjci/jes215

The role of imaging in acute aortic syndromes


Christoph A. Nienaber*
Divisions of Cardiology, Pulmology and Intensive Care Unit, Department of Internal Medicine I, Heart Center Rostock, Rostock School of Medicine, University Hospital Rostock,
Ernst-Heydemann-Str. 6, 18057 Rostock, Germany

Received 14 August 2012; accepted after revision 24 September 2012

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The classic entity of life-threatening aortic dissection represents one pathology of a spectrum of acute conditions coined the acute aortic
syndrome comprising dissection, intramural haematoma, penetrating atherosclerotic ulcer, and contained aortic rupture of any cause. The
common denominator is disruption of the aortic media layers associated with severe pain and a variety of other symptoms. Any clinical
suspicion of acute aortic syndrome should prompt immediate action and confirmatory non-invasive imaging; with respect to sensitivity and
specificity for acute aortic pathology modern contrast-enhanced CT technology, MR imaging and ultrasound techniques have similar diag-
nostic accuracy near 100%. Since the prognosis of most patients with acute aortic dissection is related to undelayed diagnosis and (often
surgical) treatment swift diagnostic imaging should be the primary goal in the work-up of any patient with suspected acute aortic syn-
drome; transfer and in-hospital logistics and local expertise for the differential use of various imaging modalities should be constantly
improved.
-----------------------------------------------------------------------------------------------------------------------------------------------------------
Keywords Aortic dissection † CT angiography † MRI † Diagnostic algorithm

for classic dissection and in Class 2 for intramural haematoma is


Introduction less popular.
Acute aortic syndrome is a modern term and consists of inter-
related emergency conditions with similar clinical characteristics
and challenges. These conditions include aortic dissection, intra- Confirmatory imaging for acute
mural haematoma (IMH), penetrating atherosclerotic ulcer (PAU
and aortic rupture); trauma to the aorta with intimal laceration
aortic syndrome
may also be considered. The common denominator of AAS is dis- Diagnostic imaging studies in the setting of the clinical suspicion of
ruption of the media layer of the aorta with bleeding within the dissection have important primary goals such as confirmation of
layers as IMH, along the wall of the aorta resulting in separation clinical suspicion, classification of dissection, localization of tears,
of the layers of the aorta (dissection), or transmurally through and the assessment of both extent of dissection and indicators
the wall in the case of ruptured PAU or trauma.1,2 In the majority of urgency (e.g. pericardial, mediastinal, or pleural hemorrhage);
of patients (90%), an intimal disruption is present that results in in addition biomarkers (such as myocardial markers, D-dimer eleva-
tracking of the blood in a dissection plane within the media poten- tion .500 mg/L, and smooth muscle myosin heavy chain) may be
tially rupturing through the adventitia or back through the intima used strategically in concert with swift aortic imaging, although an
into the aortic lumen (Figure 1). Relatively simple classification ideal algorithm has yet to be determined.5 – 10 A concise selection
systems are still being used to share both descriptive and prognos- of imaging modalities is summarized in Table 1. The suspicion of
tic information in scenarios of aortic dissection (Figure 2).3,4 The acute aortic syndrome is high with abrupt or severe retrosternal
Stanford classification divides aortic dissection in type A and or interscapular chest pain often migrating down the back; asso-
B. Type A involves the ascending aorta, and eventually progresses ciated findings can produce signs of acute aortic insufficiency, peri-
distally. Type B dissection starts at the level of the descending cardial effusion, or occluded aortic sidebranches causing ischaemia
aorta. The DeBakey classification distinguishes types I, II, and III, or pulse differential. With predisposing factors, such as hyperten-
with type I involving both the ascending and descending aorta, sion, connective tissue disorders, bicuspid aortic valve, coarctation,
type II only the ascending aorta and the arch, and type III sparing and previous cardiac surgery or recent percutaneous instrumenta-
the ascending aorta and the arch. Further subdivision in Class 1 tion, undelayed diagnostic imaging is required for any of the above

* Corresponding author. Tel: +49 0381 494 77 00; fax: +49 0381 494 77 02, Email: christoph.nienaber@med.uni-rostock.de
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2012. For permissions please email: journals.permissions@oup.com
Page 2 of 9 C.A. Nienaber

Figure 1 Schematic of aortic dissection (left), penetrating ulcer (middle), and intramural haematoma (IMH) (right) all causing acute aortic
syndrome.

