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Don’t Miss This Diagnosis!
Robert L. Rogers, MD, FAAEM
Combined Emergency Medicine/Internal
Department of Surgery/Division of
Emergency Medicine and Department of Internal Medicine
University of Maryland School of
At the conclusion of this presentation, each
participant should be able to…
1. Describe common and less well-known risk factors
for aortic dissection
2. Recognize atypical and subtle presentations of
3. List reasons for the missed or delayed diagnosis
and describe a useful approach to
decrease the chance of successful litigation
4. Describe physical examination and radiographic
pitfalls in the diagnosis of
5. Cite new developments and current literature
pertaining to aortic dissection
Why is aortic dissection an important topic for
emergency physicians to know well?
Thoracic aortic dissection can be extremely difficult
Mortality rates are estimated at 50% by 48 hours if
Mortality rates increase by 1%/hour if undiagnosed.
Prompt detection and therapy impact on survival rates.
Time is aorta.
The detection of thoracic aortic dissection (TAD)
may be greatly enhanced by performance of a thorough risk factor
Every chest pain patient should undergo a risk
factor analysis of the top three deadly chest pain entities: acute
coronary syndromes, pulmonary embolism, and thoracic aortic dissection.
Traditional Risk Factors for TAD:
Hypertension (only ~ 50% are hypertensive on
Age (tends to occur in older patients, but don’t
forget that young patients can develop TAD as well.)
Pregnancy (also a risk factor for coronary artery
Family history (not connective-tissue disease
Connective tissue disease (Marfans and Ehler-Danlos)
Less Common Risk Factors:
Cocaine (Type B more common)
Bicuspid aortic valve
Iatrogenic (cardiac catheterization)
Coarctation of the aorta
Ecstasy (NMDA) use and weight lifters-associated TAD
“There is no disease more conducive to
clinical humility than aneurysm of the aorta.”
“The tragedies of life are largely
Sir William Osler1
The key to making the diagnosis of this lethal
aortic disease may depend on how familiar you are with well-described
atypical and subtle presentations? Although textbook presentations
may occasionally occur, they tend to be the exception rather than the
rule. Atypical really is typical.
International Registry of Aortic Dissection (IRAD)-Study
by Hagan, et al. that involved 464 patients with confirmed TAD. Mean
age: 63 years, 65.3 % males, 62% type A dissections
pulse deficit 15 %
aortic murmur 31.6 %
normal chest x-ray 12 %
absence of mediastinal
widening 34 %
syncope 12 %
Conclusions from this
(1) Classic findings of
aortic dissection are often absent
(2) Don’t rely on
KNOW THE FOLLOWING PRESENTATIONS WELL:
(1) TAD and Stroke-Neurologic
deficits have been noted to occur in approximately 18-30% of TAD cases.
In 5-10% of the cases, TIA/CVA symptoms are seen. Remember that patients
who present with stroke and the inability to communicate may not be able
to give a chest pain history. If you are about to infuse tPA for a
suspected ischemic CVA, stop for a moment and convince yourself that the
patient doesn’t have an aortic dissection.
As a rule of thumb, consider TAD in the following
Chest pain and any neurologic symptoms (CVA, dysphagia,
Chest pain and limb paresthesia
(2) Painless TAD and Syncope-Patients
with TAD do not always present with chest pain. In the IRAD study, 2.2%
of TAD cases were painless. Other studies have shown that as many as 15%
of TAD cases are painless. The point is this: Absence of pain does not
rule out the diagnosis. Patients may also present after a syncopal
episode. The underlying pathophysiology of syncope is related to
proximal rupture into the pericardium with resultant tamponade. Add
TAD to your differential diagnosis of unexplained syncope.
(3) TAD and Paralysis-Spinal cord
involvement has been documented in 10% of TAD cases and is thought to be
due to perfusion abnormalities in the greater radicular artery (artery
of Adamkiewicz), a large branch from the aorta that perfuses a large
portion of the thoracic and lumbar spine.
