Summary of the 2014 ESC Guidelines on the diagnosis and ... · Guidelines Summary of the 2014 ESC...

Guidelines

Summary of the 2014 ESC Guidelines on thediagnosis and treatment of aortic diseasesPrepared by the Czech Society of Cardiology§

Josef Šťásek a, Petr Němec b, Jiří Vítovec c,*a 1st Department of Internal Medicine – Cardioangiology, Faculty Hospital Hradec Králové and Faculty of Medicine,Charles University, Hradec Kralove, Czech RepublicbCentre of Cardiovascular Surgery and Transplantation, Brno, Czech Republicc1st Internal Cardioangiology Department, Faculty Hospital St. Anne's and Faculty of Medicine, Masaryk University,Brno, Czech Republic

Authors of the original ESC full text document [1,2]: Raimund Erbel, Victor Aboyans onbehalf of the ESC Task Force for the Diagnosis and Treatment of Aortic Diseases

Contents

1 Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3002 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e300

c o r e t v a s a 5 7 ( 2 0 1 5 ) e 2 9 7 – e 3 1 9

a r t i c l e i n f o

Article history:

Received 17 February 2015

Received in revised form

23 April 2015

Accepted 4 May 2015

Available online 29 May 2015

Keywords:

Guidelines

Aortic diseases

Aortic aneurysm

Acute aortic syndrome

Aortic dissection

Abdominal aortic aneurysm

Endovascular therapy

Vascular surgery

§ For permissions: please e-mail: [email protected].* Corresponding author . Tel.: +420 543182204; fax: +420 543182205.

E-mail address: [email protected] (J. Vítovec).

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3 The normal and the ageing aorta. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3004 Assessment of the aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e300

4.1 Clinical examination. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3004.2 Laboratory testing. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3014.3 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e301

4.3.1 Chest X-ray . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3014.3.2 Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3014.3.3 Computed tomography (CT) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3024.3.4 Positron emission tomography/computed tomography (PET/CT) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3024.3.5 Magnetic resonance imaging. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3024.3.6 Aortography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3024.3.7 Intravascular ultrasound (IVUS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e302

4.4 Assessment of aortic stiffness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035 Treatment options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e303

5.1 Principles of medical therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.2 Endovascular therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e303

5.2.1 Thoracic endovascular aortic repair (TEVAR) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.2.2 Abdominal endovascular aortic repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e303

5.3 Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.3.1 Ascending aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.3.2 Aortic arch . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.3.3 Descending aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.3.4 Thoraco-abdominal aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3035.3.5 Abdominal aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e305

6 Acute thoracic aortic syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.2 Pathology and classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.3 Acute aortic dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e305

6.3.1 Definition and classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.3.2 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.3.3 Clinical presentation and complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3056.3.4 Laboratory testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3066.3.5 Diagnostic imaging in acute aortic dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3066.3.6 Diagnostic work-up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3076.3.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e308

6.4 Intramural haematoma (IMH) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3096.4.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3096.4.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.4.3 Natural history, morphological changes, and complications. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.4.4 Indications for surgery and thoracic endovascular aortic repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e310

6.5 Penetrating aortic ulcer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.5.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.5.2 Diagnostic imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.5.3 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3106.5.4 Interventional therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e310

6.6 Aortic pseudoaneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.7 (Contained) rupture of aortic aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e311

6.7.1 Contained rupture of thoracic aortic aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8 Traumatic aortic injury. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e311

6.8.1 Definition, epidemiology and classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.2 Patient presentation and diagnosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.3 Indications for treatment in traumatic aortic injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.4 Medical therapy in traumatic aortic injury. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.5 Surgery in traumatic aortic injury. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.6 Endovascular therapy in traumatic aortic injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3116.8.7 Long-term surveillance in traumatic aortic injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e311

6.9 Iatrogenic aortic dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3117 Aortic aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e312

7.1 Thoracic aortic aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3127.1.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e312

c o r e t v a s a 5 7 ( 2 0 1 5 ) e 2 9 7 – e 3 1 9e298

7.1.2 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3127.1.3 Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3127.1.4 Interventions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e312

7.2 Abdominal aortic aneurysm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.2 Risk factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.3 Natural history. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.4 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.5 Management of small abdominal aortic aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3137.2.6 Abdominal aortic aneurysm repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3147.2.7 (Contained) rupture of abdominal aortic aneurysm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3147.2.8 Long-term prognosis and follow-up of aortic aneurysm repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e315

8 Genetic diseases affecting the aorta. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1 Chromosomal and inherited syndromic thoracic aortic aneurysms and dissection . . . . . . . . . . . . . . . . . . . . . e315

8.1.1 Turner syndrome (TS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.2 Marfan syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.3 Ehlers-Danlos syndrome Type IV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.4 Loeys-Dietz syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.5 Arterial tortuosity syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.6 Aneurysms-osteoarthritis syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.7 Non-syndromic familial thoracic aortic aneurysms and dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.1.8 Genetics and heritability of abdominal aortic aneurysm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e315

8.2 Aortic diseases associated with bicuspid aortic valve. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.2.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3158.2.2 Natural history. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.2.3 Pathophysiology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.2.4 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.2.5 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.2.6 Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e316

8.3 Coarctation of the aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.3.1 Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.3.2 Diagnostic work-up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3168.3.3 Surgical or catheter interventional treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e317

9 Atherosclerotic lesions of the aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.1 Thromboembolic aortic disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e317

9.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.1.3 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e317

9.2 Mobile aortic thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.3 Atherosclerotic aortic occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.4 Calcified aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e3179.5 Coral reef aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e317

10 Aortitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e31710.1 Definition, types, and diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e317

10.1.1 Giant cell arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e31810.1.2 Takayasu arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e318

10.2 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e33011 Aortic tumours . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e318

11.1 Primary malignant tumours of the aorta. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e31812 Long-term follow-up of aortic diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e318

12.1 Chronic aortic dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e31812.2 Follow-up after thoracic aortic intervention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e31812.3 Follow-up of patients after intervention for abdominal aortic aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e318

13 Gaps in evidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e319References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e319

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1 Preamble

Guidelines and recommendations should help the healthprofessionals to make decisions in their daily practice.However, the final decisions concerning an individualpatient must be made by the responsible health profession-al(s) in consultation with the patient and caregiver asappropriate.

2 Introduction

Aortic diseases contribute to the wide spectrum of arterialdiseases: aortic aneurysms (AA), acute aortic syndromes (AAS)including aortic dissection (AD), intramural haematoma (IMH),penetrating atherosclerotic ulcer (PAU) and traumatic aorticinjury (TAI), pseudoaneurysm, aortic rupture, atheroscleroticand inflammatory affections, genetic diseases (e.g. Marfansyndrome) and congenital abnormalities including the coarc-tation of the aorta (CoA).

