ESC GUIDELINES ON THE DIAGNOSIS AND
TREATMENT OF AORTIC DISEASES
AORTIC DISEASES
ESSENTIAL MESSAGES FROM
ESC GUIDELINES
Committee for Practice Guidelines
To improve the quality of clinical practice and patient care in Europe
For more information
www.escardio.org/guidelines
The Task Force on diagnosis and treatment of aortic diseases
of the European Society of Cardiology (ESC)
Chairpersons
2014 ESC GUIDELINES ON THE DIAGNOSIS
AND TREATMENT OF AORTIC DISEASES
*
*Adapted from the 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases (European Heart Journal (2014) 35, 2873–2926 - doi:
10.1093/eurheartj/ehu281).
Raimund Erbel
Department of Cardiology
West-German Heart Center
University
Duisburg-Essen
Hufelandstr
55
DE-45122 Essen, Germany
Tel.: 49 201 723 4801
Fax: 49 201 723 5401
Email: erbel@uk-essen.de
Victor Aboyans
Department of Cardiology
Dupuytren University Hospital
2. Avenue Martin Luther King
87042 Limoges, France
Tel. +33 5 55 05 63 10
Fax +33 5 55 05 63 84
Email: victor.aboyans@chu-limoges.fr
Authors/Task Force Members
Catherine Boileau (France), Eduardo Bossone (Italy), Roberto Di Bartolomeo (Italy), Holger Eggebrecht
(Germany), Arturo Evangelista (Spain), Volkmar Falk (Switzerland), Herbert Frank (Austria), Oliver
Gaemperli (Switzerland), Martin Grabenwöger (Austria), Axel Haverich (Germany), Bernard Iung
(France), Athanasios John Manolis (Greece), Folkert Meijboom (Netherlands), Christophe A.
Nienaber (Germany), Marco Roffi (Switzerland), Hervé Rousseau (France), Udo Sechtem (Germany),
Per Anton Sirnes (Norway), Regula S. von Allmen (Switzerland), Christiaan J.M. Vrints (Belgium).
Other ESC entities having participated in the development of this document:
ESC Associations:
Acute Cardiovascular Care Association (ACCA), European Association of Cardiovascular Imaging
(EACVI), European Association of Percutaneous Cardiovascular Interventions (EAPCI).
ESC Council:
Council for Cardiology Practice (CCP).
ESC Working Groups:
Cardiovascular Magnetic Resonance, Cardiovascular Surgery, Grown-up Congenital Heart
Disease, Hypertension and the Heart, Nuclear Cardiology
ESC Staff:
Veronica Dean, Catherine Despres, Myriam Lafay, Sophia Antipolis, France
Special thanks to Jose Luis Zamorano, Jeroen J. Bax, Michal Tendera, Petros Nihoyannopoulos
European Heart Journal (2014) 35, 2873–2926 - doi: 10.1093/eurheartj/ehu281
ESSENTIAL MESSAGES FROM
FROM THE 2014 ESC GUIDELINES
ON DIAGNOSIS AND TREATMENT
OF AORTIC DISEASES
Section 1 - Take home messages
Section 2 - Major gaps in evidence
Table of contents
1. The holistic view to the aorta as “whole organ”
The guidelines on diagnosis and treatment of aortic diseases highlight the value of a holistic
approach, viewing the aorta as the whole organ; indeed, in many cases tandem lesions of the
aorta may exist, as illustrated by the increased probability of thoracic aortic aneurysm in the case of
abdominal aortic aneurysm, making a distinction between the two regions inadequate. In addition
thorako-abdominal aortic diseases are overwriting this separation.
2. Diagnostic Imaging
Whereas a clinical examination and laboratory testing play a minor role in the diagnosis and
treatment of aortic diseases, imaging techniques, particularly modern images techniques, play a
major role yielding a view of the total aorta, which requires standardized reports and measurements
at given landmarks.
In the daily work-up transthoracic echocardiography plays a major role including transoesophageal
echocardiography as well as ultrasonography for the abdominal aorta.
It is recommended to measure diameters at anatomical landmarks perpendicular to the longitudinal
axis. In case of repetitive imaging of the aorta the imaging should be used, with the lowest
iatrogenic risk. In addition, it is recommended to use the same imaging modality with the similar
method of measurement. All relevant parameters are recommended to be reported recording to
the aortic segmentation. It is recommended to assess renal function, pregnancy and history of
allergy to contrast agents in order to select the optimal imaging modality with minimal radiation
exposure.