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Figure 2 Aortic dissection classification: DeBakey and Stanford classification as the currently most frequently used classification systems for
aortic dissection.

symptoms. While transthoracic ultrasound provides vital informa- without the need for invasive angiography even in the presence
tion (new-onset aortic insufficiency, pericardial effusion, or even of ST changes.
visualization of proximal dissection), additional transoesophageal
(TOE) interrogation of the thoracic aorta is the logical next step,
or MD-CT scanning of the entire aorta if considered safe.11 – 15
Choice of imaging modality
Both imaging modalities provide further detail beyond classification Considering the excellent accuracy of all modalities, imaging proto-
as type A and B (or distal) dissection and allow for strategic plan- cols for both chronic and suspected acute aortic diseases should
ning; ultrasound technology is portable, avoids transport of a crit- adapt to local expertise and to specific questions about the
ically ill patient and may even be hold in the operating theatre15. target of interest.11,12,16 For unstable patients with suspected
MRI has no place in urgent diagnostic work-up of acutely symp- aortic syndromes in the emergency room often unfit for transpor-
tomatic patients. Additional information not essential for immedi- tation, bedside echocardiographic techniques combined with
ate management decisions such as coronary, arch vessel, and transoesophageal echogram and colour-Doppler interrogation
side-branch involvement is usually depicted on CT angiograms have first priority, even though abdominal segments of the aorta
Role of imaging in acute aortic syndromes Page 3 of 9

Table 1 Diagnostic Algorithm (Aortic Dissection).

Clinical suspicion of AAS


...............................................................................................................................................................................
Non-imaging exams Unstable/critical conditions Follow-up evaluation
...............................................................................................................................................................................
Compulsory: TEE with the colour-Doppler MRI with Gd enhancement
ECG, chest radiography, biomarkers MD-CT with CT angiography MR angiography (with or without Gd)
(TNT, TNI, D-dimer .500 mg/L) 3D reconstruction
Virtual angioscopy
Stable clinical condition
Optional: TEE with the colour-Doppler flow MRI with Gd enhancement
ECG, chest radiography, biomarkers MD-CT with CT angiography or MRI with MR angiography (with or without Gd)
(TNT, TNI, and D-dimer) MR angiography 3D reconstruction
Angiography rarely required Virtual angioscopy

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AAS, acute aortic syndrome; TNT, troponin t; TNI, troponin I; MD-CT, multidetector computer tomography; Gd, gadolinium; TEE, transoesophageal echocardiography.

Figure 3 Stanford type B aortic dissection on volume-rendered 64-slice CT angiography with severly compressed true lumen (TL) and
expanded false lumen (FL). Axial slices are displayed in the centre. The proximal entry to the dissection (black arrow) is adjacent to the offspring
of a Lusorian artery (white arrow).

may not be seen from standard subcostal views.1,3,8 Conversely, intramural haematoma and asymptomatic aortic flaps, aortic
rapid acquisition CT technology using 16-, 64-, and even ulcers, and aneurysms are reported at increasing frequency with
256-slice CT scanners has essentially replaced invasive diagnostic access to tomographic imaging.8,17
angiography for large- and medium-sized vessels of both the In contrast to both CT and MR technology, modern ultrasound
chest and the abdomen (Figure 3). The technology is robust and equipment is mobile and especially attractive at the bedside for un-
rapidly performed with high spatial resolution to differentiate intra- stable cases. TEE interrogation added to transthoracic suprasternal
mural haematoma from ulcers and dissection of the aorta, but screening ultrasound is superb for acute aortic dissection (type A)
requires transportation and stable haemodynamic conditions.13,14 even intraoperatively with near perfect sensitivity and specifi-
MR angiography (MRA) is also capable of high-resolution aortic city,12,15 even considering a blind spot at the proximal arch from
imaging with three–dimensional post-processing; delayed imaging bronchial air. Colour Doppler is instrumental to assess entry
allows the evaluation of venous structures without additional con- sites and false lumen flow in real time in order to confirm proximal
trast. The ability to image thin intimal flaps, intramural processes, dissection (Figure 5). In addition, important prognostic information,
and the morphology of aortic wall inflammation may offer new such as pericardial effusion, acute aortic regurgitation, and prox-
insight into vascular disease detection and classification. Indeed, imal coronary obstruction, can be visualized. For patients in
Page 4 of 9 C.A. Nienaber

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Figure 4 (A) Coronal MIP 3D MR-angiogram shows significant stenosis of the right common carotid artery (small arrow). The left subclavian
artery reveals two stenotic segments (large arrows) with some post-stenotic irregularities of the left common carotid artery. Axial unenhanced
(C) and gadolinium-enhanced (B) T1-weighted MR-images show wall thickening of the ascending aorta (arrows).