Fig. 1 – Segments of the ascending and desce

3 The normal and the ageing aorta

The aorta is the ultimate conduit, carrying, in an averagelifetime, almost 200 million litres of blood to the body. It isdivided by the diaphragm into the thoracic and abdominalaorta (Fig. 1). In healthy adults, aortic diameters do not usuallyexceed 40 mm and taper gradually downstream. In this regard,the rate of aortic expansion is about 0.9 mm in men and0.7 mm in women for each decade of life.

4 Assessment of the aorta

4.1 Clinical examination

While aortic diseases may be clinically silent in many cases, abroad range of symptoms may be related to different aorticdiseases. In some situations, physical examination can bedirected by the symptoms and includes palpation andauscultation of the abdomen and flank in the search for

nding aorta. rPA, right pulmonary artery.

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prominent arterial pulsations or turbulent blood flow causingmurmurs.

4.2 Laboratory testing

Baseline laboratory assessment includes cardiovascularrisk factors. Laboratory testing plays a minor role in thediagnosis of acute aortic diseases but is useful for differentialdiagnoses.

4.3 Imaging

The aorta is a complex geometric structure and severalmeasurements are useful to characterize its shape andsize. Diameter measurements should be made perpendicularto the axis of flow of the aorta (Fig. 2). Standardized measure-ments will help to better assess changes in aortic size overtime.

Fig. 2 – Thoracic and abdominal aorta in a three-dimensional rereconstruction (MPR) along the centreline (left middle part), straigI) (right side), orthogonal to the centreline orientated cross-sectioreport aortic diameters: (A) sinuses of Valsalva; (B) sinotubular juaortic arch (aorta at the origin of the brachiocephalic trunk); (E) marteries); (F) proximal descending thoracic aorta (approximately

aorta (level of the pulmonary arteries as easily identifiable landorigin; (J) right before aortic bifurcation.Provided by F Nensa, Institute of Diagnostic and Interventional

4.3.1 Chest X-rayChest X-ray obtained for other indications may detectabnormalities of aortic contour or size. Chest X-ray is,however, only of limited value for diagnosing an AAS.

4.3.2 Ultrasound4.3.2.1 Transthoracic echocardiography (TTE). Echocardio-graphic evaluation of the aorta is a routine part of the standardechocardiographic examination. Transthoracic echocardiogra-phy is an excellent imaging modality for serial measurement ofmaximal aortic root diameters for evaluation of aortic regurgi-tation, and timing for elective surgery in cases of TAA. TTE oftensuffices for screening. Aortic arch aneurysm, plaque calcifica-tion, thrombus, or a dissection membrane may be detectable ifimage quality is adequate. Aortic coarctation can be suspectedby continuous-wave Doppler; a patent ductus arteriosus may alsobe identifiable by colour Doppler. The lower abdominal aorta,below the renal arteries, can be visualized to rule out AAA.

construction (left lateral image), parasagitale multiplanarhtened-MPR along the centreline with given landmarks (A–

ns at the landmarks (A–J). Landmarks A–J should be used tonction; (C) mid ascending aorta (as indicated); (D) proximalid aortic arch (between left common carotid and subclavian2 cm distal to left subclavian artery); (G) mid descendingmarks, as indicated); (H) at diaphragm; (I) at the celiac axis

Radiology, Essen.

Recommendations on imaging of the aorta.

Recommendations Classa Levelb

It is recommended that diameters bemeasured at pre-specified anatomicallandmarks, perpendicular to thelongitudinal axis.

I C

In the case of repetitive imaging of the aortaover time, to assess change in diameter, itis recommended that the imagingmodality with the lowest iatrogenic risk beused.

I C

In the case of repetitive imaging of the aortaover time to assess change in diameter, itis recommended that the same imagingmodality be used, with a similar methodof measurement.

I C

It is recommended that all relevant aorticdiameters and abnormalities be reportedaccording to the aortic segmentation.

I C

It is recommended that renal function,pregnancy, and history of allergy tocontrast media be assessed, in order toselect the optimal imaging modality of theaorta with minimal radiation exposure,except for emergency cases.

I C

The risk of radiation exposure should beassessed, especially in younger adults andin those undergoing repetitive imaging.

IIa B

Aortic diameters may be indexed to thebody surface area, especially for theoutliers in body size.

IIb B

a Class of recommendation.b Level of evidence.

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4.3.2.2 Transoesophageal echocardiography (TOE). The rela-tive proximity of the oesophagus and the thoracic aortapermits high-resolution images with higher-frequency. Also,multi-plane imaging permits improved assessment of theaorta from its root to the descending aorta. Real-time 3D TOEappears to offer some advantages over 2D TOE.

4.3.2.3 Abdominal ultrasound. Duplex ultrasound providesadditional information on aortic flow. Colour Doppler is ofgreat interest in the case of abdominal aorta dissection, todetect perfusion of both false and true lumen and potential re-entry sites or obstruction of tributaries (e.g. the iliac arteries).Contrast-enhanced ultrasound is useful in detecting, localiz-ing, and quantifying endoleaks when this technique is used tofollow patients after EVAR.

4.3.3 Computed tomography (CT)Computed tomography plays a central role in the diagnosis,risk stratification, and management of aortic diseases (Fig. 2).CT allows detection of the location of the diseased segment,the maximal diameter of dilation, the presence of atheroma,thrombus, IMH, penetrating ulcers, calcifications and, inselected cases, the extension of the disease to the aorticbranches. In most patients with suspected AD, CT is thepreferred initial imaging modality. Similar diagnostic accuracyhas been reported for detecting traumatic aortic injury. Otherfeatures of AAS, such as penetrating ulcers, thrombus, pseudo-aneurysm, and rupture, are readily depicted by CT.

4.3.4 Positron emission tomography/computed tomography(PET/CT)PET/CT imaging is based on the distribution of the F-fluorodeoxyglucose (FDG), which is taken up with high affinityby hypermetabolic cells, and can be used to detect vascularinflammation in large vessels. The advantages of PET may becombined with CT imaging with good resolution.

4.3.5 Magnetic resonance imagingThe salient features necessary for clinical decision-making,such as maximal aortic diameter, shape and extent of theaorta, involvement of aortic branches in aneurysmal dilationor dissection, relationship to adjacent structures, and pres-ence of mural thrombus, are reliably depicted by MRI.

Table 1 – Comparison of methods for imaging the aorta.

