Severe Acute Chest Pain in an Adolescent
Radiology Cases in Pediatric Emergency Medicine
Volume 3, Case 12
Andrew K. Feng, M.D.
Kapiolani Medical Center For Women And Children
University of Hawaii John A. Burns School of Medicine
A 14 year-old boy is brought into the Emergency
Department just after midnight after having woken from
sleep approximately ten minutes ago because of severe
back pain and abdominal pain radiating into his throat.
The pain is also described as "pressure" pain. No
history of trauma or similar attacks of pain in the past.
No other concurrent illnesses or symptoms.
Exam: VS T35.8 C, P86, R32, BP 110/74, O2
saturation 100% on room air. Weight approximately 70
kg. He is obviously writhing in intense pain. Heart
regular without murmurs. Lungs sounds are clear on
auscultation with equal breath sounds bilaterally.
Peripheral pulses are normal and equal bilaterally.
Skin is warm and dry. Abdominal exam is also
unremarkable with good bowel sounds, and no palpable
masses or costovertebral tenderness.
What would you do next?
At this point, the father settles down enough to be
able to give you more of a coherent history. He relates
to you that he had another son who had died at about
12 years of age from an aortic dissection and
subsequent rupture. A chest radiograph is obtained.
The CXR is read as being relatively normal although
there may be a suggestion of some mediastinal
widening. A CT of the chest and abdomen is ordered.
The CT of the thorax shows an aortic dissection
extending from the aortic root to at least the level of the
renal arteries. In the upper image taken through the
aortic arch, an intimal flap is visible in the aortic arch
(black arrow). In the lower image taken through the
abdomen, the intimal flap is more obvious in the
descending aorta (black arrow). There also appears to
be an infarcted area in the left kidney. An aortogram is
This contrast study shows the catheter tip at the
aortic root. The aortic root is irregular. Since contrast
does not enter the carotid vessels, the catheter is
presumed to be in the false lumen of the aortic
dissection which is dilated at the aortic root. An imprint
of the brachiocephalic artery (a non-contrast filled
vessel impinging on the contrast filled false aortic
lumen) is seen overlying the aorta. Subsequent views
revealed a complex flap of the dissection in the
Aortic dissection is rare, but catastrophic when it
occurs. Therefore, a high index of suspicion and
appropriate early intervention are paramount if the
patient is to survive. The majority of non-traumatic
causes of aortic dissection occurs in the older
population, and hypertension is the overwhelming
leading risk factor. Those who develop aortic
dissections under 40 years of age more commonly have
an underlying connective tissue disorder, congenital
heart defect, or are pregnant. For the purposes of this
teaching case, most of the discussion will focus on the
Classification of aortic dissection is either by the
DeBakey or the Stanford types. The former was
classified first and divides the types into those involving
the ascending, transverse, and/or descending aorta
(Type I), those involving the ascending aorta only (Type
II), and those involving the descending aorta only (Type
III). The latter classification distinguishes dissections
mainly by the involvement of the ascending aorta: any
dissection involving the ascending limb is Type A, and
those that involve just the descending aorta are Type B.
Approximately 75% of the cases are of Type A, which
also is more commonly associated with the younger
patients and those with connective tissue and
congenital heart disorders.
Pathophysiology centers mainly on the integrity of
the vessel wall. Most cases show a weakening in the
media including lesions involving smooth muscle,
elastin and collagen. In Marfan's Syndrome, medial
cysts with mucopolysaccharide deposits, disarray of
muscle fibers, and fragmentation and loss of elastin
fibers can be seen histologically in the face of
dissection. Furthermore, the aortic root is made up of
approximately 60% elastin and is the most common
point of dilatation and dissection in these patients. In
one study, up to 60% of infants and children under 4
years old were found to have a certain degree of aortic
dilatation (Sisk). Other familial conditions without
Marfanoid features but presenting with aortic dissection
include anuloaortic ectasia, familial dissecting
aneurysm, Ehlers-Danlos Syndrome, and osteogenesis
imperfecta. In addition, history of hypertension or a
specific triggering event is often absent for this
Patients may present with a wide range of signs and
symptoms. One of the most common (90%) as well as
the most impressive complaints is excruciating pain.
This is often described as being "cutting," "ripping," or
"tearing," and originating in the anterior chest, although
less commonly in the interscapular, epigastric, and
lumbar areas. Back pain also tends to correlate more
with distal dissections. Flank pain may suggest
involvement of the renal arteries. In addition, the pain
will usually begin suddenly and will persist unremittingly,
often inciting the fear of death in the patient.