shock and very high clinical suspicion of ascending aortic Specific considerations in the
dissection, transthoracic echocardiography (TTE) alone seems rea-
sonable prior to surgery, with TEE performed prior to sternotomy choice of a modality
even in the operating room. Although both ultrasound techniques MRA is not as severely affected by calcification as is CT. Heavily
are important bedside tools for acute dissection, both fail to calcified arteries may still induce an artefact on MR angiograms,
provide sufficient anatomic detail to plan endovascular but luminal narrowings and intramural haematoma are depicted
interventions.13 even in the presence of atherosclerotic calcification. Thus, MRA
Catheter-based and digital subtraction angiography as well as is better suited for evaluating occlusive disease of medium-sized ar-
ultrasound-based techniques have recently been replaced by teries and, therefore, modality of choice for patients with lower or
contrast-enhanced CT and MRA; MRA requires no iodinated con- upper extremity vascular disease (Figure 4). Despite easier post-
trast or ionizing radiation, allows three-dimensional multiplanar ac- processing MRA has lower spatial resolution than CT and images
quisition, and is particularly useful for patients unable to tolerate are sensitive to metal (implanted clips or stents) causing distortion
contrast due to allergy or renal failure. With acute aortic dissection and artefact. Patients with pacemakers, defibrillators, or older
linked to young patients with fibrillinopathies, diagnostic evaluation mechanical valves are confined to CT angiography (contraindica-
during pregnancy and lactation becomes an issue. Whereas foetal tions for MRI). CT is less sensitive to small implants, but large me-
radiation exposure needs to be avoided (in the initial 20 weeks tallic objects, such as hip prostheses degrade image quality. With
of pregnancy) for teratogenic and carcinogenic reason,18 any recent scanners complex vessel morphology as seen in dissections,
iodine component of contrast media given during pregnancy has irregular aneurysms, and vascular tortuosity is better delineated
potential to initiate neonatal hypothyroidism. Even with MR con- ECG gated by CT, whereas MR imaging may produce an artefact
trast agents a minimal teratogenic risk cannot be excluded since in areas of the turbulent blood flow. 21,22 In cases of iodinated con-
paramagnetic agents like gadolinium cross the placenta with trast allergy, CT angiography can also be performed after i.v. injec-
unknown long-term effects. Although exposure during the first tri- tion of gadolinium (60–80 mL); although gadolinium provides less
mester has not been associated with an adverse impact on the intense enhancement, diagnostic images are feasible. The status of
foetus, controversy is ongoing. While European guidelines consider branch arteries and quantitative morphology of both aneurysm or
gadolinium ‘probably safe during pregnancy’ since distributed dissection on CT or MRI is essential for strategic planning and thus
extracellularly and eliminated into urine, most centres in the either modality is recommended prior to endovascular repair of
USA discourage gadolinium during pregnancy in fear of retention aneurysm and dissection.13,22
in amniotic fluid and toxicity.19 For suspected aortic disease in Both MR and CT angiography are useful for extra-aortic path-
pregnancy, non-contrast MRI using steady-state free precession ologies, often vascular in nature such as mycotic aneurysms or
(SSFP) techniques ensures both maximum safety and high diagnos- traumatic pseudoaneurysm, and even for inflammatory vascular
tic precision.20 disease. Moreover, the loss of elastic properties, aortic shear
Role of imaging in acute aortic syndromes Page 5 of 9

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Figure 5 Diagnosis of type A aortic dissection by TEE with arrows pointing to the intimal flap (left) and surgical treatment options (right). FL,
false lumen; TL, true lumen.