Advantages/disadvantages TTE TOE

Ease of use +++ ++

Diagnostic reliability + +++

Bedside/interventional usea ++ ++

Serial examinations ++ +

Aortic wall visualizationc + +++

Cost � �

Radiation 0 0

Nephrotoxicity 0 0

+ means a positive remark and � means a negative remark. The numbea IVUS can be used to guide interventions (see web addenda).b +++ only for follow-up after aortic stenting (metallic struts), otherwisec PET can be used to visualize suspected aortic inflammatory disease.CT = computed tomography; MRI = magnetic resonance imaging; TOE = tran

4.3.6 AortographyCatheter-based invasive aortography visualizes the aorticlumen, side branches, and collaterals. As a luminographytechnique, angiography provides exact information about theshape and size of the aorta, as well as any anomalies, althoughdiseases of the aortic wall itself are missed, as well asthrombus-filled discrete aortic aneurysms (Table 1).

4.3.7 Intravascular ultrasound (IVUS)To optimize visualization of the aortic wall, IVUS can be used,particularly during endovascular treatment.

CT MRI Aortography

+++ ++ ++++ +++ ++� � ++++(+)b +++ �+++ +++ ��

r of signs indicates the estimated potential value.

limit radiation.

soesophageal echocardiography; TTE = transthoracic echocardiography.

Recommendation for (thoracic) endovascular aortic repair((T)EVAR).


It is recommended that the indication forTEVAR or EVAR be decided on an individualbasis, according to anatomy, pathology,comorbidity and anticipated durability, ofany repair, using a multidisciplinaryapproach.

I C

A sufficient proximal and distal landing zone ofat least 2 cm is recommended for the safedeployment and durable fixation of TEVAR.

I C

In case of aortic aneurysm, it is recommendedto select a stent-graft with a diameterexceeding the diameter of the landing zonesby at least 10–15% of the reference aorta.

I C

During stent graft placement, invasive bloodpressure monitoring and control (eitherpharmacologically or by rapid pacing) isrecommended.

I C

Preventive cerebrospinal fluid (CSF) drainageshould be considered in high-risk patients.

IIa C


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4.4 Assessment of aortic stiffness

Aortic stiffness has independent predictive value for all-causeand cardiovascular mortality, fatal and non-fatal coronaryevents, and fatal strokes in patients with various levels ofcardiovascular risk, with a higher predictive value in subjectswith a higher baseline cardiovascular risk. Carotid-femoralpulse wave velocity is the 'gold standard' for measuring aorticstiffness.

5 Treatment options

5.1 Principles of medical therapy

The main aim of medical therapy in this condition is toreduce shear stress on the diseased segment of the aorta byreducing blood pressure and cardiac contractility. In casesof AD, treatment with intravenous beta-blocking agentsis initiated to reduce the heart rate and lower the systolicblood pressure to 100–120 mm Hg. Other agents may beuseful in achieving the target. In chronic conditions, bloodpressure should be controlled below 140/90 mm Hg. Use ofstatins has been associated with improved survival afterAAA repair.

5.2 Endovascular therapy

5.2.1 Thoracic endovascular aortic repair (TEVAR)5.2.1.1 Technique. Thoracic endovascular aortic repair aims atexcluding an aortic lesion from the circulation by theimplantation of a membrane-covered stent-graft across thelesion. Contrast-enhanced CT represents the imaging modali-ty of choice for planning TEVAR. The diameter (<40 mm) andlength (≥20 mm) of the healthy proximal and distal landingzones are evaluated to assess the feasibility of TEVAR. Insituations involving important aortic arch side branchesTEVAR is often preceded by limited surgical revascularizationof these branches.

5.2.1.2 Complications. In TEVAR, vascular complications atthe puncture site, as well as aortic and neurological complica-tions, and/or endoleaks have been reported. Different types ofendoleaks are illustrated in Fig. 3.

5.2.2 Abdominal endovascular aortic repair5.2.2.1 Technique. Endovascular aortic repair is performed toprevent infrarenal AAA rupture. The proximal aortic neckshould have a length of at least 10–15 mm and should notexceed 32 mm in diameter. Angulation above 608 of theproximal neck increases the risk of device migration andendoleak. Aneurysmal disease of the iliac arteries needsextension of the stent graft to the external iliac artery.

5.2.2.2 Complications. Immediate conversion to open surgeryis required in approximately 0.6% of patients. Endoleak is themost common complication of EVAR. Type I and Type IIIendoleaks demand correction while Type II endoleak may sealspontaneously in about 50% of cases.

5.3 Surgery

5.3.1 Ascending aortaIf the aneurysm is proximally limited to the sinotubularjunction and distally to the aortic arch, resection of theaneurysm and supra-commissural implantation of a tubulargraft is performed. If the aneurysm extends proximally belowthe sinotubular junction and one or more aortic sinuses aredilated, the surgical repair is guided by the extent ofinvolvement of the aortic annulus and the aortic valve.Reconstructive aortic root surgery, preserving the tricuspidvalve, aims for restoration of natural haemodynamics. If thereis any doubt that a durable repair can be achieved—or in thepresence of aortic sclerosis or stenosis—root replacementshould be performed with either a mechanical composite graftor a xenograft, according to the patient's age and potentialcontraindications for long-term anticoagulation.

5.3.2 Aortic archThe continuous use of antegrade cerebral perfusion hasproven itself as safe cerebral protection. This is the case forthe majority of reconstructions, including acute and chronicAD, requiring total arch replacement and arrest times from 40to 60 min.

5.3.3 Descending aortaEstablished methods for operation of the descending aortainclude the left heart bypass technique, the partial bypass,and the operation in deep hypothermic circulatory arrest.At a core temperature of 18 8C the proximal anastomosis isperformed.

5.3.4 Thoraco-abdominal aortaWhen the disease affects both the descending thoracic andabdominal aorta, the surgical approach is a left thoracotomyextended to paramedian laparotomy.

Fig. 3 – Classification of endoleaks. Type I: Leak at graft attachment site above, below, or between graft components(Ia: proximal attachment site; Ib: distal attachment site). Type II: Aneurysm sac filling retrogradely via single (IIa) or multiplebranch vessels (IIb). Type III: Leak through mechanical defect in graft, mechanical failure of the stent-graft by junctionalseparation of the modular components (IIIa), or fractures or holes in the endograft (IIIb). Type IV: Leak through graft fabric asa result of graft porosity. Type V: Continued expansion of aneurysm sac without demonstrable leak on imaging(endotension, controversial).Modified from White GH, May J, Petrasek P. Semin Interv Cardiol. 2000;5:35–46.

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5.3.5 Abdominal aortaThe aneurysmal aorta is replaced either by a tube or bifurcatedgraft, according to the extent of aneurysmal disease into theiliac arteries. The excluded aneurysm is not resected, but isclosed over the graft.

