We
present the clinical case of an 80 year old woman admitted to the emergency
department with jaw pain and acute neurovascular impairment with right arm
monoparesis, pulse deficit and pallor of the limb, without chest pain as a
cardinal symptom. The history of arterial hypertension, aortic stenosis, and
clinical manifestation suggested an acute vascular compromise, for which acute
aortic syndrome (AAS) was suspected. Chest x-rays revealed mediastinal is
widening; angio-CT scan confirmed type A aortic dissection affecting the right
subclavian artery (thrombosis). The patient passed away after expectant
non-surgical management.
INTRODUCTION
Acute aortic
dissection (AAD) is a vascular emergency with high mortality; prompt detection
from emergency services is important based on the likelihood according to risk
factors and clinical presentation, which is not always limited to chest pain.
The angio-CT scan is of great importance to define the diagnosis and surgical
management in most cases. Conservative treatment of AAD, as in this case, has a
high mortality rate. This report demonstrates the variety of the presentation
of the acute aortic syndrome.
CLINICAL CASE
A 80 year old
female patient with past medical history of arterial hypertension,
hypothyroidism, chronic lung disease, hiatal hernia and moderate aortic
stenosis was admitted to the emergency department for 1 h of evolution of pain
in the submandibular region and the right arm, with loss of strength in this
limb, without dyspnea or chest pain.
Physical
examination revealed hypotension, without altered consciousness or tachycardia,
right arm with decreased force, coldness, weak radial pulse and prolonged
capillary refill, adequate femoral pulses and normal cardiopulmonary
auscultation. Acute aortic pathology was suspected; chest X-rays showed
mediastinal widening; due to the high probability and clinical suspicion of an
acute aortic syndrome (AAS), an angio-CT scan was requested, showing type A
aortic dissection with involvement of the right subclavian artery (thrombosis)
and the brachiocephalic trunk. The patient was referred to another institution
for assessment by cardiovascular surgery, who analyzed the case and due to the
patient’s comorbidities, age and extension of the vascular affection; non-surgical
expectant management was decided. She remained in ICU for 24 h. The next day,
the patient presented a convulsive episode, followed by cardiorespiratory
arrest. No CPR maneuvers were performed due to previous dissent; the patient
died (Figure 1).
DISCUSSION AND CONCLUSION
Acute aortic
syndrome (AAS) encompasses aortic dissection (AD), intramural hematoma and
aortic ulcer, the former being the most prevalent, up to 80% [1,2]. It may also
be due to traumatic causes, constituting the second cause of mortality in about
80% of the subjects [3,4]. The dissection can be classified into two groups:
Stanford type A and B, with the first one involving the ascending aorta, and
the second one confined to the descending part; and the DeBakey classification
with type 1 that involves the entire extension of the aorta, type 2 the
ascending portion and type 3 is limited to the descending aorta [5].
The most known risk
factors for the appearance of this condition are atherosclerosis, smoking,
dyslipidemia, cardiac surgery, autoimmunity, connective tissue disease,
cardiovascular family history, valvulopathy and in more than 70%, arterial
hypertension [6].
In the IRAD study
(International Registry of Acute Aortic Dissection) conducted in 12 reference
centers, it was observed that the clinical presentation of patients with AD is
protean, evidencing that chest pain occurs in 72.2% of patients [7]; for
Spanish surveys such as RESA, this symptom presented in 95.9% [1]; the sudden
onset of symptoms occurred in 95.5%, back pain 53%, abdominal pain 29% and
syncope (14% in RESA and 9.4% in IRAD studies). Recent publications describe
increased neurological manifestations with respect to previous studies: 17% are
limb motor deficits, being these manifestations more persistent in type A
dissection [6].
The most frequent
clinical findings in the IRAD study were the presence of aortic murmur (31.6%)
and pulse deficit in 15% of the cases. In the present report, the patient was
diagnosed under neurological findings; according to the literature, this type
of manifestations correspond to less than 10% of the cases, including: syncope,
coma, dysphonia, spinal cord injury or cerebrovascular accident [7], most of
these are transient (in 15-40% of the cases) and explained by four physio-pathological
mechanisms: poor perfusion, hypotension, distal thromboembolism and compression
of peripheral nerves [5]. The ischemic neuropathy of the limbs secondary to
poor perfusion is mainly due to subclavian and femoral involvement in up to 10%
[5]. For European publications, the neurological deficit occurs in the same
proportion but includes 8% of patients who present with pain in one limb [1,5].
The diagnosis of
the SAA proposed by AHA in 2010 [8] is based initially on the pre-test
probability according to the risk factors, the characteristics of the pain, and
the clinical examination; the presence of two or more of these factors
constitute a high probability, being necessary an imaging request; they
initially recommend transthoracic echocardiography followed by an angio-CT
scan. Patients with low probability require additional studies such as chest
X-rays and D-dimer [5,8]. X-rays may be normal in 21% of cases [7]. Angio-CT
scan is the gold standard for diagnosis [5].