Complete list of normal values for all discussed imaging techniques are found in the Full Text and
Web Addenda.
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
Comparison of methods for imaging the aorta
Advantages/disadvantages
TTE
TOE
CT
c
MRI
c
AORTOGRAPHY
Ease of use
+++
++
+++
++
+
Diagnostic reliability
+
+++
+++
+++
++
Bedside/interventional use
a
++
++
–
–
++
Serial examinations
++
+
++(+)
b
+++
–
Aortic wall visualization
c
+
+++
+++
+++
–
Cost
–
–
– –
– – –
– – –
Radiation
0
0
– – –
–
– –
Nephrotoxicity
0
0
– – –
– –
– – –
CT = computed tomography; MRI = magnetic resonance imaging; TOE = transoesophageal echocardiography;
TTE = transthoracic echocardiography.
+ means a positive aspect and – means a negative point. The number of signs indicates the estimated potential value.
a
IVUS can be used to guide interventions (see web addenda art www.escardio.org/guidelines).
b
+++ only for follow-up after aortic stenting (metallic struts), otherwise limit radiation.
c
PET can be used to visualize suspected aortic inflammatory disease.
3. The acute aortic syndrome
Acute aortic syndromes (AAS) are defined as emergency conditions with similar clinical characteristics
involving the aorta: aortic dissection, intramural haematoma, penetrating aortic ulcer, complete
rupture of the aorta, traumatic aortic injury, iatrogenic aortic dissection. A flowchart for the
emergency room has been developed in order to enhance the standardization of decision making
in acute aortic syndromes, because survival is strongly related to time. In the diagnostic work-up
clinical data are useful to assess the priori probability of AAS including the high-risk patient conditions,
high-risk pain features and signs of high-risk examination features.
Based on the probability of acute aortic syndromes the decision making can be based according to
developed flow-chart.
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
Clinical data useful to assess the
a priori probability of acute aortic syndromes
High-risk conditions
High-risk pain features
High-risk examination features
• Marfan syndrome
(or other connective tissue
diseases)
• Family history of aortic disease
• Known aortic valve disease
• Known thoracic aortic aneurysm
• Previous aortic manipulation
(including cardiac surgery)
• Chest, back, or abdominal
pain described as any of
the following:
- abrupt onset
- severe intensity
- ripping or tearing
• Evidence of perfusion deficit:
- pulse deficit
- systolic blood pressure difference
- focal neurological deficit (in
conjunction with pain)
• Aortic diastolic murmur
(new and with pain)
• Hypotension or shock
Table modified from Rogers AM et al Circulation 2011;123:2213-8.
Risk score varies from 0–3 according the number of positive categories (1 point per column).
ACUTE CHEST PAIN
High probability (score 2-3)
or typical chest pain
Medical history + clinical examination + ECG
STEMI
a
: see ESC guidelines
HAEMODYNAMIC STATE
UNSTABLE
Low probability (score 0-1)
TTE + TOE/CT
b
STABLE
AAS
confirmed
AAS
excluded
Consider
alternate
diagnosis
D-dimers
d,e
+ TTE + Chest X-ray
TTE
Consider
alternate
diagnosis
No argument
for AD
Signs
of AD
Widened
media-
stinum
Definite
Type A-AD
c
Inconclusive
Refer on emergency
to surgical team and
pre-operative TOE
CT (or TOE)
AAS
confirmed
Consider
alternate
diagnosis
repeat CT
if necessary
AAS
confirmed
Consider
alternate
diagnosis
CT (MRI or TOE)
b
AAS = acute aortic syndrome; AD = aortic dissection; ESC = European Society of Cardiology; CT = computed tomography; ECG = electrocardiogram; MRI = magnetic
resonance imaging; STEMI = ST-segment elevation myocardial infarction; TTE = transthoracic echocardiography; TOE = transoesophageal echocardiography.
a
STEMI can be associated with AAS in rare cases. -
b
Pending local availability, patient characteristics, and physician experience.
c
Proof of Type A AD by the presence of flap, aortic regurgitation, and/or pericardial effusion. -
d
Preferably point-of-care, otherwise classical.
e
Also troponin to detect non-ST-segment elevation myocardial infarction.
