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Acute Aortic Syndromes: Diagnosis and Management, An Update
Acute Aortic Syndromes: Diagnosis and Management, An Update
Acute Aortic Syndromes: Diagnosis and Management, An Update
doi:10.1093/eurheartj/ehx319
Clinical update
Received 17 March 2016; revised 1 April 2017; editorial decision 10 May 2017; accepted 13 June 2017; online publish-ahead-of-print 30 June 2017
Acute aortic syndromes (AAS) encompass a constellation of life-threatening medical conditions including classic acute aortic dissection
(AAD), intramural haematoma, and penetrating atherosclerotic aortic ulcer. Given the non-specific symptoms and physical signs, a high
clinical index of suspicion is necessary to detect the disease before irreversible lethal complications occur. In order to reduce the diagnos-
tic time delay, a comprehensive flowchart for decision-making based on pre-test sensitivity of AAS has been designed by the European
Society of Cardiology guidelines on aortic diseases and should be thus applied in the emergency scenario. When the definitive diagnosis is
made, prompt and appropriate therapeutic interventions should be undertaken if indicated by a highly specialized aortic team. Urgent sur-
gery for AAD involving the ascending aorta (Type A) and medical therapy alone for AAD not involving the ascending aorta (Type B) are
typically recommended. In complicated Type B AAD, thoracic endovascular aortic repair (TEVAR) is generally indicated. On the other
hand, in uncomplicated Type B AAD, pre-emptive TEVAR rather than medical therapy alone to prevent late complications, while intuitive,
requires further study in randomized cohorts. Finally, it should be highlighted that there is an urgent need to increase awareness of AAS
worldwide, including dedicated education/prevention programmes, and to improve diagnostic and therapeutic strategies, outcomes, and
lifelong surveillance.
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Keywords Acute aortic syndromes • Aortic dissection • Intramural haematoma • Penetrating aortic ulcer
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Introduction .. Acute aortic syndromes
..
..
The aorta, called ‘the greatest artery’ by the ancients, is the ulti- .. Acute aortic syndromes are a constellation of life-threatening medical
mate conductance vessel carrying roughly 200 million litres of .. conditions, including classic acute aortic dissection (AAD), intramural
..
blood to the body in an average lifetime. Anatomically, it is divided .. haematoma (IMH), penetrating atherosclerotic aortic ulcer (PAU)
into thoracic and abdominal components that are located above .. (even thoracic aortic rupture), which share common pathophysiolog-
..
and below the diaphragm, respectively. The thoracic aorta is div- .. ical pathways (breakdown of the intima and media), clinical character-
ided into the aortic root and the ascending, arch, and descending .. istics, and diagnostic and therapeutic challenges.2,9
..
segments; and the abdominal aorta into the suprarenal and ..
infrarenal segments.1,2 ..
..
As a ‘whole organ’, the aorta may be affected by several congenital .. Classification
or acquired diseases, either acute or chronic, involving the thoracic .. Anatomically, there are two commonly used classification schemes
..
and/or abdominal components (holistic approach).2,3 In this review, .. for aortic dissection. The DeBakey system categorizes dissections
we focus on recent advances in the diagnostic and therapeutic path- .. based on the origin of the intimal tear and the extent of the dissec-
..
ways of acute aortic syndromes (AAS) derived in large measures .. tion, and the Stanford system divides dissections according
from multiple registries/population-based studies and recent consen- .. to whether the ascending aorta is involved (Type A) or not
..
sus statements/guidelines developed by American and European .. involved (Type B), regardless of the site of origin (Table 1, Figures 1
Specialty Societies.2–8
.. and 2).2,9–11
* Corresponding authors. Email: keagle@med.umich.edu; Tel: þ39 081 824 0067, þ39 328 541 5438, Fax: þ39 081 824 0067, Email: ebossone@hotmail.com
Published on behalf of the European Society of Cardiology. All rights reserved. V
C The Author 2017. For permissions, please email: journals.permissions@oup.com.
740 E. Bossone et al.
..
.. Historically, acute dissection has been defined as occurring within
Table 1 Classification schemes of aortic dissection .. 2 weeks of symptom onset, with chronic dissection occurring beyond
..