Recommendations for surgical techniques in aortic dis-ease.


Cerebrospinal fluid drainage is recommendedin surgery of the thoraco-abdominal aorta, toreduce the risk of paraplegia.

I B

Aortic valve repair, using the re-implantationtechnique or remodelling with aorticannuloplasty, is recommended in youngpatients with aortic root dilation andtricuspid aortic valves.

I C

For repair of acute Type A AD, an open distalanastomotic technique avoiding aorticclamping (hemiarch/complete arch) isrecommended.

I C

In patients with connective tissue disordersc

requiring aortic surgery, the replacement ofaortic sinuses is indicated.

I C

Selective antegrade cerebral perfusion shouldbe considered in aortic arch surgery, toreduce the risk of stroke.

IIa B

The axillary artery should be considered as firstchoice for cannulation for surgery of theaortic arch and in aortic dissection.

IIa C

Left heart bypass should be considered duringrepair of the descending aorta or the thoraco-abdominal aorta, to ensure distal organperfusion.

IIa C

a Class of recommendation.b Level of evidence.c Ehlers-Danlos IV, Marfan- or Loeys-Dietz syndromes.

Table 2 – Main clinical presentations and complications ofpatients with acute aortic dissection.

Type A Type B

Chest pain 80% 70%Back pain 40% 70%Abrupt onset of pain 85% 85%Migrating pain <15% 20%Aortic regurgitation 40–75% N/ACardiac tamponade <20% N/AMyocardial ischaemia or infarction 10–15% 10%Heart failure <10% <5%Pleural effusion 15% 20%Syncope 15% <5%Major neurological deficit (coma/stroke) <10% <5%Spinal cord injury <1% NRMesenteric ischaemia <5% NRAcute renal ischaemia <20% 10%Lower limb ischaemia <10% <10%

NR = not reported; NA = not applicable. Percentages are approxi-mated.

6 Acute thoracic aortic syndromes

6.1 Definition

Acute aortic syndromes are defined as emergency conditionswith similar clinical characteristics involving the aorta. Thismay result in IMH, PAU, or in separation of aortic wall layers,leading to AD or even thoracic aortic rupture.

6.2 Pathology and classification

Acute aortic syndromes occur when either a tear or an ulcerallows blood to penetrate from the aortic lumen into the mediaor when a rupture of vasa vasorum causes a bleed within themedia. Fig. 4 displays the Stanford and the DeBakeyclassifications. The most common features of AAS aredisplayed in Fig. 5.

6.3 Acute aortic dissection

6.3.1 Definition and classificationAortic dissection is defined as disruption of the medial layerprovoked by intramural bleeding, resulting in separation of the

aortic wall layers and subsequent formation of a TL and an FLwith or without communication. This process is followedeither by an aortic rupture in the case of adventitial disruptionor by a re-entering into the aortic lumen through a secondintimal tear. Other complications include tamponade, aorticvalve regurgitation, and proximal or distal malperfusionsyndromes.

6.3.2 EpidemiologyUp-to-date data on the epidemiology of AD are scarce. Thisincidence is higher in men than in women and increases withage. The prognosis is poorer in women, as a result of atypicalpresentation and delayed diagnosis.

6.3.3 Clinical presentation and complications6.3.3.1 Chest pain. Abrupt onset of severe chest and/or backpain is the most typical feature. The pain may be sharp,ripping, tearing, knife-like, and typically different from othercauses of chest pain. Cardiac complications are the mostfrequent in patients with AD (Table 2).

6.3.3.2 Aortic regurgitation. In AD includes dilation of theaortic root and annulus, tearing of valve cusps, downwarddisplacement of one cusp below the line of the valve closureand loss of support of the cusp.

6.3.3.3 Myocardial ischaemia. Myocardial ischaemia or infarc-tion may be present in 10–15% of patients with AD.

6.3.3.4 Congestive heart failure. Congestive heart failure in thesetting of AD is commonly related to aortic regurgitation.Hypotension and shock may result from aortic rupture, acutesevere aortic regurgitation, extensive myocardial ischaemia,cardiac tamponade, or major blood loss.

6.3.3.5 Large pleural effusions. Large pleural effusions result-ing from aortic bleeding into the mediastinum and pleural

Fig. 4 – Classification of aortic dissection localization. Schematic drawing of aortic dissection class 1, subdivided into DeBakeyTypes I, II, and III. Also depicted are Stanford classes A and B. Type III is differentiated in subtypes III A to III C (sub-typedepends on the thoracic or abdominal involvement according to Reul et al.).

Table 3 – Laboratory tests required for patients with acuteaortic dissection.

Laboratory tests To detect signs of

Red blood cell count Blood loss, bleeding, anaemiaWhite blood cell count Infection, inflammation (SIRS)C-reactive protein Inflammatory responseProCalcitonin Differential diagnosis between

SIRS and sepsisCreatine kinase Reperfusion injury,

rhabdomyolysisTroponin I or T Myocardial ischaemic, myocardial

infarctionD-Dimer Aortic dissection, pulmonary

embolism, thrombosisCreatinine Renal failure (existing or

developing)Aspartate transaminase/alanine aminotransferase

Liver ischaemia, liver disease

Lactate Bowel ischaemia, metabolicdisorder

Glucose Diabetes mellitusBlood gases Metabolic disorder, oxygenation

SIRS = systemic inflammatory response syndrome.

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space are rare, because these patients usually do not surviveup to arrival at hospital.

6.3.3.6 Pulmonary complications. Pulmonary complications ofacute AD are rare.

6.3.3.7 Syncope. Syncope is an important initial symptom ofAD!!!

6.3.3.8 Neurological symptoms. Neurological symptoms mayoften be dramatic and dominate the clinical picture, maskingthe underlying condition.

6.3.3.9 Mesenteric ischaemia. The perfusion disturbance canbe intermittent if caused by a dissection flap prolapse, orpersistent in cases of obliteration of the organ arterial supplyby FL expansion.

6.3.3.10 Renal failure. Renal failure may be encountered atpresentation or during hospital course in up to 20% of patientswith acute Type A AD and in approximately 10% of patientswith Type B AD.

6.3.4 Laboratory testingIf D-dimers are elevated, the suspicion of AD is increased. Thelevel of D-dimers is immediately very high (Table 3).