The importance of
knowing this entity lies in the variability of the presentation along with the
morbidity and mortality that it comprises; type A dissections are considered
the most common according to the IRAD survey, corresponding to 62%; and of
these, those that undergo surgery have an in-hospital mortality of 26% against
58% in those that receive medical treatment (due to comorbidities, age or
rejection of surgical management). Type B dissection has a mortality rate of
31% in surgical management versus 10% under medical management [7]. The early
recognition of this pathology depends on the clinical suspicion of the
physicians in the emergency department; there are reports that demonstrate that
this pathology only occurs in up to 15% of patients as the first diagnostic
impression [9]; in 85% of the cases, the diagnosis is delayed. Other
publications report that up to 40% of patients are not diagnosed with the
initial symptom [2,10,11].
Surgical
complications include spinal cord injury (6.8%), stroke (9%), mesenteric
ischemia (4.9%) and acute kidney injury (19%). Endovascular management is
another invasive method that is used for type B dissection and carries similar
surgical risks [12].
Medical management
is based on anti-impulse therapy consisting of management in high-dependency
units, pain control and mainly stabilization of heart rate and blood pressure,
seeking for goals of 60 to 80 beats/min and 100-120 mm Hg of systolic blood
pressure [7]. The drugs of choice for analgesia are opioids and beta-blockers
such as esmolol or labetalol to control blood pressure and heart rate.
These atypical
manifestations of the case are presented to inform the key professionals for
the diagnosis of this cardiovascular emergency, whose manifestation is not
always the usual; and, in this way, to be able to establish prompt management,
impacting on the morbidity and mortality of the patients.
1. Evangelista
A, Padilla F, Lopez-Ayerbe J, Calvo F, Lopez J, et al. (2009) Registro Español
del síndrome aórtico agudo (RESA). La mejora en el diagnóstico no se refleja en
la reducción de la mortalidad. Rev Eso Cardiol 62: 255-262.
2. Lech C,
Swaminathan A (2017) Abdominal aortic emergencies. Emerg Med Clin N Am 35:
847-867.
3. Strayer
R (2017) Thoracic aortic syndromes. Emerg Med N Am 35: 713-725.
4. Trust M,
Teixeira P (2017) Blunt trauma of the aorta: Current guidelines. Cardiol Clin
35: 441-451.
5. Erbel R,
Aboyans V, Borleau C, Bossone E, Di Bartolomeo R, et al. (2015) Guía ESC 2014
sobre el diagnóstico y tratamiento de la patología de la aorta. Rev Esp Cardiol
68: 242.e1-e69.
6. Lech C,
Swaminathan A (2017) Abdominal aortic emergencies. Emerg Med Clin N Am 35:
847-867.
7. Hagan P,
Nienaber C, Isselbacher E, Bruckman D, Karavite D, et al. (2000) The
International Registry of Acute Aortic Dissection (IRAD). New insights into an
old disease. JAMA 283: 897-903.
8. Hiratzka
L, Bakris G, Beckman J, Bersin R, Carr V, et al. (2010)
ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM. Guidelines for the diagnosis and
management of patients with Thoracic Aortic Disease: A report of the American
College of Cardiology Foundation/American Heart Association Task Force on
Practice Guidelines, American Association for Thoracic Surgery, American
College of Radiology, American Stroke Association, Society of Cardiovascular
Anesthesiologists, Society for Cardiovascular Angiography and Interventions,
Society of Interventional Radiology, Society of Thoracic Surgeons and Society
for Vascular Medicine. Circulation 121: e266-e369.
9. Mészáros
L, Mórocz J, Szlávi J, Schmidt J, Tornóci L, et al. (2000) Epidemiology and
clinicopathology of aortic dissection. Chest 117: 1271-1278.
10. Rylski
B, Suedkamp M, Beyersdorf F, Nitsch B, Hoffmann I (2011) Outcome after surgery
for acute aortic dissection type A in patients over 70 years: Data analysis
from the German Registry for Acute Aortic Dissection Type A (GERAADA). Eur J
Cardiothorac Surg 40: 435-440.
11. Fattori R, Cao P, De Rango P, Czerny M,
Evangelista A (2013) Interdisciplinary expert consensus document on management
of type B aortic dissection. J Am Coll Cardiol 61: 1661-1678.
12. Higa C,
Guetta J, Borraci R, MeribilHaa R, Marturano M, et al. (2009) Registro
multicéntrico de disección aórtica aguda. Estudio RADAR. Resultados
preliminares. Rev Argent Cardiol 77: 354-360.
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