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22 Nienaber and Ince
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24 Nienaber and Ince
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Classification of Aortic Dissection 25
DISCUSSION AND COMMENTARY
Question for the Authors
In discussing classification by acuity of presentation, Dr. Nienaber mentions that
some dissections are discovered incidentally at the time of imaging for other
purposes. Does Dr. Nienaber have any hints for surmising the age of these dissec-
tions? How can we be sure that they are not acute? This question has medico-
legal implications as well—for fear that one of these lesions, interpreted as
incidental and non-acute—may rupture soon after detection.
It can indeed be difficult to determine whether an incidentally discovered dissec-
tion is acute or chronic. The absence of acute symptoms argues for chronicity.
At times, the dissection flap appears thick and fibrotic, again arguing for chronic-
ity. If in doubt, we admit the patient for 48 hours of observation, on the off-chance
that an acute aortic dissection may have been “caught in the making” by the
incidental radiographic imaging study.
Editor’s Counterpoint
Utility of Stanford Classification
The Editor differs respectfully with the chapter authors (whom he respects
immensely) with regards to the utility of the Stanford classification of aortic dis-
section (type A and type B). The editor has found this simple classification extremely
useful clinically. This classification is simple, easily understood, easily communi-
cated, and very meaningful in terms of clinical implications: type A—surgery,
type B—“anti-impulse” therapy. Yes, there are nuances that are not fully accom-
modated by this simple schema, but they are uncommon and not of overwhelming
clinical significance. The alternate, more complex classification schemas are
useful for extreme experts, like the chapter author, and for research, but perhaps a
bit too cumbersome for every day applications in the emergency department, the
diagnostic imaging suite, and the clinical wards.
Categorization of Dissections with Arch Tear Propagating Distally
The author is pleased to learn that Dr. Nienaber, in his immense wisdom and expe-
rience, groups dissections starting in the aortic arch (around and between the head
vessels) with the Type B category. This has been the Editor’s policy at his own
institution. We prefer to treat these dissections non-operatively, and they do well.
We remain alert for proximal propagation, which would mandate surgery, but this
does not eventuate frequently.
Dissection Variants—Penetrating Aortic Ulcer and
Intramural Hematoma
We use a very simple schema for distinguishing between typical dissection, on the
one hand, and the variant entities penetrating aortic ulcer (PAU) and intramural
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26 Nienaber and Ince
hematoma (IMH). This is our schema: “No flap, no dissection.” Please see
Figure 1 in Chapter 12, which makes this point schematically.
We believe that PAU and IMH are not the same as typical dissection, and the
simple distinguishing criterion encompassed in that one phrase has proved useful
for us from an educational and communication standpoint.
We have a somewhat different approach to descending PAU and IMH than
some centers, certainly vis-à-vis centers in Japan. We believe these are serious lesions
that behave malignantly. Yes, we understand that descending PAU and IMH can be
controlled medically and that patients can usually leave the hospital alive. However,
we operate on these lesions at about three to four weeks, if the patient is not too
old or frail. [PAU, especially, is a disease of old age (Fig. A)]. We changed our
management—from non-operative to operative—when we accomplished medium-
term follow-up on these patients (1). We found that (1) Many of the PAU/IMH
patients died in only mid-term follow-up, of causes compatible with an aortic etiology,
and (2) Many of the IMH and PAU went on to manifest as typical dissections. We feel
that a non-operative policy permits fairly frequent death of these patients (1,2).
Figure A Note advanced age of IMH and, especially, PAU patients, compared to those
with typical ascending (1) or descending (2) dissection. Abbreviations: IMH, intramural
hematoma; PAU, penetrating aortic ulcer.
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Classification of Aortic Dissection 27
Figure B (See color insert) Note multiple penetrating aortic ulcers, although only one
was suspected radiographically.
Furthermore, we find at surgery that the PAU patients, despite radiologic
detection of a single penetrating ulcer, often harbor many such ulcers over a wide
segment of aorta. In the operating room, these appear “ready to burst” (Fig. B).
We feel this factor also argues in favor of an aggressive surgical approach.
For ascending IMH and PAU, we have an aggressive posture, like most
centers. We operate on these lesions acutely, like for type A dissection.
Figure C (See color insert) Note from schematic depictions that typical dissection (1)
often impairs branch vessel flow, but this never happens with IMH and PAU (2).
Abbreviations: IMH, intramural hematoma; PAU, penetrating aortic ulcer.
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28 Nienaber and Ince
One good feature regarding IMH and PAU, on which Dr. Nienaber touches
briefly, which we wish to emphasize, is the following: IMH and PAU, in sharp
contradistinction to typical aortic dissection, never produce branch vessel
occlusion (Fig. C). We have not encountered a single violation of this principle;
colleagues from around the world have similar impressions.
References
1. Coady MA, Rizzo JA, Elefteriades JA. Pathologic variants of thoracic aortic dissections:
Penetrating atherosclerotic ulcers and intramural hematomas. Cardiol Clin No Amer
1999; 17:637−657.
2. Tittle SL, Lynch RJ, Cole PE, Singh H, Rizzo JA, Kopf GS, Elefteriades JA. Midterm
follow-up of penetrating ulcer and intramural hematoma of the aorta. J Thorac
Cardiovasc Surg 2002; 123:1051−1059.
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29
2
The Symptoms and Signs of Acute
Aortic Dissection: Clinical Diagnosis
of “The Great Masquerader”
Eric M. Isselbacher
Department of Medicine, Harvard Medical School and
Thoracic Aortic Center, Massachusetts General Hospital,
Boston, Massachusetts, U.S.A.