6.3.5 Diagnostic imaging in acute aortic dissectionThe main purpose of imaging in AAD is the comprehensiveassessment of the entire aorta. Computed tomography, MRI,

Fig. 5 – Classification of acute aortic syndrome in aortic dissection. Class 1: Classic AD with true and FL with or withoutcommunication between the two lumina. Class 2: Intramural haematoma. Class 3: Subtle or discrete AD with bulging of theaortic wall. Class 4: Ulceration of aortic plaque following plaque rupture. Class 5: Iatrogenic or traumatic AD, illustrated by acatheter induced separation of the intima.

Recommendations on diagnostic work-up of acute aorticsyndrome.


History and clinical assessmentIn all patients with suspected AAS, pre-testprobability assessment is recommended,according to the patient's condition,symptoms, and clinical features.

I B

Laboratory testingIn case of suspicion of AAS, the interpretation ofbiomarkers should always be considered alongwith the pretest clinical probability.

IIa C

In case of low clinical probability of AAS,negative D-dimer levels should be consideredas ruling out the diagnosis.

IIa B

In case of intermediate clinical probability of AASwith a positive (point-of-care) D-dimer test,further imaging tests should be considered.

IIa B

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and TOE are equally reliable for confirming or excluding thediagnosis of AAD (Table 4).

6.3.5.1 Echocardiography. The diagnosis of AD by standardtransthoracic M-mode and two-dimensional echocardiogra-phy is based on detecting intimal flaps in the aorta. Limitationsof the TTE have been overcome by TOE.

6.3.5.2 Computed tomography. The key finding on contrast-enhanced images is the intimal flap separating two lumens.‘‘Triple-rule out’’ is a relatively new term that describes an ECG-gated 64-detector CT study to evaluate patients with acute chestpain, in the emergency department, for three potential causes:AD, pulmonary embolism, and coronary artery disease.

6.3.5.3 Magnetic resonance imaging. MRI is considered theleading technique for diagnosis of AD, with a reportedsensitivity and specificity of 98%. MRI is also very useful fordetecting the presence of pericardial effusion, aortic regurgi-tation, or carotid artery dissection.

6.3.5.4 Aortography. The angiographic diagnosis of AD isbased on 'direct' angiographic signs, such as the visualizationof the intimal flap.

6.3.6 Diagnostic work-upThe diagnostic work-up to confirm or to rule out AD is highlydependent on the a priori risk of this condition. The diagnosticflow chart combines the pre-test probabilities according toclinical data, and the laboratory and imaging tests (Table 5 andFig. 6).

(Continued )


In patients with high probability (risk score 2 or 3)of AD, testing of D-dimers is not recommended.

III C

ImagingTTE is recommended as an initial imaginginvestigation.

I C

In unstablec patients with a suspicion of AAS, thefollowing imaging modalities are recommendedaccording to local availability and expertise:

� TOE I C� CT I CIn stable patients with a suspicion of AAS, thefollowing imaging modalities arerecommended (or should be considered)according to local availability and expertise:

� CT I C� MRI I C� TOE IIa CIn case of initially negative imaging withpersistence of suspicion of AAS, repetitiveimaging (CT or MRI) is recommended.

I C

Chest X-ray may be considered in cases of lowclinical probability of AAS.

IIb C

In case of uncomplicated Type B AD treatedmedically, repeated imaging (CT or MRI)d

during the first days is recommended.

I C

a Class of recommendation.b Level of evidence.c Unstable means very severe pain, tachycardia, tachypnoea,hypotension, cyanosis, and/or shock.d Preferably MRI in young patients, to limit radiation exposure.AAS = abdominal aortic aneurysm; AD = aortic dissection;CT = computed tomography; MRI = magnetic resonance imaging;TOE = transoesophageal echocardiography; TTE = transthoracicechocardiography.

Table 4 – Details required from imaging in acute aorticdissection.

Aortic dissectionVisualization of intimal flapExtent of the disease according to aortic anatomic segmentationIdentification of the false and true lumens (if present)Localization of entry and re-entry teats (if present)Identification of antegrade and/or retrograde aortic dissectionIdentification grading, and mechanism of aortic valve regurgitationInvolvement of side branchesDetection of malperfusion (low flow or no flow)Detection of organ ischaemia (brain, myocardium, bowel,kidneys, etc.)

Detection of pericardial effusion and its severityDetection and extent of pleural effusionDetection of peri-aortic bleedingSigns of mediastinal bleeding

Intramural haematomaLocalization and extent of aortic arch thickeningCo-existence of atheromatous disease (calcium shift)Presence of small intimal tears

Penetrating aortic ulcerLocalization of the lesion (length and depth)Co-existence of intramural haematomaInvolvement of the peri-aortic tissue and bleedingThickness of the residual wall

In all casesCo-existence of other aortic lesions: aneurysms, plaques, signs ofinflammatory disease, etc.

Recommendations for treatment of aortic dissection.


In all patients with AD, medical therapy includingpain relief and blood pressure control isrecommended.

I C

In patients with Type A AD, urgent surgery isrecommended.

I B

In patients with acute Type A AD and organmalperfusion, a hybrid approach (i.e. ascendingaorta and/or arch replacement associated withany percutaneous aortic or branch arteryprocedure) should be considered.

IIa B

In uncomplicated Type B AD, medical therapyshould always be recommended.

I C

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6.3.7 TreatmentWhether or not the patient undergoes any intervention,medical therapy to control pain and the haemodynamic stateis essential.

6.3.7.1 Type A aortic dissection. Surgery is the treatment ofchoice. Acute Type A AD has a mortality of 50% within the first48 h if not operated. Despite improvements in surgical andanaesthetic techniques, perioperative mortality (25%) andneurological complications (18%) remain high. Although com-monly associated with a poor post-operative prognosis, recov-ery has been reported when rapid brain reperfusion is achieved,especially if the time between symptom onset and arrival at theoperating room is <5 h.

6.3.7.2 Type B aortic dissection. The course of Type B AD isoften uncomplicated so—in the absence of malperfusion orsigns of disease progression—the patient can be safely stabilizedunder medical therapy alone, to control pain and blood pressure.

6.3.7.2.1 Uncomplicated Type B aortic dissection. 6.3.7.2.1.1Medical therapy. Patients with uncomplicated Type B ADreceive medical therapy to control pain, heart rate, and bloodpressure, with close surveillance to identify signs of diseaseprogression and/or malperfusion.6.3.7.2.1.2 Thoracic endovascular aortic repair. Thoracic endo-vascular aortic repair (TEVAR) aims at stabilization of thedissected aorta, to prevent late complications by inducing

aortic remodelling processes. Obliterating the proximal inti-mal tear by implantation of a membrane-covered stent-graftredirects blood flow to the TL, thus improving distal perfusion.