DeBakey (Figure 1) .. the second week. This cut-off of 14 days is based on survival esti-
.. mates derived in part from the work of Hirst et al.12 in the late 1950s.
Category I Dissection tear in the ascending aorta propagat- ..
ing distally to include at least the aortic arch .. As a result of substantial advances in diagnostic modalities, treatment
.. strategies, and their relative impact on early and late outcomes, the
and typically the descending aorta ..
Category II Dissection tear only in the ascending aorta
.. European Society of Cardiology (ESC) guidelines have recently sug-
.. gested to further divide the time course of aortic dissection into
Category III Dissection tear in the descending aorta propa- ..
gating most often distally
.. acute (<14 days), subacute (15–90 days), and chronic (>90 days)
.. phases.2 Booher et al.,13 using data from the International Registry of
Category IIIa Dissection tear only in the descending thoracic ..
aorta
.. Aortic Dissection (IRAD), developed Kaplan–Meier survival curves
.. distinct for dissection and treatment type (surgical þ medical or med-
..
Figure 1 Classification of aortic dissection localization (see Table 1). Modified from Erbel et al.,2 with copyright permission.
Acute aortic syndromes 741
..
Table 2 Risk factors for development of thoracic
.. disease before irreversible lethal complications occur,24 which can
.. sometimes be challenging, given the potential for overlapping symp-
aortic dissection ..
.. toms between AAD and the more common acute coronary syn-
Conditions associated with increased aortic wall stress .. dromes and/or stroke.14,24
..
Hypertension, particularly if uncontrolled ..
Pheochromocytoma
..
..
Cocaine or other stimulant use .. Laboratory testing
Weightlifting or other Valsalva manoeuvre
..
..
Trauma .. Laboratory testing complements the clinical assessment of
Deceleration or torsion injury (e.g. motor vehicle crash, fall)
.. patients with suspect or overt AAD and may be useful for differ-
..
Coarctation of the aorta .. ential diagnosis and/or detection of life-threatening complications
Conditions associated with aortic media abnormalities
.. (Table 4).2,25,26
..
Table 3 Presenting symptoms, signs, chest X-ray, and electrocardiographic features from the International Registry
of Aortic Dissection registry
Category Overall (n 5 5638) Type A AAD (n 5 3747) Type B AAD (n 5 1891) P-value
....................................................................................................................................................................................................................
Symptoms and signs
Chest or back pain 4692 (87.9%) 3113 (87.5%) 1579 (88.7%) 0.191
Severe or worst ever pain 4692 (87.9%) 3113 (87.5%) 1579 (88.7%) 0.191
Abrupt onset of pain 4220 (84.0%) 2789 (83.3%) 1431 (85.4%) 0.052
Migrating pain 664 (14.8%) 400 (13.7%) 264 (16.8%) 0.005
Pain presenting within 6 h of symptom onset 2950 (75.8%) 1700 (77.0%) 790 (73.1%) 0.015
Any focal neurological deficit 695 (13.7%) 575 (17.2%) 120 (7.0%) <0.001
A B
Figure 3 Imaging pitfalls in acute aortic dissection. (A) A 62-year-old male who presented with chest pain and a known thoracic aortic aneurysm.
The initial ungated computed tomography of the thoracic aorta has significant motion artefact, which can be confused with a possible dissection flap
(arrows). (B) Repeat computed tomography of the thoracic aorta with electrocardiographic gating, demonstrating the absence of any dissection flap.
(C) A 51-year-old male with a transthoracic echocardiographic examination ordered for a systolic ejection murmur, which showed a linear echoden-
sity (arrows) in the ascending aorta that could represent a dissection flap. (D) Subsequent computed tomography displayed a normal ascending aorta,
suggesting that this echodensity represented an artefact.