Consider TAD in the following scenarios:
Chest pain and limb (particularly lower extremity)
weakness or paresthesia
Chest pain and spinal cord syndromes: transverse myelitis,
progressive myelopathy, paraplegia, quadriplegia, and anterior spinal
Unexplained lower extremity weakness (without chest pain)
(4) TAD and Myocardial Infarction-The
coexistence of TAD and myocardial infarction occurs in about 1-7% of
cases of TAD. Due to greater shear forces against the right lateral
aortic wall, right coronary ostial occlusion and malperfusion is more
(5) Isolated Abdominal Pain-Many
case reports have been published over the last several years documenting
the fact that TAD (proximal or distal) may present with isolated
abdominal pain. Given the frequency of abdominal pain ED visits and the
extensive differential diagnosis, picking up TAD-associated abdominal
pain may be difficult. In general, have a very low threshold to obtain a
CT scan to evaluate for TAD.
Consider TAD in the following scenarios:
Unexplained abdominal pain in the presence of hypertension
Combination of chest and abdominal pain
Abdominal pain and cocaine use
Unexplained abdominal pain and an “ill-appearing” patient
(6) TAD and “the other complaint”-Many
cases of missed TAD have been shown to be related to the failure to
address two or more seemingly separate complaints.
Here are a few examples of real cases from my
A 37 year-old male presented with a complaint of chest
pain and left lower leg pain and numbness. His only medical problem was
hypertension. His BP on presentation was 180/100. The physician who saw
this patient essentially dismissed the leg complaint and thus missed a
valuable opportunity to make the diagnosis of TAD. The patient had a
type- B aortic dissection and subsequently died.
A 49 year-old male presented with a complaint of
substernal chest tightness and transient decreased vision in his left
eye. The treating physician completely ignored the visual complaint and
addressed the chest pain. Chest pain and blindness, or transient visual
disturbance, is actually described in the literature as a presentation
of TAD. Once again…chest pain in combination with “the other complaint.”
A 29 year-old female 8 months pregnant presented with
blurry vision and shortness of breath. The patient ended up dying of a
ruptured, proximal aortic dissection. Pregnancy is a risk factor for
TAD, and every pregnant patient with chest, back, or chest and back pain
may very well have a TAD. Authorities on this subject would argue that a
diagnosis of TAD simply could not be made in a case such as this one. It
is mentioned only to highlight the risk factor and mention that TAD is
more common in the 3rd trimester.
Consider TAD under the following circumstance:
Chest pain combined with “the other complaint” (especially
(7) Cough, Hoarseness, and SVC Syndrome-Thoracic
aortic dissection may present with symptoms that are subtle, vague, or
not easily attributable to aortic disease. Cough has been described and
is thought to be secondary to compression of the left main-stem bronchus
by the aortic hematoma/dissection. Proximal aortic dissection may also
cause recurrent laryngeal compression and lead to hoarseness (Ortner’s
syndrome). Lastly, proximal dissections may rarely compress the superior
vena cava and lead to clinical signs/symptoms of SVC syndrome.
(8)Young patients with TAD-Most
patients with TAD are between the ages of 50-70. However, emergency
physicians are seeing more young patients (even without connective
tissue disease) with the disease. A key mistake is to not consider the
diagnosis simply because the patient is young.
Young patients can have acute aortic diseases such as
Consider the case of Jonathan Larson, writer of the
Broadway musical Rent.
Jonathan Larson died at the age of 35 from a type A
thoracic aortic dissection. He was seen three separate times in an
emergency department in New York before he was found dead in his
apartment by his roommate. Mr. Larson presented initially with a
complaint of severe chest pain and was diagnosed with food poisoning,
despite no symptoms of nausea, vomiting, or diarrhea. What is
interesting is that his chest x-ray showed a significantly widened
mediastinum. He later presented with chest and back pain. He was quoted
as saying, “You’d better call 911. I think I am having a heart attack.”
Mr. Larson was later diagnosed as having Marfans disease.
Why was the diagnosis of
TAD not entertained? Probably the most important reason was the fact
that Jonathan Larson was only 35 years old and had no known medical
Why do we miss the diagnosis?
Daniel Sullivan in his work, “High-Risk Acute Care:
The Failure to Diagnose…”, has outlined several reasons for the delay or
missed diagnosis of TAD. Having an understanding of these reasons may
help us do a better job at detecting this disease early.
Here are the reasons cited by Sullivan for a delay
or missed diagnosis:
The “legal fiction”-This refers to cases of TAD
that simply cannot be made. Like other diagnoses in medicine, not all
cases of TAD can be diagnosed.
Failure to evaluate risk factors for TAD.