Flowchart for decision-making based on pretest sensitivity of AAS
4. Treatment of acute aortic syndrome
4.1 Medical management
A lot of patients with aortic diseases have comorbidities such as coronary artery disease, chronic
kidney disease, diabetes, dyslipidaemia, hypertension and others. Therefore treatment and
prevention strategies have to be similar to those indicated for the above diseases. Specific treatments
in different aortic diseases are addressed in each specific chapter.
4.2 (Thoracic) endovascular aortic repair ((T)EVAR)
• It is recommended to decide the indication of endovascular repair on individual basis according
to anatomy, pathology, comorbidity and anticipated durability, of any repair using multidisciplinary
approach (Class I C).
• A sufficient proximal and distal landing zone of at least 2 cm is recommended for the safe
deployment and durable fixation of TEVAR (Class I C).
• In case of aortic aneurysm it is recommended to select a stent-graft with a diameter exceeding the
diameter of the landing zones by at least 10-15% of the reference aorta (Class I C).
• During stent graft placement, invasive blood pressure monitoring and control either
pharmacologically or by rapid pacing is recommended (Class I C).
• For complicated type B aortic dissection, TEVAR is recommended (Class I C).
• If the anatomy is suitable and the expertise available endovascular repair should be preferred over
open surgery in contained rupture of thoracic aortic aneurysm (Class I C).
• For uncomplicated type B aortic dissection endovascular therapy should be considered (Class IIa B)
as well as in complicated type B intramural haematoma, complicated type B penetrating aortic
ulcer and traumatic aortic injury (Class IIa C)
4.3 Surgery in acute aortic syndrome
• In patient with type A aortic dissection urgent surgery is recommended (Class I B).
• Surgery is also indicated in typ A intramural haematoma (Class I C).
• In case of type B penetrating aortic ulcer, surgery should be considered (Class IIa C).
• For complicated type B aortic dissection, intramural haematoma and penetrating aortic ulcer
surgery may be considered (Class IIb C).
5. Aortic aneuryms
• When an aortic aneurysm is identified at any location, assessment of the entire aorta and aortic
valve is recommended at baseline and during follow-up (Class I C).
• In case of aneurysm of the abdominal aorta, duplex ultrasound for screening of peripheral artery
disease and peripheral aneurysms should be considered (Class IIa C).
• Patients with aortic aneurysms are at increased risk of cardiovascular disease, general principles of
cardiovascular prevention should be considered (Class IIa C).
5.1 Indication for intervention for ascending and arch aortic aneuryms
• Surgery is indicated in patients who have aortic root aneurysms with maximal aortic diameter
≥50 mm for patients with Marfan syndrome (Class I C).
• Surgery should be considered in patients who have aortic root aneurysm with maximal ascending
aortic diameter:
≥45 mm for patients with Marfan syndromes with risk factors (family history of aortic dissection
and/or aortic diameter increase >3 mm/year
≥50 mm for patients with bicuspid valve with risk factors
≥55 mm for patients with no elastopathy (Class IIa C).
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
• Surgery should be considered in patients who have isolated aortic arch aneurysm with maximal
diameter ≥55 mm (Class IIa C).
• Lower levels thresholds for intervention may be considered according to body surface area in
patients of small stature or in case of rapid progression, aortic valve regurgitation, planned
pregnancy, and patients preference (Class IIb C).
• Aortic arch repair may be considered in patient with aortic arch aneurysm who already have an
indication for surgery of an adjacent aneurysm located in the ascending aorta or descending aorta
(Class IIb C).
5.2 Intervention for descending aortic aneurysms
Valvular problems associated with bicuspid aortic valve (BAV) are covered in the 2012 ESC/EACTS
guidelines on management of valvular diseases
(European Heart J 2012;33:2451-2496).
6. Abdominal aortic aneurysm
• Abdominal aortic aneurysms (AAA) have a prevalence of about 2% and are particularly found in
men >65 years and women who are smoking >65 years. Aortic AAA are usually asymptomatic
until rupture occurs. The aortic diameter relates to risks of rupture. As a screening tool ultrasound
is recommended in all men >65 years (Class I A) and considered in women >65 years and tobacco
smoking (Class IIb C).
• Very new is the advice, to use a 2 minutes extra time during TTE to check for existence of an
asymptomatic AAA in men >65 years (Class IIa B) and women >65 years who are smoking
(Class IIb C).