stress, and increased wall tension can be quantified with flow- advantages of modern CT include rapid image acquisition,
sensitive MR sequences. For serial follow-up after surgical or endo- post-processing flexibility, and less image noise resulting in high-
vascular repair, three-dimensional MRI sequences are preferred definition CTA from the neck to the abdomen in ,20 s. A signifi-
particularly when stent-graft components consist of nitinol; in cant drawback of MD-CT is a radiation dose of 10 –25 mSv,
the presence of stainless steel MD-CT is better to identify endo- especially of concern in young patients often subject to serial car-
leak or confirm complete isolation of an aneurysm since steel diovascular imaging.12,14,23
causes MRI artefact from magnetic disturbances. In suspected acute aortic syndrome, a non-contrast scan
through the chest is useful to screen for acute haemorrhage or
intimal vascular calcifications, particularly in the aortic wall (intra-
CT angiography of the aorta mural haematoma or separation of wall layers). Subsequently, to
Whereas invasive angiography visualizes two-dimensional lumino- facilitate three-dimensional reformatting a thin (1 mm) scan is
grams, non–invasive multi-detector CT is the result of rapid acquired during rapid bolus administration of contrast at
image acquisition and three-dimensional reconstruction in high 4 –5 mL/s. Since vascular imaging is dependent on the iodine flux
resolution during brief contrast opacification. The technical evolu- (iodine concentration multiplied by the flow rate) excellent vascu-
tion with MD-CT was the cone-shaped X-ray beam generating lar images require injections via venous catheters or implanted
image data over a volume, rather than from an axial section per ro- ports.24 Arterial phase imaging has to coincide with arterial con-
tation, being displayed as three –dimensional image along the cen- trast opacification; delayed venous phase imaging is also useful to
treline of the flow (useful for evaluating vascular disease evaluate solid organs for mass lesions or for endoleaks in patients
morphology and planning endovascular procedures). The ability with stent-grafts (by the use of three-dimensional multi-planar re-
to view vessels in multiple projections and orientations helps construction). In aortic sidebranches such as coronaries or renal
evaluating three –dimensional anatomy of the aorta accentuated arteries, CT angiography tends to overestimate calcified stenoses
by tortuosity, dilation, or spiralling dissection (Figure 3). The and underestimate luminal narrowing by a non-calcific plaque,
Page 6 of 9 C.A. Nienaber

potentially relevant when correlating clinical signs with imaging and interpretation. Yet, the adjunctive use of colour-Doppler in-
findings. Occasionally, CT artefacts occur in obese or uncoopera- terrogation is instrumental to confirm the blood flow in both
tive patients with noise and motion causing image distortion. ECG- true and false lumen, to identify communication sites, to visualize
gated CT acquisition can improve image quality especially in the dynamic side-branch obstruction and other aortic emergencies15;
aortic root, where pulsation artefacts are common and likely to in selected cases contrast-enhanced echocardiography (including
mimic dissection. Meanwhile, first reports emphasize the potential TEE) may facilitate the differentiation of true and false lumen27,28
of virtual vascular endoscopy based on gated acquisition for both and three-dimensional echograms may soon further improve
detailed anatomic evaluation and therapeutic guidance. 25,26 image interpretation. TEE is limited in assessing abdominal side
branches and unpleasant for patients who cannot tolerate
topical anaesthesia or conscious sedation. Given these issues,
Ultrasound/transoesophageal and considering availability, excellent quality, and scanning
speed of multi-detector CT angiography, TEE may be advanta-
echocardiography geous for urgent cases of suspected type A dissection. Never-

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TTE has limited value for the evaluation of the entire aorta, but theless, TEE is an important imaging adjunct to perform
is highly useful in identifying aortic valve dysfunction, pericardial endovascular stent-grafting in complicated type B dissection
tamponade or wall motion abnormalities, and may screen for and to document immediate procedural success.27
proximal and descending aortic dissection in patients with
shock in the emergency room. It is limited, however, in visualiz-
ing the distal ascending and transverse aorta. Advantages of TEE Magnetic resonance imaging of the
for the detection of acute aortic syndromes result from close
proximity of the oesophagus to the thoracic aorta and its
thoracic aorta
ability to visualize both the ascending and the descending aorta MRA is a complementary, rather than competing imaging modality
and parts of the arch with high spatial resolution in real time. for the thoracic aorta. With neither ionizing radiation, nor iodi-
Although TEE requires oesophageal intubation, it is portable nated contrast required, MRA is ideal for patients with multiple
and images are acquired at bedside and immediately interpreted follow-up scans and/or contrast allergies.
(Figure 5). Aortic dissection is confirmed when two lumens are Spin echo T1-weighted imaging provides best patho-anatomical
separated by an intimal flap visualized within the aorta. Tears detail of intramural haematoma, intimal flaps, or atheromas,
can be identified and differentiation between true and false whereas T2-weighted images allow tissue characterization of
lumen is often easy and diagnostic with optional colour-Doppler aortic wall or blood compounds (Figure 6). ECG triggering is essen-
flow mapping or contrast ultrasound27; intimal tear(s) can be tial to minimize pulsatility artefact. With additional preparatory
localized in the majority of patients.7,8 Furthermore, variants of radiofrequency pulses superior black blood fast T1- and
acute aortic syndromes such as IMH, atherosclerotic penetrating T2-weighted sequences are generated and improve image quality
ulcers, and side-branch obstruction can also be identified.17 in any plane.26 Dynamic and functional information is derived
Overall, the European Cooperative Study Group and others from gradient-echo MRI based on flow-related signal enhancement.
showed that TEE can reach a sensitivity of 99% with a specificity Although MR angiographic methods without contrast enhance-
of 89%, positive-predictive accuracy of 89%, and negative predict- ment have been available for a long time, gadolinium-enhanced
ive accuracy of 99% findings later confirmed in IRAD.1,2,11 Al- MRA has dramatically shortened examination time and emerged
though TEE is swiftly performed in unstable patients at as preferred MR modality for aortic disease; adequate images
bedside, an experienced operator is needed for image acquisition result from only 15 mL of gadolinium.29 Although MRA was

