Thoracic Aorta - the Acute Aortic Syndrome

Aortic Dissection, Intramural Hematoma and Penetrating Ulcer

Ferco Berger, Robin Smithuis, Otto van Delden
From the Radiology Department of the Academical Medical Centre, Amsterdam and the Rijnland Hospital, Leiderdorp, the
Netherlands

Publicationdate April 10, 2006

The term Acute Aortic Syndrome (AAS) is used to describe

three closely related emergency entities of the thoracic
aorta: classic Aortic Dissection (AD), Intramural Hematoma
(IMH) and Penetrating Atherosclerotic Ulcer (PAU).
Clinically these conditions are indistinguishable.
CT is the most accurate imaging modality for the initial
diagnosis, differentiation and staging.
This review will discuss the imaging features and important
pitfalls.

by Ferco Berger, Otto van Delden and Robin Smithuis

Radiology department of the Academical Medical Centre,
Amsterdam and the Rijnland Hospital, Leiderdorp, the
Netherlands.

Imaging Protocol
 Image protocol will be based on the type of scanner that is
available.
Our imaging protocol is based on a 4 slice helical CT-
scanner.
For the evaluation of patients with suspected AAS, we use
4x2,5 mm collimation technique with 5 mm axial
reconstructions and coronal, sagittal and oblique MPRs.

A non-enhanced scan of the thoracic aorta is included for

the detection of an intramural hematoma (IMH).
This is followed by a contrast-enhanced scan of the aorta in
the arterial phase with bolus triggering and in the venous
phase (100 ml Visipaque, 3ml/sec).
Contrast differences between arterial and venous phase can
be helpful in differentiating true and false lumen.
The iliac tract is included for evaluation of endovascular
treatment possibilities.
The branches of the arch are visualized to evaluate the
extend of dissection and awareness of possible neurological
complications.

Important reduction of artifacts and thus decreasing pitfalls,

can be achieved by:

Contrast injection in RIGHT arm: no contrast crossing

Type A dissection extending into the brachiocephalic arteries.LEFT:
not visible on left arm injection.RIGHT: Same patient with right
arm injection.
in front of arch side branches which distorts visibility
of intima flaps (figure)
saline bolus chaser (only contrast in lumina of
interest)
EKG-triggered imaging (less motion artifacts
mimicking intima flap)
Also keep in mind that placing ROIs can be difficult
with dissected lumina (should be just distal to the
aortic arch).)
Have the technician be prepared to manually start
upon visual feedback.

Classification of Acute Aortic Syndrome

 Classic Aortic Dissection (AD), Intramural Hematoma (IMH)
and Penetrating Atherosclerotic Ulcer (PAU) are distinct
entities, but closely related.
This is reflected upon in their identical therapeutical
strategies.
The main goal for the radiologist is not only to detect which
entity is causing the clinical problem, but more importantly
to differentiate between type A and B!
Typical Aortic Dissection, Intramural Hematoma and Penetrating
Aortic Ulcer.

Stanford classification

The Acute Aortic Syndrome (AAS) is classified according to

Stanford.
Stanford Type A lesions involve the ascending aorta and
aortic arch and may or may not involve the descending
aorta.
Stanford Type B lesions involve the thoracic aorta distal to
the left subclavian artery.
The Stanford classification has replaced the DeBakey
classification (type I= ascending, arch and descending
aorta: type II= only ascending aorta: type III= only
descending aorta).

Treatment options for the 2 subgroups of the acute aortic

syndrome (AAS) are very different:
- Stanford type A will be treated with surgery or
endovascular therapy.
- Stanford Type B will be treated medically.

Aortic Dissection (AD)

 Classic Aortic Dissection is the most common entity causing
an acute aortic syndrome (70%).

Incidence: 1-10 : 100.000

mostly men
rarely
hypertension > 70%
Type A mortality 1-2% per hour after onset of
symptoms, total up to 90% non-treated, 40% when
treated.
1 year survival Type B up to 85% if medically treated
(5 year > 70%)

Classic Aortic Dissection

Management decisions are based on the following

information:

Type A or Type B
Place of entry & re-entry
Side branches involved, originating form true / false
lumen
Organs at risk (1/3 of mortality is caused by organ
failure)
Complications (rupture, coronairy occlusion, aortic
insufficiency, neurological )
Diameters of true and false lumina at: proximal and
distal landing zones, at entry and at minimum
Iliac vessel tortuosity
LEFT: Type A dissection with clear intimaflap seen within the aortic
arch.RIGHT: Type B dissection. Entry point distal to left subclavian
artery.
 Imaging features

In Aortic dissection an intima flap is seen in only 70% of

cases.
When there are 2 lumina, these will spiral around each
other (figure).
On the left consecutive images are seen of a Type B
dissection.
The true lumen is surrounded by calcifications.
The true lumen is smaller, as the false lumen wedges
around the true lumen due to permanent systolic pressure
(so called Beak-sign).
Thrombus material invariably is located in the false lumen,
which enhances later than the true lumen.

