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Review of Literature

Acute aortic dissection is defined as occurring within 2 weeks of onset of pain; subacute, between 2 and
6 weeks from the onset of pain; and chronic, more than 6 weeks from the onset of pain.(1)

Classical anatomical aortic dissection is classified according to debakey or Stanford classification.(2) The
most commonly used is the Stanford classification 3, which is based on involvement of the ascending
aorta.

Stanford classification

Type A involves the ascending aorta but may extend into the arch and descending aorta(debakey type I
and II).
Type B involves the descending aorta only(debakey type III).

Debakey classification(3) ,Type I involves ascending aorta, aortic arch, and descending aorta,
Type II is confined to ascending aorta only and Type III is confined to descending aorta distal to the left
subclavian artery only;

.
The Penn classification categorizes type A aortic dissection on the basis of clinical presentation . Class Aa
is absence of branch vessel malperfusion , class Ab is symptoms or signs of localized organ
ischaemia ,class Ac is characterized by circulatory collapse with or without cardiac involvement, and
Abc is a combination of generalized and localized ischemia .(4)

1. Nienaber CA, Powell JT. Management of acute aortic syndromes. Eur Heart J. 2012;33(1):26–35.

2. Daily PO. Management of acute aortic dissections. Ann Thorac Surg. 1970;10:237–47.

3. De Bakey ME, Henly WS, Cooley DA, Morris Jr GC, Crawford ES, Beall Jr AC. Surgical management of
dissecting aneurysms of the aorta. J Thorac Cardiovasc Surg. 1965;49(1):130–49.

4. Augoustides JG, Andritsos M. Innovations in aortic disease: the ascending aorta and aortic arch. J
Cardiothorac Vasc Anesth. 2010;24(1):198–207.

5. Howard DP, Banerjee A, Fairhead JF, Perkins J, Silver LE, Rothwell PM. Population-based study of
incidence and outcome of acute aortic dissection and premorbid risk factor control: 10-year results
from the Oxford Vascular Study. Circulation. 2013;127(20):2031–7.

6. Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The
International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. Jama.
2000;283(7):897–903.
 Howard et al. In their study found that about 35% of all aortic dissections occur in patients older than
75 years of age (5) , and age is an independent predictor of aortic dissection (6). Population with
increased age is probably contributing to the higher incidence (8.7 per 100,000 patient years) as
reported by Landenhed et al. (2015) [28], and it has been estimated that the proportion of dissections in
patients over 75 years could reach 50% by 2030 and 57% by 2050 [29,32].
The most common predisposing factor for type A aortic dissection was hypertension (72%) in the IRAD
series . 31% of patients had a history of atherosclerosis . In the total registry, 5 and 4% of cases of acute
aortic dissection were thought to be related to Marfan’s syndrome and iatrogenic causes, respectively. 1

In Younger patients(<40 years ) with type A aortic dissection had lower incidence of history of
hypertension (34%) or atherosclerosis (1%), but more commonly have Marfan´s syndrome, bicuspid
aortic valve and/or prior aortic surgery.2 

Aortic dissection has a diversity of presentations. Therefore, a high index of suspicion should be used in
patients with predisposing risk factors, e.g. Hypertension, aneurysmal disease of the aorta, or a familial
connective tissue disorders. Typically the patient is a hypertensive male in his 60s, with a history of
abrupt onset of chest pain, Marfan’s syndrome, Turner syndrome, Ehlers-Danlos syndrome, aortic
aneurysm, anulo-aortic ectasia, aortic arch hypoplasia, coarctation of aorta, bicuspid aortic valve,
connective tissue disorders, pregnancy, and cocaine abuse 18.

85% of patients in NORCAAD presented with sudden, intense chest pain [17]. The pain is usually located
retrosternally or substernally, and may propagate in a distal or proximal direction as the dissection
evolves. The innervations of aortic adventitia is by autonomic afferent nerve fibres, thus intimal tear
and disruption of the media results in intense visceral pain [47]. In rare cases, the dissection presents
with less symptoms and is revealed in a subacute phase, or leads to sudden death [17].