6.3.7.2.2 Complicated Type B aortic dissection: endovasculartherapy. 6.3.7.2.2.1 Thoracic endovascular aortic repair(TEVAR). Thoracic endovascular aortic repair (TEVAR) is thetreatment of choice in complicated acute Type B AD. The term'complicated' means persistent or recurrent pain, uncontrolledhypertension, early aortic expansion, malperfusion, and signsof rupture.

There is increasing evidence that TEVAR shows a signifi-cant advantage over open surgery in patients with acutecomplicated Type B AD.6.3.7.2.2.2 Surgery. Lower extremities artery disease, severetortuosity of the iliac arteries, a sharp angulation of the aorticarch, and the absence of a proximal landing zone for the stentgraft are factors that indicate open surgery for the treatment ofacute complicated Type B AD.

Fig. 6 – Flowchart for decision-making based on pre-test sensitianeurysm; AD, aortic dissection; CT, computed tomography; MRechocardiography; TTE, transthoracic echocardiography.

Table 5 – Clinical data useful to assess the a priori probability

High-risk conditions High-risk pain featur

Marfan syndrome (or other connectivetissue diseases)

Chest, back, or abdominal

described as any of the follFamily history of aortic disease - abrupt onset

Known aortic valve disease - severe intensity

Known thoracic aortic aneurysm - ripping or tearing

Previous aortic manipulation (includingcardiac surgery)

(Continued )


In uncomplicated Type B AD, TEVAR should beconsidered.

IIa B

n complicated Type B AD, TEVAR is recommended. I CIn complicated Type B AD, surgery may beconsidered.

IIb C

a Class of recommendation.b Level of evidence.AD = aortic dissection; TEVAR = thoracic endovascular aortic repair.

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6.4 Intramural haematoma (IMH)

6.4.1 DefinitionAortic IMH is an entity in which a haematoma develops in themedia of the aortic wall in the absence of an FL. Intramuralhaematoma is diagnosed in the presence of a circular orcrescent-shaped thickening of >5 mm of the aortic wall in theabsence of detectable blood flow.

vity of acute aortic syndrome. AAS, abdominal aorticI, magnetic resonance imaging; TOE, transoesophageal

of acute aortic syndrome.

es High-risk examination features

painowing:

Evidence of perfusion deficit:

- pulse deficit- systolic blood pressure difference- focal neurological deficit (in conjunction with pain)

Aortic systolic murmur (new and with pain)

Hypertension or shock

Table 7 – Diagnostic value of different imaging modalitiesin acute aortic syndromes.

Lesion TTE TOE CT MRI

Ascending aortic dissection ++ +++ +++ +++Aortic arch dissection + + +++ +++Descending aortic dissection + +++ +++ +++Size ++ +++ +++ +++Mural thrombus + +++ +++ +++Intramural haematoma + +++ ++ +++Penetrating aortic ulcer ++ ++ +++ +++Involvement of aortic branches +a (+) +++ +++

+++ = excellent; ++ = moderate; + = poor; (+) = poor and inconstant;CT = computed tomography; MRI = magnetic resonance imaging;TOE = transoesophageal echocardiography; TTE = transthoracicechocardiography.a Can be improved when combined by vascular ultrasound (carotid,subclavian, vertebral, celiac, mesenteric and renal arteries).

Table 6 – Predictors of intramural haematoma complica-tions.

Persistent or recurrent pain despite aggressive medical treatmentDifficult blood pressure controlAscending aortic involvementMaximum aortic diameter ≥50 mmProgressive maximum aortic wall thickness (>11 mm)Enlarging aortic diameterRecurrent pleural effusionPenetrating ulcer or ulcer-like projection secondary to localizeddissections in the involved segment

Detection of organ ischaemia (brain, myocardium, bowel,kidneys, etc.)

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6.4.2 DiagnosisFor the detection of an acute aortic IMH, CT and MRI are theleading techniques for diagnosis and classification of intra-mural haematoma.

6.4.3 Natural history, morphological changes, andcomplicationsThe mortality rates of medically treated patients are high. Thein-hospital mortality of Type A IMH was similar to Type A AD(Table 6).

6.4.4 Indications for surgery and thoracic endovascular aorticrepairTherapeutic management in acute IMH should be similar tothat for AD.

6.4.4.1 Type A intramural haematoma. Emergency surgery isindicated in complicated cases with pericardial effusion,periaortic haematoma, or large aneurysms, and urgent surgeryis required in most of the Type A.

6.4.4.2 Type B intramural haematoma. Medical treatment isthe initial approach to this condition. Endovascular therapy orsurgery would have the same indications as for Type B AD.

Recommendations on the management of intramuralhaematoma.


In all patients with IMH, medical therapyincluding pain relief and blood pressure controlis recommended.

I C

In cases of Type A IMH, urgent surgery is indicated. I CIn cases of Type B IMH, initial medical therapyunder careful surveillance is recommended.

I C

In uncomplicatedc Type B IMH, repetitiveimaging (MRI or CT) is indicated.

I C

In complicatedc Type B IMH, TEVAR should beconsidered.

IIa C

In complicatedc Type B IMH, surgery may beconsidered.

IIb C

a Class of recommendation.b Level of evidence.c Uncomplicated/complicated IMH means absence or presentrecurrent pain, expansion of the IMH, periaortic haematoma,intimal disruption.CT = computed tomography; IMH = intramural haematoma; MRI =magnetic resonance imaging; TEVAR = thoracic endovascular aorticrepair.

6.5 Penetrating aortic ulcer

6.5.1 DefinitionPenetrating aortic ulcer (PAU) is defined as ulceration of anaortic atherosclerotic plaque penetrating through the internalelastic lamina into the media. The natural history of this lesionis characterized by progressive aortic enlargement and devel-opment of saccular or fusiform aneurysms in the ascendingaorta (Type A PAU). The most common location of PAU is themiddle and lower descending thoracic aorta (Type B PAU).

6.5.2 Diagnostic imagingContrast-enhanced CT is the technique of choice for diagnosisof PAU (Table 7).

6.5.3 ManagementIn the presence of AAS related to PAU, the aim of treatment isto prevent aortic rupture and progression to acute AD. It hasbeen suggested that asymptomatic PAUs with diameter>20 mm or neck >10 mm represent a higher risk for diseaseprogression and may be candidates for early intervention.

6.5.4 Interventional therapyThe choice of treatment is commonly based on anatomicalfeatures, clinical presentation, and comorbidities. These aorticlesions represent an ideal anatomical target for stenting.

Recommendations on management of penetrating aorticulcer.


In all patients with PAU, medical therapyincluding pain relief and blood pressurecontrol is recommended.

I C

In the case of Type A PAU, surgery should beconsidered.

IIa C

In the case of Type B PAU, initial medicaltherapy under careful surveillance isrecommended.