True lumen:

Surrounded by calcifications (if present)

Smaller than false lumen
Usually origin of celiac trunk, SMA and right renal
artery

False lumen:

Flow or occluded by thrombus (chronic).

Delayed enhancement
Wedges around true lumen (beak-sign)
Collageneous media-remnants (cobwebs)
Larger than true lumen
Circular configuration (persistent systolic pressure)
Outer curve of the arch
Usually origin of left renal artery
Surrounds true lumen in Type A dissection

Type B dissection. Green arrow indicates entry. False lumen is

indicated by yellow arrows and is seen spiraling around the true
lumen.
 On the left an aortic dissection is seen with a large false
lumen.
The compressed true lumen is seen on the inner side and is
brighter than the false lumen.
Thrombus formation within the false lumen.
The true lumen usually is smaller as the false lumen wedges
around the true lumen due to permanent systolic pressure.
The false lumen usually adheres to the outer curvature of
the aortic arch, as is seen in this case.

Type B Dissection

Collageneous media-remnants (cobwebs) are only seen in

the false lumen.
The same holds true for thrombusmaterial.

Cobweb seen within the false lumen

 If one of the lumina is surrounded by the other, it invariably
is the true lumen.
This almost only occurs in type A dissections.
The figures on the left both show a type A dissection with
clear entry points in the ascending aorta.
The true lumen is surrounded by the false lumen, which is
bigger and wedges around the true lumen due to permanent
systolic pressure.

Type A dissections. The true lumen is surrounded by the false

lumen.

Dissection into brachiocephalic arteries

Carefully sort out which branches of the aortic arch are

involved.
Make sure from which lumina they arise.

Aortic dissection involving brachiocephalic branches.


Left: Continued dissection into the celic trunk showing bigger false
lumen, significantly contributing to organ perfusion.Right: : SMA
and renal artery involvement, illustrating possible cause of organ
malperfusion
LEFT: Dissected SMARIGHT: No change at 20 months follow up
Dissection into abdominal arteries
The celiac trunc, SMA and right renal artery flow usually
originates from the true lumen.
Left renal artery flow mostly originates from the false
lumen.
Impaired perfusion of end-organs can be due to 2
mechanisms:
1) static = continuing dissection in the feeding artery
(usually treated by stenting)
2) dynamic = dissection flap hanging in front of ostium like
a curtain (usually treated with fenestration).
This may be hard to discern, MPR's can be helpfull.
Look for the re-entry point, usually to be found in the iliac
tract.
Provide information about tortuisity and calcifications of the
iliac tract if endovascular procedures are being considered.
When no end-organs are compromised and there is
sufficient perfusion, dissection can be left alone.
This may persist for a long time without clinical
consequence, as is seen in the patient on the left with
follow-up of 2 years.
Some dissections remained unchanged during a follow up of
more than 5 years.
 Rupture into pericardium and thoracic cavity

Even the slightest amount of fluid in pericardium,

mediastinum or pleural cavity is suggestive of rupture of the
dissection.
The cases on the left show evident rupture, with presence of
extensive hematoma in above mentioned locations.
Note extreme hematothorax and hematomediastinum,
causing shift of the mediastinum and compression on the
pulmonary veins and even aorta.
No pericardial effusion visible.

Left: pericardial fluid / hematoma indicates rupture of the

dissected aorta. Even small amounts are proving rupture, though
hematoma can be extensive such as in this case.Right: Massive
hematoma caused by rupture of the dissected aorta into the
mediastinum and pleural cavity, no pericaldial hematoma.

The case on the left is a patient who presented with a fully

thrombosed false lumen.
5 days after initial presentation this patient complained of
acute chest pain mimicking the earlier episode.
Re-examination showed recurrence of flow in the false
lumen, locally contained, but with alarming adhering pleural
effusion.
The patient could not undergo surgical or endovascular
repair for various reasons and was treated consevatively.

Type B aortic dissection in a non-operable patient. At 5 days flow

reappeared in false lumen. Finally at 9 months a saccular
aneurysm has formed.
 Aneurysm with thrombus versus thrombosed
dissection

It can be difficult to differentiate an aneurysm with

thrombus from a dissection with a thrombosed false lumen.
If there are intima calcifications this will be very helpfull.
A false lumen displaces the intimal calcifications.