Cardiac involvement depends on the site of
dissection and the location of the external rupture. One
of the distinguishing features of aortic dissection is an
elevated blood pressure, which may seem contradictory
to what one would expect. This is, in part, due to renal
ischemia and severe stress. Hypotension, on the other
hand, occurs in approximately 20% of ascending
dissections and is an ominous sign as it suggests a
significant external rupture. In fact, rupture into the
pericardial sac is, unfortunately, relatively common and
can result in pericardial tamponade. Syncope may be a
presenting symptom or sign and almost always reflects
leaking into the pericardium. Because of this, syncope
is usually an ominous sign and, therefore, warrants
prompt surgical intervention. Aortic dissection also
often leads to heart failure secondary to aortic
incompetence. This may be exhibited by aortic
regurgitation and can be appreciated as a new diastolic
Peripheral pulses may also be affected, depending
on the extent of the dissection. Pulses in one or more
extremities may be diminished or even absent. It is
important, therefore, to evaluate the quality of pulses
(and, possibly, the blood pressure) in all four
Neurologic changes are actually very common
occurring approximately 40% of the time. Dissections
involving the carotid arteries may result in
encephalopathy or even stroke. Dissections extending
through branches supplying the spinal cord may also
lead to paraplegia. Aortic dilatation or hematoma within
a dissection may also compress the recurrent laryngeal
nerve resulting in Horner's Syndrome and/or
The most critical preliminary diagnostic test for
suspected aortic dissection is a chest radiograph.
Aortic shadows are seen in approximately 80-90% of
the time. Other initial tests may include a hemoglobin
and hematocrit, with a low value suggesting an external
rupture; a urinalysis, with hematuria reflecting renal
ischemia; and an electrocardiogram to detect
Once a dissection is suspected, it may be confirmed
by one of the following tests. Transesophageal
echocardiography is gaining popularity as a quick and
sensitive test that can be performed at the patient's
bedside. One study shows a sensitivity of 99% and a
specificity of 98% with results of the test provided within
20 minutes (2). An echocardiogram will also provide
information on myocardial function, valvular
insufficiency, and any pericardial effusion.
Computerized tomography is also gaining favor as it is
highly sensitive and specific and readily accessible in
most centers. The main disadvantage concerns the
time necessary to perform these tests. However,
ultrafast CT has improved the time factor as well as
heightened the accuracy. However, the traditional gold
standard, has been retrograde aortography, which
involves the use of contrast to determine the full extent
of the dissection as well as involvement in arterial
branches. Magnetic resonance imaging also is an
option, but the length of time necessary to perform the
test and the accessibility are the main limiting factors.
The mainstay of medical treatment revolves around
controlling blood pressure (systolic and pulse pressure).
Antihypertensives should be instituted immediately if
the blood pressure is high, or as soon as possible once
the pressure is stable and the diagnosis is confirmed.
The combination of sodium nitroprusside and
propranolol is most commonly used. Labetolol can also
be used as monotherapy or in place of propranolol.
Surgical intervention should be started emergently in
aortic dissection if pericardial tamponade is suspected,
if there are any signs of shock, or if the dissection is
Outcome remains relatively poor, but is improving
with a current mortality rate of 5-30%. Death is mostly
due to hemorrhage and heart failure with one study
finding 93% of deaths secondary to cardiovascular
complications (5). For patients with Marfan's Syndrome
and other connective tissue disorders and congenital
heart diseases, prevention with routine cardiac
evaluations as well as elective, prophylactic surgical
intervention (when indicated) are currently the best
means of minimizing poor outcome.
In summary, aortic dissection and rupture is very
rare, but must be considered in patients presenting with
acute, intense chest pain. A good history and physical
exam should lead the examiner to suspect this
diagnosis, and successful outcome will be determined
by timely intervention.
1. Wernly JA. Thoracic Aortic Dissection. In:
Crawford MH (ed). Current Diagnosis and Treatment in
Cardiology, 1st Ed., 1995, Norwalk, Appleton & Lange,
2. Erbel R et al. Detection of Aortic Dissection by
Transesophageal Echocardiogram. Br Heart J
3. Fuster V, Ip JH. Medical Aspects of Acute Aortic
Dissection. Seminars in Thoracic and Cardiovascular
4. Roman MJ, Devereux RB. Heritable Aortic
Diseasea. In Lindsay J (ed). Diseases of the Aorta.
1994, Philadelphia, Lea & Febiger, pp. 55-74, 127-143.
5. Murdoch JL et al. Life Expectancy and Causes
of Death in the Marfan's Syndrome. N Engl J Med
6. Sisk HE et al. The Marfan Syndrome in Early
Childhood: Analysis of 15 Patients Diagnosed at less
than 4 Years of Age. Am J Cardiol 1983;53:353.
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