I C

In uncomplicated Type B PAU, repetitiveimaging (MRI or CT) is indicated.

I C

In complicated Type B PAU, TEVAR shouldbe considered.

IIa C

Recommendations for traumatic aortic injury.

(Continued )


In complicated Type B PAU, surgery may beconsidered.

IIb C

a Class of recommendation.b Level of evidence.CT = computed tomography; MRI = magnetic resonance imaging;PAU = penetrating aortic ulcer; TEVAR = thoracic endovascularaortic repair.

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6.6 Aortic pseudoaneurysm

Aortic pseudoaneurysm (false aneurysm) is defined as adilation of the aorta due to disruption of all wall layers.Pseudoaneurysms of the thoracic aorta are commonlysecondary to blunt thoracic trauma. Iatrogenic aetiologiesinclude aortic surgery and catheter-based interventions. Inpatients with aortic pseudoaneurysms—if feasible and inde-pendently of size—interventional or open surgical interven-tions are always indicated.

6.7 (Contained) rupture of aortic aneurysm

Contained rupture should be suspected in all patientspresenting with acute pain, in whom imaging detects aorticaneurysm with preserved integrity of the aortic wall.

6.7.1 Contained rupture of thoracic aortic aneurysm6.7.1.1 Clinical presentation. Patients with contained ruptureof a TAA usually present with acute onset of chest and/or backpain. Concurrent abdominal pain may be present in patientswith symptomatic thoraco-abdominal aneurysms.

6.7.1.2 Diagnostic work-up. With the suspicion of (contained)rupture of a TAA, CT is indicated. Contained ruptures of TAAare indications for urgent treatment because of the risk ofimminent internal bleeding and death.

6.7.1.3 Treatment. Contained rupture of TAA is a conditionrequiring urgent treatment. Traditionally, this condition hasbeen treated by open repair, but endovascular repair hasemerged as an alternative treatment option for suitablepatients case by case, depending also on local expertise.

Recommendations for (contained) rupture the thoracicaortic aneurysm.


In patients with suspected rupture of the TAA,emergency CT angiography for diagnosisconfirmation is recommended.

I C

In patients with acute contained rupture ofTAA, urgent repair is recommended.

I C

If the anatomy is favourable and the expertiseavailable, endovascular repair (TEVAR)should be preferred over open surgery.

I C

a Class of recommendation.b Level of evidence.CT = computed tomography; TAA = thoracic aortic aneurysm; TE-VAR = thoracic endovascular aortic repair.

6.8 Traumatic aortic injury

6.8.1 Definition, epidemiology and classificationMost often blunt traumatic thoracic aortic injury (TAI) occursas a consequence of sudden deceleration resulting from head-on or side-impact collisions, or falling from a great height. Aclassification scheme for TAI has been proposed: Type I(intimal tear), Type II (IMH), Type III (pseudoaneurysm), andType IV (rupture).

6.8.2 Patient presentation and diagnosisThe clinical presentation of TAI ranges from minor non-specific symptoms to mediastinal or interscapular pain.Emergency CT should be performed.

6.8.3 Indications for treatment in traumatic aortic injuryPatients with free aortic rupture or large periaortic haematomashould be treated as emergency cases. For all other conditions,the intervention may be delayed for up to 24 h. An initialconservative management, with serial imaging, has beenproposed for patients with minimal aortic injuries (intimaltear/Type I lesions), as most lesions remain stable or resolve.

6.8.4 Medical therapy in traumatic aortic injuryIn polytrauma patients, multidisciplinary management is vitalto establish the correct timing of the interventions andtreatment priorities.

6.8.5 Surgery in traumatic aortic injuryOpen surgical repair of a TAI at the classic isthmus locationrequires exposure of the aorta via a left fourth interspacethoracotomy.

6.8.6 Endovascular therapy in traumatic aortic injuryAvailable data indicate that TEVAR, in suitable anatomies,should be the preferred treatment option in TAI.

6.8.7 Long-term surveillance in traumatic aortic injuryCT is currently considered the standard imaging modality forfollow-up in patients who benefit from TEVAR. It seemsrational to adopt a combination of a multiview chest X-ray andMRI, instead of CT.


In case of suspicion of TAI, CT is recommended. I CIf CT is not available, TOE should be considered. IIa CIn cases of TAI with suitable anatomy requiringintervention, TEVAR should be preferred tosurgery.

IIa C

a Class of recommendation.b Level of evidence.CT = computed tomography; TAI = traumatic aortic injury; TE-VAR = thoracic endovascular aortic repair; TOE = transoesophagealechocardiography.

6.9 Iatrogenic aortic dissection

Iatrogenic aortic dissection (IAD) may occur in the setting of (i)catheter-based coronary procedures, (ii) cardiac surgery, (iii) as

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a complication of endovascular treatment of aortic coarcta-tion, (iv) aortic endografting, (v) peripheral interventions, (vi)intra-aortic balloon counterpulsation and, (vii) during trans-catheter aortic valve implantation. Clinical manifestationsmay range from the absence of symptoms to excruciatingchest, back, or abdominal pain, according to the site of the AD.Hypotension, haemodynamic compromise, and shock mayensue. Treatment is conservative in most cases, with completespontaneous healing observed in most instances. Dissectionsextending over several centimetres into the ascending aorta orfurther propagating do require emergency cardiac surgery.

7 Aortic aneurysms

Aneurysm is the second most frequent disease of the aorta afteratherosclerosis. The presence of aortic aneurysm may beassociated with other locations of aneurysms. The 10-year riskof mortality from any other cardiovascular cause may be as highas 15 times the risk of aorta-related death in patients with AAA.

Recommendations in patients with aortic aneurysm.


When an aortic aneurysm is identified at anylocation, assessment of the entire aorta andaortic valve is recommended at baseline andduring follow-up.

I C

In cases of aneurysm of the abdominal aorta,duplex ultrasound for screening of peripheralartery disease and peripheral aneurysmsshould be considered.

IIa C

Patients with aortic aneurysm are at increased riskof cardiovascular disease: general principles ofcardiovascular prevention should be considered.

IIa C


Recommendations on interventions on ascending aorticaneurysms.


Surgery is indicated in patients who have aorticroot aneurysm, with maximal aortic diameterc

≥50 mm for patients with Marfan syndrome.

I C

7.1 Thoracic aortic aneurysms

TAA encompasses a wide range of locations and aetiologies,the most frequent being degenerative aneurysm of theascending aorta.

7.1.1 DiagnosisPatients with TAA are most often asymptomatic and thediagnosis is made following imaging performed either for thispurpose or other investigative reasons.