LEFT: Dissection with a thrombosed false lumen. RIGHT:

Aneurysm with thrombus on the inner side of the intimal
calcifications.

Intramural Hematoma

Brief facts:

Spontaneous hemorrhage caused by rupture of vasa

vasorum in media
13% of dissections, usually no pulse deficit
Difficult to distinguish from thrombosed AD
Can proceed to classic dissection (16-47%)
Long time to diagnosis: usually overlooked due to lack
of non-enhanced scan
Mortality at 1 year after dismission ~ 25%

Intramural Hematoma is a result of ruptured vasa vasorum

 What the clinician needs to know

Type A or Type B
Regression of aortic ? to normal in 80% of patients
Predictors of mortality:
- Ascending Aorta > 5 cm ?
- IMH thickness > 2 cm
- Pericardial effusion (to less extend pleural effusion)
IMH may persist or evolve into aneurysm or PAU
Associated PAU - worse prognostic outcome

On the left a Intramural hematoma, hyperdense on a NECT.

Classic example of IMH. Hyperdense hematoma on NECT. Intima

calcifications surround the true lumen.

Same case contrast enhanced CT.

Note that the IMH does not spiral around the true lumen,
like in classic AD, helping to differentiate both.
Essentially, this is not important, therapeutical decision will
be made by whether this IMH is classified as Type A or Type
B IMH!
Note that there is no pericardial effusion.
IMH thickness stays below 2 cm, making regression of this
Type B IMH likely (up to 80%).
Same case. CECT of Intramural hematoma type B.

Penetrating Atherosclerotic Ulcer

PAU is defined as an ulceration of an atheromatous plaque

that has eroded the inner elastic layer of the aortic wall.
It has reached the media and produced a hematoma within
the media.

Brief facts:

Patients with severe systemic atherosclerosis

 Rarely rupture, yet worse prognosis due to extensive
atherosclerosis which causes organfailure (e.g. acute
myocardial infarction)
Cause of most saccular aneurysms
Located in arch and descending aorta
Often multiple (therefore surgical treatment difficult,
mostly treated medically)

What the clinician needs to know

Type A or Type B
Single or multiple
Associated IMH (if not present, be cautious to mention
PAU, clinical symptoms might not be caused by PAU,
which is probably stable)
Possibility of endovascular treatment

Typical illustration of PAU, focal outpouchings of contrast,

separating extensive intimal calcifications

Imaging features

Extensive atherosclerosis with severe intima

calcifications and atherosclerotic plaques
Focally displaced and separated intimacalcifications
Crater and/or contrast extravasation
-Focal IMH, longitudinal spread limited by
mediafibrosis
Possibly enhancing aortic wall

Complications
1. CT in Nontraumatic Acute Thoracic Aortic Disease: Typical and
Atypical Features and Complications.
E. Casta?er et al, Radiographics 2003; 23:S93- S110 The complications of a Penetrating Atherosclerotic Ulcer
include:
2. T.T. Tsai et al. Acute Aortic Syndromes. Circulation
2005;112;3802-3813 Saccular aneurysm formation
 3. D. Mukherjee et al. Aortic Dissection. An Update. Current Compression of nearby structures
Problems in Cardiology 2005;30:287-325 Rupture
4. H. Hayashi et al. Penetrating Atherosclerotic Ulcer of the Aorta:
Imaging Features and Disease Concept. Radiographics However most patients have a poor prognosis because of
2000;20:995-1005 generalized atherosclerosis leading to diffuse organ failure.
5. K.R. Cho et al. Penetrating atherosclerotic ulcer of the
descending thoracic aorta and arch. J Thorac Cardiovasc Surg 2004;127:1393-401
6. F.Ganaha et al. Prognosis of Aortic Intramural Hematoma With and Without Penetrating Atherosclerotic Ulcer. A Clinical and
Radiological Analysis. Circulation. 2002;106:342-348.
7. Y. von Kodolitsch et al. Intramural Hematoma of the Aorta Predictors of Progression to Dissection and Rupture. Circulation.
2003;107:1158-1163
8. S. Willoteaux et al. Imaging of aortic dissection by helical CT. Eur Radiol, 2004;14:1999?2008
9. J-K Song. Diagnosis of aortic intramural haematoma Heart 2004;90:368-371.
10. A. Evangelista et al. Acute Intramural Hematoma of the Aorta. A Mystery in evolution. Circulation 2005;111:1063-1070
11. N. Mangat et al. Multi-detector row computed tomography: Imaging in Acute Aortic Syndrome Clin Rad 2005;60:1256-1267