In a recent IRAD study by Pape et al. assessing outcomes and trends in 4428 consecutive patients with
acute aortic dissection enrolled between December 1995 and February 2013, 2,952 (67%) had type A
dissection (8). The mean age of TAAD patients was 62±14.6 years. Although less frequently affected by
TAAD, women enrolled in IRAD were significantly older than men (67.9 vs. 60.6 years) and had a higher
mortality than males (33.5% vs. 24.1%) (9).

A history of hypertension (74.4%) was the most common predisposing condition and there was also a
relatively high proportion of patients who had a history of atherosclerosis (23.8%). Furthermore, in the
total registry, 4.5% and 3% of TAAD were related to Marfan syndrome and iatrogenic causes respectively
(8). Several race-related differences have also been described in IRAD patients (10). Among all
dissections, TAAD was less frequent in the black population compared with whites (47.6% vs. 60.7%).
One-third of the patients have symptoms accompanied with malperfusion due to impaired flow in end-
organ arteries [48]. Lower-extremity malperfusion and pulse deficit are clinically detectable , while signs
of renal and intestinal ischemia are difficult to detect in the acute phase. In rare cases, cerebral
ischaemia or paraplegia are the first presenting symptoms of acute aortic dissection [3].
. Patients with aortic dissection typically present a cataclysmic onset and chest and/or back pain of a
blunt, sometimes radiating nature. However, in contrast to classic teaching, ‘tearing’, ‘ripping’ or
‘migratory’ were not common descriptors of pain in IRAD. Chest pain was significantly more common in
patients with type A than type B dissections (79 versus 63%), whereas both back pain (64 versus 47%)
and abdominal pain (43 versus 22%) were significantly more common in type B dissection. Hypertension
at the time of presentation was more frequent in type B than in type A dissection (70 versus 36%). 1

Syncope a well known symptom of acute aortic dissection, often indicates the development of
dangerous complications like cardiac tamponade and cerebrovascular accident . IRAD reported syncope
in 13% of patients .7 These patients were more likely to die in the hospital (34 versus 23% of those
without syncope) and were more likely to have cardiac tamponade, stroke, neurological deficits and a
proximal dissection.. A pulse deficit has been described in 30% of patients with an acute type A
dissection in IRAD . These patients have a higher rate of in-hospital complications and mortality than
those without a pulse deficit.8

In study from IRAD9 concluded that the group of patients presenting with predominantly abdominal pain
(5%) experienced higher mortality than those with more typical symptoms (10 versus 8%; p=0.02). This
emphasises the atypical symptomatology in some patients and the possibility for acute aortic dissection
to mimic other disorders such as stroke, myocardial infarction, vascular embolisation and abdominal
pathology. Thus, diagnosis of this disease requires a high index of suspicion of an aortic dissection in
patients who have related risk factors.
For successful management of type a aortic dissection a prompt diagnosis based early suspicion is
necessary.
Harris et al. (11) demonstrated that delay in acute aortic dissection diagnosis is more likely in female
patients, patients without abrupt onset of pain or those presenting without pain, patients with an
absence of pulse deficit or hypotension or patients who initially presented to a non-tertiary care
hospital. The registry reported that 6.4% of patients presented with painless aortic dissection. In these
patients hospital mortality was significantly higher than patients with painful dissection (33.3% vs.
23.2%) (12). Typical symptoms (abrupt onset of chest or back pain) and signs (aortic regurgitation
murmur or pulse deficits) of dissection were less common among the elderly ( 13). In the 2010
Guidelines on Thoracic Aortic Disease, a risk assessment tool had been proposed in this setting to
provide clinicians with a simple and systematic method for rapidly identify patients at high risk and to
provide a framework for additional diagnostic testing based on a pretest probability of disease (14).
According to an autopsy study16,aortic dissection was the most common missed diagnosis, and according
to a recently published analysis, physicians suspected diagnosis of aortic dissection on initial
presentation in 43 % of patients in whom diagnosis was confirmed subsequently. The index of suspicion
was highest(86 %) in patients who presented with chest and back pain, followed by chest pain (45 %)
and abdominal pain (8 %)17.Due to a tremendous tragedy associated with late or missed diagnosis of
aortic dissection, it is imperative to be aware of all the clinical manifestations of aortic dissection.