• Target screening should be considered in first degree siblings of AAA patients (Class IIa B).
• Please check 2014 ESC/ESA Guidelines on non-cardiac surgery for cardiovascular risk assessment
and management
(European Heart J 2014:35:2383-2431). Additional information concerning
reduction of cardiac risk in case of intervention and surgery are given.
6.1 Endovascular aortic repair and open vascular surgery
About 60% of all AAA are suitable for endovascular therapy. In randomized controlled studies
endovascular aortic repair (EVAR) reduced mortality threshold. But long-term result were similar
due to high re-intervention rates.
• Smoking cessation is recommended to slow the AAA growth (Class I B).
• AAA repair is indicated if AAA diameter exceeds 55 mm (Class I B).
• If the anatomy is suitable for EVAR, either open or endovascular aortic repair is recommended
(Class I A).
• If the aneurysm is anatomically not suitable for EVAR, open endovascular aortic surgery is
recommended (Class I C).
6.2 Management of symptomatic abdominal aortic aneurysms
In case of rupture of abdominal aortic aneurysms two randomized controlled trials are available
demonstrating similar 30 days mortality results (30.4% versus 37.4%). Based on these results
following recommendations are given.
• In suspected ruptured AAA, immediate abdominal ultrasound is recommended (Class I C).
• In case of rupture, AAA emergency repair is indicated (Class I C).
• In case of symptomatic but non rupture AAA, urgent repair is indicated (Class I C).
• In case of symptomatic AAA anatomical suitable for EVAR, either open or endovascular repair is
recommended (Class I A).
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
7. Long-term follow-up for chronic aortic dissection
• Contrast CT or MRI is recommended to confirm the diagnosis of chronic aortic dissection (Class I C).
• Close imaging surveillance in aortic dissection is indicated to detect signs of complications
(Class I C).
• In patients with chronic aortic dissection, tight blood pressure control (> 130/80 mmHg) is indicated
(Class I C).
• After TEVAR or EVAR surveillance is recommended after 1 month, 6 months, 12 months and then
yearly (Class I C).
• CT is recommended as the first choice imaging technique for follow-up after TEVAR or EVAR
(Class I C).
• In AAA Doppler ultrasound with or without contrast agents should be considered for annual
postoperative surveillance, with non-contrast CT imaging over 5 years (Class IIa C).
• For follow-up in young patients MRI should be preferred to CT for imaging magnetic resonance-
compatible stent grafts (Class IIa C).
8. Genetic diseases affording the aorta
Chromosomal and inherited syndromic thoracic aneurysms
During the last years more insight into chromosomal aortic diseases have been given not only for
the Marfan syndrome, but also for the Loeys-Dietz syndrome, the Turner syndrome, the Ehlers
Danlos syndrome Typ IV, non syndromic familiar aortic aneurysms and even aneurysms –
osteoarthritis syndrome and arterial tortuosity syndrome.
• It is recommended to investigate first degree relatives (siblings and parents) of a subject with thoracic
aortic disease to indentify a familiar form in which relatives all have a 50% chance of carrying the
familiar mutation-disease (Class I C).
• Once a familial form of thoracic acute aortic dissection (TAAD) is highly suspected, it is recommended
to refer the patient to geneticist for family investigation and molecular testing (Class I C).
• Variable of age of oncet warrants screening every 5 years of “healthy” at-risk relatives until diagnosis
(clinical or molecular) is established or ruled out (Class I C).
• In familial non syndromic TAAD, screening for aneurysm should be considered not only in the
thoracic aorta, but also throughout the arterial tree (including cerebral arteries) (Class IIa C).
8.1 Medical therapy in genetic diseases
• In Marfan syndrome beta-blockers are prescribed to reduce the progression rate.
• Angiotensin 2 receptor blockers demonstrated attenuation of the dilatation rate of aortic
aneurysms.
• In Ehlers-Danlos syndrome beta-blockers reduce arterial complications.
• No specific data are available for other genetic diseases.
9. Management of bicuspid aortic valve
BAV have a prevalence of about 1%. Fusion of the right and left coronary cusp or fusion of the
right and none coronary cusp or found combined with normal size aorta, supra-coronary dilatation
or cylindric aortic shape. The maximal aortic dilatation rate does not differ for bicuspid aortic valve
and Marfan syndrome and is maximal in the tubular aorta (0.42 ± 0.6 and 0.49 ± 0.5 mm/year).