Figure 6 Magnetic resonance spin-echo images in a patient with intramural haematoma identified by arrows of the ascending and descending
thoracic aorta: Acute phase T1-weighted SE images show circular wall thickening of the ascending and descending aorta (A); T2-weighted images
show high signal intensity indicative of fresh intramural blood (B). In the subacute phase, the formation of methaemoglobin within the aortic wall
is identified by high signal intensity in T1-weighted SE sequences (C).
Role of imaging in acute aortic syndromes Page 7 of 9

considered ideal in renal failure, discovery of nephrogenic systemic Catheter angiography and
fibrosis in patients with renal dysfunction receiving gadolinium has
tempered enthusiasm and contributed to a renaissance of intravascular ultrasound
non-enhanced MRA.30 Among established non-enhanced Although no longer used for an initial diagnosis of aortic dissection,
sequences beyond ECG-gated partial Fourier fast spin echo, catheter angiography and IVUS may be useful during endovascular
balanced SSFP has emerged as a central technique to provide procedures or when non-invasive modalities were inconclu-
vivid imaging of flowing blood.30 Non-contrast SSFP imaging sive.31,32 The sensitivity and specificity of angiography for diagnos-
enables rapid exclusion of dissection in the ‘single shot’ mode ing aortic dissection are 88 and 95%, respectively,33 with a
and a more detailed evaluation (for entry location and flow relatively high rate of false-negative studies because of occasionally
pattern) in the cine mode both visualizing dissection in any inadequate opacification of the false lumen or in the stage of IMH.
plane. The high signal-to-noise and contrast-to-noise ratio (due Incomplete visualization of the false lumen can also occur when the
to cardiac and respiratory gating) renders SSFP particularly useful catheter tip is placed distal to the primary tear. Other important
for patients incapable of breath holding, or in suspected aortic syn- features of aortic dissection that cannot be visualized by a lumino-

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drome with better detection of aortic wall pathologies such as graphical modality include aortic wall thickness in patients with
intramural haematoma than by MRA alone. True MRA without aortitis or the aortic diameter in the presence of mural thrombus
gadolinium is also feasible, but inferior to gadolinium-enhanced and of course extraluminal findings. Moreover, cost and time
MRA and three –dimensional volume-rendering or maximum in- required for angiography is considerably greater than for cross-
tensity reconstruction (Figure 7). sectional imaging. With almost no role in the diagnostic work-up
The ability of MRI acquisition in any plane and three dimensions in the setting of suspected dissection angiography is being used
enables swift and high-resolution imaging for both chronic patholo- during endovascular management of these conditions, primarily
gies or even acute aortic dissection. Because of the closed bore to seal communications between the true and false lumen, and
design of the magnet and the need for monitoring and resuscitative for the placement of endovascular stent-grafts.34,35
equipment close to magnetic field, MRI is less suitable for unstable
patients than CT. Both for CT and MRI, real-time video sequences
and serial examinations allow assessing instantaneous haemo-
dynamics and longitudinal evolution in transition from acute to
Integration of imaging and
chronic aortic dissection; four-dimensional imaging including time intervention
domain is eventually becoming standard. Post-processing involves
In contrast to both CT and MR technology, modern ultrasound
multi-planar, volume-rendered, and MIP reconstructions, as well
equipment is mobile and especially attractive at the bedside for un-
as virtual endoscopy for complex evaluation of aortic dimensions,
stable emergency cases. TEE interrogation added to transthoracic
coarctation, parietal thrombus and ulcers, dissection, intramural
suprasternal screening ultrasound is superb for acute aortic dissec-
haematoma, and perivascular fat.
tion (type A) even intraoperatively with near perfect sensitivity and

Figure 7 Contrast-enhanced MR angiogram of type B dissection originating from the aortic arch in maximum intensity projection (MIP) (A)
and as volume-rendered 3D reconstruction (B). Arrow identified the entry location. Follow-up MR angiogram at 7 days reveals no communi-
cation after stent-graft, a sealed proximal entry, and a thrombosed false lumen. The TL diameter is normalized and the descending aorta is
reconstructed (C).
Page 8 of 9 C.A. Nienaber

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