Failure to integrate patient complaints-For
emergency physicians, this may very well be one of the most important.
All of us are familiar with patients who present with seemingly
unrelated complaints, such as chest pain and leg pain. A review of
missed TAD literature highlights the fact that many cases have been
missed because the treating physician failed to address all of the
patient’s complaints. Also, the EP may assign a diagnosis that simply
doesn’t make sense.
The “obvious miss” and inadequate knowledge base.
Inadequate knowledge of atypical and subtle
presentations-Examples include unexplained syncope, chest pain and
lower extremity weakness, painless TAD, chest pain and stroke-like
How can you decrease your chances of missing
In reality, there simply is no way that every case
of aortic dissection can be diagnosed in the emergency department. There
are, however, steps that can be taken to decrease the chance of missing
this lethal aortic disease.
Know the subtle and atypical presentations well. Think
beyond classic textbook descriptions and think of TAD more often.
Perform a detailed risk factor profile for TAD on every
chest pain patient. Detection of one risk factor may be all you need to
pick up the diagnosis.
Decrease your own threshold to obtain CT scans in chest
pain patients. Increasingly, isolated anterior chest pain has been
associated with TAD. If a patient isn’t responding to ACS therapy or
something doesn’t seem right with the patient, obtain a CT scan.
Approach every chest pain patient as if they could have a
TAD and convince yourself the patient doesn’t have it.
Realize that young patients without connective tissue
disease can have TAD.
What should we be documenting on chest pain
In order to decrease the chance of successful
litigation for a missed or delayed diagnosis, emergency physicians
should consider documenting certain key features of the history and
physical examination. In essence, a chest pain chart should look like
the search for lethal chest pain entities, acute coronary syndromes,
pulmonary embolism, and thoracic aortic dissection.
Consider documenting the following in all chest
Risk factor profile for TAD (HTN, cocaine, family history,
Blood pressure in both arms (equal)
Absence of aortic murmur
Absence of marfanoid body habitus
Limitations of the physical examination and
Physical examination and imaging studies are
important in any patient with chest pain but both have significant
What about the physical examination?
Bilateral blood pressure measurement-Traditionally,
physicians have relied on differences in bilateral arm blood pressure to
help detect TAD. Recent data over the last few years, however, has shown
that as many as 19% of the population may have arm differences greater
than 20 mm Hg. One high quality prospective, observational study by Von
Kodolitsch et al. did show that a blood pressure differential > 20 mm Hg
was an independent predictor of TAD. Measurement of bilateral blood
pressures may very well be an important risk management strategy but
normal or abnormal values do not rule in or out the diagnosis. The other
important thing to mention is that only about 50% of patients who
present with TAD are hypertensive. The other half are either
normotensive or hypotensive.
Aortic murmur-The presence or absence of a
diastolic murmur is not particularly helpful. Approximately 1/3 of
patients with TAD will have this finding.
Pulse deficit-Noted to be present in only about 15%
of cases of TAD.
What about the chest x-ray?
According to data from the IRAD study, up to 12 % of
patients with TAD had a normal chest x-ray (completely normal).
Approximately 37 % had no evidence of mediastinal widening.
Numerous other studies have found that the chest x-ray may
A normal chest x-ray might help lower your suspicion for
What’s new in the literature?
A number of interesting developments have occurred
over the past few years.
D-dimer-Small studies have suggested that all cases
of TAD were found to have an elevated D-dimer result. Could D-dimer in
combination with another study, such as a chest x-ray, be used to rule
out TAD? This clearly isn’t ready for prime time, but there are
researchers looking into this as a potential strategy.
Soluble elastin fragments-These fragments are
released into the circulation in TAD and have been shown in small
studies to be helpful in diagnosing TAD. Again, we are not ready to
begin testing for this quite yet, but be on the look out for this in the
Blood pressure-Recent studies have shown that
normal patients without aortic dissection may have significant
differences in bilateral blood pressure values. On the other hand, many
patients with TAD have differences < 20 mm Hg. Use caution when using
these values to make clinical decisions.
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Any questions or comments?
Please e-mail me: firstname.lastname@example.org
Robert Rogers, M.D.
Emergency Medicine and Combined EM/IM Residency
Department of Surgery Division of Emergency
Department of Medicine
The University of Maryland School of Medicine
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