BAV have a high heritability with about one quarter with bicuspid aortic valve found in the first
degree relatives. The aortic root dilatation is found in about one third in first degree relatives.
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
• In case of BAV, surgery is indicated when the aortic root or thoracic aorta diameter is >55 mm,
>50 mm in presence of other risk factors, >45 mm when surgical aortic valve replacement is scheduled
(Class I C).
• Because of the familial occurence screening of first degree relatives should be considered
(Class IIa C).
10. Future developments
The installation of hybrid rooms have been shown to be pacemakers for the development of new
diagnostic and treatment options like thoracic endovascular aortic repair, debranching or aortic
arch surgery, and the frozen elephant trunk. This can lead in the future to further paradigma
changes in the diagnosis and treatment of aortic diseases.
It is time to form aortic teams and centers in order to provide full access to experts in the field
of cardiology, radiology, pediatric cardiology, genetics, aortic and cardiovascular surgery, which is
needed not only for the acute but also for the intense follow-up of patients with aortic diseases.
Take home messages
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
• The 2014 ESC Guidelines on diagnosis and treatment of aortic diseases contains in 118
recommendations 4% Class I A recommendations, and in 15% Class I-II B recommendations.
Thus, 80% are consensus decision.
• We need more epidemiological data on acute aortic syndrome in Europe.
• More evidence needed on the caseload-outcome relationship in the field aortic diseases.
• The implantation and efficacy of aortic centers in Europe should be assessed. The establishment
of a European network of aortic centers should be encouraged along with establishment of
large registries and multicenter studies.
• The value of biomarkers should be clarified.
• More data of accuracy and reproducibility of aortic measurements are needed.
• The knowledge on relationship between aortic size and outcome should be improved and the
superiority of 3D on 2D better documented.
It has to been investigated, if there are difference of aortic diameters related to age, gender and
body-size taken into account outcome of interventions.
• Data for female patients with aortic diseases are lacking.
• The lack of evidence on the efficacy of medical therapy and the role of antihypertensive drugs,
statins, inflammatory drugs an non-syndromic aortic diseases like aortic dissection, thoracic aortic
aneurysms and AAA, genetic diseases is present.
• For thoracic aortic aneurysms, randomized studies are needed and the optimal timing for
preventive intervention according to lesion size and other characteristics as well as individual
patient features.
• The optimal timing and techniques of intervention in chronic aortic dissection is still unclear.
Major gaps in evidence
ESSENTIAL MESSAGES FROM THE 2014 ESC GUIDELINES ON DIAGNOSIS AND TREATMENT OF AORTIC DISEASES
For more information
www.escardio.org/guidelines
©2014 The European Society of Cardiology
No part of these Pocket Guidelines may be translated or reproduced in any form without written permission from the ESC.
The following material was Adapted from the 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases
((European Heart Journal (2014) 35, 2873–2926 - doi: 10.1093/eurheartj/ehu281).
To read the full report as published by the European Society of Cardiology, visit our Web Site at:
www.escardio.org/guidelines
Copyright © European Society of Cardiology 2014 - All Rights Reserved.
The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial
use is authorized. No part of the ESC Guidelines may be translated or reproduced in any form without written permission from the ESC.
Permission can be obtained upon submission of a written request to ESC, Practice Guidelines Department, Les Templiers - 2035 route des colles
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the evidence available at the time of their dating.
The ESC is not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC Guidelines and any other official
recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of health care or therapeutic
strategies. Health professionals are encouraged to take the ESC Guidelines fully into account when exercising their clinical judgment as well as
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EUROPEAN SOCIETY OF CARDIOLOGY
LES TEMPLIERS
2035 ROUTE DES COLLES
CS 80179 BIOT
06903 SOPHIA ANTIPOLIS CEDEX - FRANCE
PHONE: +33 (0)4 92 94 76 00
FAX: +33 (0)4 92 94 76 01
E-mail: guidelines@escardio.org
EUROPEAN SOCIETY OF CARDIOLOGY
LES TEMPLIERS
2035 ROUTE DES COLLES
CS 80179 BIOT
06903 SOPHIA ANTIPOLIS CEDEX - FRANCE
PHONE: +33 (0)4 92 94 76 00
FAX: +33 (0)4 92 94 76 01
E-mail: guidelines@escardio.org
For more information
www.escardio.org/guidelines
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