Computed tomography and magnetic .. department for the differential diagnosis of acute chest pain (high
..
resonance imaging .. negative predictive value) among AAS, pulmonary embolism, and
Tomographic imaging—whether by CT or MRI—enables a complete
.. coronary artery disease (‘triple rule out’ strategy).44,45 Computed
..
and detailed map of the entire aorta and its branches. Both modalities .. tomography also permits assessment of aortic calcification patterns
..
are characterized by high spatial/contrast resolution without limits for .. and can be helpful in follow-up imaging of patients with aortic stents.
imaging window, giving an excellent accuracy to detect AAS and related .. The quick pulsatile movement of the aortic wall during the cardiac
..
complications such as pericardial and/or pleural effusion. Compared .. cycle may produce fake linear intraluminal structures on CT images
with echocardiography, tomographic imaging modalities do not allow .. of the ascending aorta, mimicking an intimal flap (pulsation artefacts),
..
bedside use and this may limit their feasibility in unstable patients.36,42,43 .. particularly in non-ECG-gated studies (Figure 3). When considering
Non-enhanced CT followed by contrast-enhanced CT angiogra- .. CT angiography, it should be remembered that the use of iodinated
..
phy should generally be preferred (16 detector systems or high-end .. contrast agents leads to a non-negligible risk for allergic reaction and
devices) to assess patients with suspected AAS, considering its excel- .. renal insufficiency. In the case of serial imaging, over a period of years,
..
lent diagnostic accuracy (pooled sensitivity 100% and pooled specific- .. substantial concern remains over the use of ionizing radiation and the
ity 98%), widespread availability, easier patient access and tolerance, .. related cancer risk, which is considerably higher in women and in
..
and short-time image acquisition/processing, enabling rapid collection .. young individuals.2,46
of key information for clinical decision-making.2,32 Emergency CT .. Magnetic resonance imaging also provides high levels of diagnostic
..
angiography is also considered the first-line technique (followed by .. accuracy in the evaluation of patients with suspected AAS (pooled
TOE) in case of clinically suspected traumatic thoracic aortic injury .. sensitivity 98% and pooled specificity 98%).32
..
(sensitivity and specificity close to 100%).2 Recently, ECG-gated 64- .. In addition to detailed anatomical information on the aortic vessel
detector CT has been more often implemented in the emergency
.. wall, MRI can also provide a reliable characterization of aortic
Acute aortic syndromes 745
..
insufficiency typically associated with Type A aortic dissection, by .. suspected AAS. Owing to its invasive nature, time and cost, it is now
including phase-contrast velocity mapping for flow quantification. .. rarely performed (except in the case of coronary angiography or
..
Furthermore, the time-resolved angiographic techniques as well as .. endovascular interventions), having been replaced by non-invasive
the most common cine bright blood images may add dynamic infor- .. imaging techniques.2 Coronary artery involvement may be present in
..
mation by visualizing flow patterns within the true lumen and the false .. approximately 20% of Type A AAD patients as a direct consequence
lumen. Newer approaches with 4D flow MRI have shown promise in .. of flow obstruction by the dissection flap occluding the orifice and/or
..
defining the flow characteristics and associated parameters of aortic .. propagating down the coronary artery with or without pre-existing
dissection but need to be further refined.9,47–49 Owing to the long .. atherosclerotic disease. However, routine coronary angiography is
..
scan time duration (20–30 min for the aortic evaluation protocol), .. not recommended before surgery. Major concerns include additional
MRI is certainly less suited in emergent or unstable scenarios than .. time delay to emergency surgery and technical difficulties along with
..
CT. Additional drawbacks of MRI include potential contraindications .. an increased risk of aorta injury. In an IRAD review of 1343 Type A
such as ferromagnetic and/or magnetically activated implants and .. AAD, only 156 patients (11.6%) underwent pre-operative coronary
..
B
A
FL
TL
Figure 4 Multimodality imaging of acute aortic dissection. A common approach to suspected acute aortic dissection includes the combination of
computed tomography and echocardiography. This patient was a 75-year-old male presenting with acute abdominal pain, who was imaged by a non-
gated computed tomography at another hospital, which identified a Type A AAD, with a flap originating just above the right coronary artery (arrow),
which came off the true lumen (A) and extended to the abdominal aorta [thoracic aorta dissection flap indicated by arrows on the 3D image from
non-gated computed tomography (B)]. A transthoracic echocardiogram was obtained, which also visualized the dissection flap in the aortic root
[arrow, (C)], with a communication between the true and false lumen on color Doppler [arrow, (D)]. There was only mild aortic regurgitation
[arrow, (E)], and left ventricular function and wall motion were normal, suggesting normal flow into the coronary arteries. The patient underwent
emergent surgery for aortic repair, with transoesophageal echocardiography performed during the operation and survived with no significant post-
operative complications.