INTRODUCTION
There is a “classic” constellation of symptoms and signs of acute aortic dissection
that is commonly taught in medical school. One classic example would be a
72 year-old man with a history of longstanding hypertension, who presents with
the acute onset of severe stabbing, interscapular back pain; on arrival he is diapho-
retic and restless, with a blood pressure of 195/110 mmHg. A second example
would be a 35-year-old woman, who is tall and thin, presenting with the acute
onset of severe tearing chest pain that radiates to her neck and then moves to her
back as well; her blood pressure is 20 mmHg lower in the right arm than in the
left, and on physical examination she has a loud decrescendo diastolic murmur
at the left sternal border. Offered these clinical vignettes, most medical students
would readily propose aortic dissection as the most likely diagnosis.
Indeed, if all of the patients with aortic dissection presented in such a
“classic” fashion, aortic dissection could be easily distinguished from other acute
syndromes and thus be more promptly diagnosed. Unfortunately, in reality, only
a minority of patients with aortic dissection actually present with fully classic
symptoms and signs; rather, the majority present with less typical symptoms and
often few, if any, of the classic signs. Moreover, the mix of symptoms and signs
are often quite varied, so that even experienced clinicians find diagnosing aortic
dissection consistently challenging.
SECTION II: IMAGING AND DIAGNOSIS
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30 Isselbacher
SYMPTOMS
Pain
Experience of Pain
One feature of aortic dissection present in almost all cases is pain. In the
International Registry of Acute Aortic Dissection (IRAD), which initially reported
on a series of 464 consecutive patients with acute aortic dissection from 12 inter-
national referral centers, 96% of those with dissection presented with pain
(Table 1) (1). Three-quarters of patients will experience pain in the chest, either
anterior, interscapular, or both, and sometimes the pain will extend to the neck as
well. Half will experience back pain, and a third will experience abdominal pain.
Sometimes patients will experience leg pain as well, although this symptom is
sometimes transient.
Table 1 Symptoms of Aortic Dissection
Symptom Comment
Experience of pain Almost ubiquitous—96% of patients report pain
Location of pain Anterior characterizes ascending dissection;
posterior characterizes descending dissection
Severity of pain “10 out of 10”
Onset of pain Abrupt (distinguishes from MI)
Quality of pain “Tearing” quality
Variability of pain Classical patterns common, but not invariable;
4% of patients experience no pain
Waxing and waning Variability in severity of pain reflects physiological
of pain (decrease in aortic wall tension with rx of BP) and
anatomic events (spontaneous re-entry).
Pleuritic component Reflects pericardial and pleural inflammation
Cardiac ischemia True anginal/infarction symptoms may occur from
involvement of RCA
Abdominal pain Vigilant search for intestinal ischemia essential
(lethal phenomenon)
Renal ischemia Usually asymptomatic, except in case of infarction
Leg pain From involvement of iliac artery by dissection
process
Paralysis of legs From spinal cord ischemia or peripheral nerve ischemia
(paraplegia)
Syncope From either:
Involvement of head vessels
Tamponade
Acute aortic insufficiency
Vaso-vagal response to pain
Dyspnea Acute aortic insufficiency poorly tolerated
Abbreviations: MI, myocardial infarction; RCA, right coronary artery; BP, blood pressure.
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Symptoms and Signs of Acute Aortic Dissection 31
Location of Pain
Not surprisingly, the location of the pain often corresponds to the underlying
segment of the dissected aorta; anterior chest or neck pain tends to be associated
with dissection of the ascending thoracic aorta, whereas interscapular, lower
back pain, or abdominal pain are typical of dissection of the descending thoracic
aorta (2). The pain sometimes radiates or frankly migrates from one location
to another, such as from the front of the chest to between the shoulder blades, or
from the back to the abdomen.
Severity of Pain
The pain is usually severe and often the worst pain a patient has ever experienced;
not uncommonly, the pain is rated as a 10 on a scale of 1 to 10. Indeed, the pain
may be so severe and unrelenting that patients appear restless or writhe around the
examination room or on the stretcher, and sometimes even drop to their knees
when standing. This is in contrast to the chest pain associated with angina or acute
myocardial infarction, which is rarely rated 10 out of 10.
Abrupt Onset of Pain
In addition, the pain of aortic dissection is typically abrupt in onset and described as
at its most severe, right at the outset. In fact, given the abruptness of the onset,
patients will often recount exactly what they were doing the moment the pain began.
This abrupt pattern distinguishes the pain of aortic dissection from the pain of myo-
cardial infarction or angina, which is typically mild at the outset and crescendos.
Tearing Quality of Pain
Classically, the quality of aortic dissection pain is described as “tearing” or
“ripping.” Indeed, according to the IRAD study, 51% of patients confirmed that
these adjectives were fitting descriptors of their pain (1). However, whereas many
will confirm these descriptors if asked, in reality, patients do not often offer these
particular adjectives spontaneously. Instead, they are more likely to describe the
pain as “sharp” or “stabbing,” which are the adjectives used by 64% of patients in
the IRAD population.
Variability of Pain
Once again, the qualities of the pain described in the previous section are typical,
and not all patients with aortic dissection experience symptoms in the typical
fashion. For example, in 15% of those with aortic dissection the pain is gradual in
onset rather than abrupt, and in 10% the pain is not severe. A minority will describe
the pain as tightness or pressure, which can be easily mistaken for anginal pain.
Finally, 4% will present without any symptoms of pain at all.
Waxing and Waning of Pain
Most physicians assume that the pain of aortic dissection must be constant and
unrelenting, and in some cases it is. However, in other instances the pain may wax
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