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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

CS 80179 Biot - 06903 Sophia Antipolis Cedex - France. Email: guidelines@escardio.org

Disclaimer:

The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge and 

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 

in the determination and the implementation of preventive, diagnostic or therapeutic medical strategies. However, the ESC Guidelines do not 

override in any way whatsoever the individual responsibility of health professionals to make appropriate and accurate decisions in consideration 

of each patient’s health condition and in consultation with that patient and the patient’s caregiver where appropriate and/or necessary. Nor do 

the ESC Guidelines exempt health professionals from taking careful and full consideration of the relevant official updated recommendations or 

guidelines issued by the competent public health authorities in order to manage each patient’s case in light of the scientifically accepted data 

pursuant to their respective ethical and professional obligations. It is also the health professional’s responsibility to verify the applicable rules and 

regulations relating to drugs and medical devices at the time of prescription.

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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