..
treatment, was associated with improved 5-year aorta-specific survival, ..
..
Long-term follow-up
delayed disease progression, and positive remodelling. However, no
.. The 10-year actuarial survival rate among patients with aortic
difference regarding total mortality was observed, and the trial was rel- ..
atively small in size.8 .. dissection who survive initial hospitalization ranges from 30% to
.. 60%.2,9,74–80 AAS should be considered a lifelong problem involving
Currently, TEVAR is recommended by ESC guidelines for compli- ..
cated Type B dissection defined by persistent or recurrent pain, .. the entire aorta and its branches that remain at high risk for redissec-
..
uncontrolled hypertension, despite full medication, early aortic .. tion, aneurysm formation, and rupture even after successful treat-
expansion, malperfusion, and signs of rupture (haemothorax, increas- .. ment of the acute index event.81 Thus, patients with AAS, regardless
..
ing periaortic and mediastinal haematoma).2,65–67 .. of the initial therapeutic strategy (medical, interventional, or surgical),
A small cohort of Type B AAD patients may require surgery .. require lifelong clinical and imaging monitoring, patient education,
..
usually when TEVAR is contraindicated and/or not feasible.2,65–67 .. and, if appropriate, screening of family members for aortic disease.
It should be underlined that open surgery carries high in-hospital .. Given that the risk of aortic complications (especially false lumen
..
..
Table 6 Long-term follow-up
.. consists of a circular or crescentic thickening of >5 mm of the aortic
.. wall, without evidence of blood flow on imaging examination; CT and
..
Ten-year survival rate from 30% to 60% .. MRI are the techniques of choice. In particular, the combination of an
.. unenhanced acquisition. followed by a contrast-enhanced CT acquisi-
Late complications ..
Progressive aortic insufficiency .. tion yields a sensitivity of up to 96% for the detection of IMH.2 IMH is
.. more frequently observed in the descending thoracic aorta (Type B
Progressive diameter increase, aneurysm formation, and rupture ..
Recurrent dissection or progression of dissection .. IMH, 60–70%) and less commonly in the ascending aorta and aortic
.. arch (Type A IMH; 30% and 10%, respectively). Compared with clas-
Leakages/haemorrhage at surgical anastomoses/stent-grafted sites ..
Malperfusion .. sic aortic dissection, patients with IMH have quite similar symptoms
.. and risk factors, with acute chest pain/back pain predominating.
Patients at particularly high risk ..
Those with Marfan syndrome—very high risk of recurrent .. However, they tend to be older and less frequently show aortic valve
.. insufficiency, pulse deficits, acute myocardial infarction, and aneurys-
..
..
An asymptomatic lesion may also be identified as an incidental finding .. It remains, thus, essential to design and implement regionalized sys-
during axial imaging screening performed for other indications. In .. tems of care for cardiovascular emergencies along with multidiscipli-
..
addition, incidental aortic findings are not uncommon on cardiovas- .. nary aortic centres equipped with advanced imaging techniques that
cular imaging, and future research may be helpful to determine which .. include structural and functional assessment of the entire aorta in a
..
findings should prompt further testing and/or management changes. .. comprehensive concept of personalized treatment for every
Among imaging modalities, contrast-enhanced CT, including axial .. patient.118,119 In this regard, more evidence is needed on the
..
and multiplanar reformations, is considered the diagnostic technique of .. volume–outcome relationship in the field of aortic diseases.
choice.2 The natural course of PAU continues to be debated and may .. In the near future, we may see an increasing number of uncompli-
..
include the formation of medial haematoma, classical dissection, and/or .. cated Type B dissection patients receiving pre-emptive TEVAR
adventitial false aneurysm, and transmural rupture. In this regard, com- .. instead of medical therapy alone to prevent late complica-
..
pared with aortic dissection, the risk of rupture (7% for Type A and 4% .. tions.9,64,120–123 This strategy, while intuitive, requires further study in
for Type B AAD) is considerably higher (up to 40% for PAU).107 Thus, .. randomized cohorts.
..