Given the frequency of atypical presentations and time dependent mortality, timely and accurate
imaging is paramount to the prompt detection and treatment of TAAD.

Conventional chest X-ray can show a wide mediastinum , which is not specific for diagnosis [27]. Chest
radiographic findings may be normal in 10%–40% of aortic dissections. In the study by Hagan et al 19, a
widened mediastinum was noted in 61.1% of aortic dissection cases. Displacement of calcification of the
aorta was reported in 14.1% of cases, with an abnormal cardiac contour being noted in 25.8% 19.
Marked enlargement of the heart indicates a pericardial effusion or presence of a new pleural effusion
suggests complication.

The most accurate and quickest method for confirming the diagnosis of type A aortic dissection is by
computed tomography (CT) scan of the aorta.

The aim is to visualize the true and the false lumen of the aorta during dissection, to identify tears of the
intima in-between the lumen, and to evaluate the extent of the dissection and the involvement of the
branching arteries of the aorta including the arch vessels . The sensitivity and specificity of CT are
excellent [50], but in patients with poor renal function or allergy to iodinated dye, CT angiography is
questionable and other imaging modalities such as magnetic resonance imaging (MRI) or
ultrasonography can be considered instead. On the other hand, MRI is seldom used―as it is too time-
consuming and still often unavailable, but it may complement CT angiography in follow-up imaging of
the aorta [51].

Transthoracic echocardiography (TTE) reveals the functional state of the heart, valves and aortic root,
and can show dilatation of the aorta, pericardial effusion and tamponade, but it is associated with poor
visibility in the obese and in patients on mechanical ventilation [52]. More detailed information is
obtained with Transesophageal echocardiography (TEE), especially for detection of pericardial effusion
and aortic valve regurgitation, and often identification of the primary intimal tear in the aortic root. In
expert hands, TEE is a powerful diagnostic tool, and its sensitivity and specificity approach 100% [52,53].
Coronary angiography is not indicated as a primary diagnostic method for ATAAD, but sometimes it is
detected at coronary angiography, when acute coronary syndrome (ACS) is initially suspected. In highly
specialized centres, coronary angiography is performed as part of a complete work-up done in a hybrid
suite, immediately before open surgery, as up to 30% of ATAAD patients have unknown but significant
coronary artery disease that may warrant treatment and affect outcomes [54].
Transesophageal echocardiography (TEE) is reported to have a sensitivity of 94%–100% and a specificity
of 77%–100% for identifying an intimal flap 22,23. However, the distal part of the ascending aorta and the
branches of the aortic arch may not be adequately evaluated with TEE 23. In patients with an identifiable
dissection flap, secondary signs of an aortic dissection such as aortic root dilatation, aortic regurgitation,
coronary ostial patency, pericardial effusions, or regional abnormal wall motion can be diagnosed 24. TEE
can be performed in the emergency department at the bedside of unstable patients, thereby helping
prevent delays in accurate diagnosis and treatment. At many centers, however, the value of TEE is
limited by a lack of expertise and availability .

With similar sensitivity, computed tomography (CT) and trans-esophageal echocardiography (TEE) have
become the standard of care for the diagnosis of this acute aortic syndrome. In IRAD, almost 70% of
patients underwent multiple imaging studies. The first diagnostic modality of choice was CT in 63%,
followed by TEE in 32%, aortography in 4% and MRI in 1% (16).

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