7.1.2 AnatomyMarfan syndrome, aortic enlargement is generally maximal atthe sinuses of Valsalva, responsible for annulo-aortic ectasia.In patients with BAV, three enlargement patterns aredescribed, according to whether the maximal aortic diameteris at the level of the sinuses of Valsalva, the supracoronaryascending aorta, or the sinotubular junction level.

7.1.3 EvaluationOnce aortic dilation is suspected, based on echocardiographyand/or chest X-ray, CT or MRI is required to adequatelyvisualize the entire aorta and identify the affected parts.

7.1.3.1 Aortic growth in familial thoracic aortic aneurysms.Familial TAAs grow faster, up to 2.1 mm/year. In patients withMarfan syndrome, the TAA growth is on average at 0.5–1 mm/year, whereas TAAs in patients with Loeys-Dietz syndrome(LDS) can grow even faster than 10 mm/year, resulting in deathat a mean age of 26 years.

7.1.3.2 Descending aortic growth. TAAs of the descending aortagrow faster (at 3 mm/year) than those in ascending aorta(1 mm/year).

7.1.3.3 Risk of aortic dissection. There is a rapid increase in therisk of dissection or rupture when the aortic diameter is >60 mmfor the ascending aorta and >70 mm for the descending aorta.

7.1.4 Interventions7.1.4.1 Ascending aortic aneurysms. Surgery should be per-formed in patients with Marfan syndrome, who have amaximal aortic diameter ≥50 mm. A lower threshold of45 mm can be considered in patients with additional riskfactors, including family history of dissection, size increase>3 mm/year, severe aortic regurgitation, or desire for preg-nancy. Earlier interventions have been proposed for aorticdiameters >42 mm in patients with LDS.

Surgery should be performed in patients with a BAV, whohave a maximal aortic diameter ≥55 mm. A lower threshold of50 mm can be considered in patients with additional riskfactors, such as family history, systemic hypertension, coarcta-tion of the aorta, or increase in aortic diameter >3 mm/year, andalso according to age, body size, comorbidities, and type ofsurgery. Regardless of aetiology, surgery should be performed inpatients who have a maximal aortic diameter ≥55 mm.

For patients who have an indication for surgery on theaortic valve, lower thresholds can be used for concomitantaortic replacement (>45 mm) depending on age, body size,aetiology of valvular disease, and intraoperative shape andthickness of the ascending aorta.

7.1.4.2 Aortic arch aneurysms. Surgery should be consideredin patients who have an aortic arch aneurysm with a maximaldiameter ≥55 mm or who present symptoms or signs of localcompression.

7.1.4.3 Descending aortic aneurysms. The treatment of des-cending aortic aneurysms has been re-orientated with thedevelopment of TEVAR using stent grafts.

TEVAR should be considered in patients who have adescending TAA with a maximal diameter ≥55 mm. Whensurgery is the only option, it should be considered in patientswith a maximal diameter ≥60 mm. Surgery and TEVAR may becombined in hybrid approaches. In cases of Marfan disease,surgery should be preferred over TEVAR.

(Continued )


Surgery should be considered in patients whohave aortic root aneurysm, with maximalascending aortic diameters:

� ≥45 mm for patients with Marfan syndromewith risk factors.d

� ≥50 mm for patients with bicuspid valve withrisk factors.e,f

� ≥55 mm for other patients with noelastopathy.g,h

IIa C

Lower thresholds for intervention may beconsidered according to body surface areain patients of small stature or in the caseof rapid progression, aortic valveregurgitation, planned pregnancy, andpatient's preference.

IIb C

Interventions on aortic arch aneurysmsSurgery should be considered in patients whohave isolated aortic arch aneurysm withmaximal diameter ≥55 mm. Aortic archrepair may be considered in patients withaortic arch aneurysm who already have anindication for surgery of an adjacentaneurysm located in the ascending ordescending aorta.

IIa C

Aortic arch repair may be considered inpatients with aortic arch aneurysm whoalready have an indication for surgery of anadjacent aneurysm located in the ascendingor descending aorta.

IIb C

Interventions on descending aortic aneurysmsTEVAR should be considered, rather thansurgery, when anatomy is suitable.

IIa C

TEVAR should be considered in patients whohave descending aortic aneurysm withmaximal diameter ≥55 mm.

IIa C

When TEVAR is not technically possible,surgery should be considered in patients whohave descending aortic aneurysm withmaximal diameter ≥60 mm.

IIa C

When intervention is indicated, in cases ofMarfan syndrome or other elastopathies,surgery should be indicated rather thanTEVAR.

IIa C

a Class of recommendation.b Level of evidence.c Decision should also take into account the shape of the differentparts of the aorta. Lower thresholds can be used for combiningsurgery on the ascending aorta for patients who have an indicationfor surgery on the aortic valve.d Family history of AD and/or aortic size increase >3 mm/year (onrepeated measurements using the same imaging technique, at thesame aorta level, with side-by-side comparison and confirmed byanother technique), severe aortic or mitral regurgitation, or desirefor pregnancy.e Coarctation of the aorta, systemic hypertension, family history ofdissection, or increase in aortic diameter >3 mm/year (on repeatedmeasurements using the same imaging technique, measured atthe same aorta level, with side-by-side comparison and confirmedby another technique).f Pending comorbidities in the elderly.g See text in Section 8.h For patients with LDS or vascular type IV Ehlers-Danlossyndrome (EDS), lower thresholds should be considered, possiblyeven lower than in Marfan syndrome. There are no data to providefigures and a sensible case-by-case approach is the only option.

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7.2 Abdominal aortic aneurysm

7.2.1 DefinitionAAA—almost exclusively infrarenal—is usually defined as adiameter ≥30 mm. An alternative definition of it is a >50%increased diameter.

7.2.2 Risk factorsAge, male gender, personal history of atherosclerotic cardio-vascular disease, smoking and hypertension are all associatedwith the presence of AAA. A family history of AAA is apredictor of prevalent AAA.

7.2.3 Natural historyLarge and life-threatening AAA is preceded by a long period ofsubclinical growth in the diameter of the aneurysm, estimatedat <1–6 mm/year. The risk of rupture rises exponentially withthe aneurysm's maximal diameter and is higher in women.

7.2.4 DiagnosisAAA is mostly silent. The most frequent mode of detection isincidental. Acute abdominal pain and shock are usuallypresent in the case of ruptured AAA.

7.2.4.1 Presentation. Before its cataclysmic presentation whenruptured, AAA is mostly silent. The most frequent mode ofdetection is incidental, during abdominal imaging for anyindication. Atypical abdominal or back pain may be present butshould not be awaited in order to reach a diagnosis.

7.2.4.2 Diagnostic imaging. Ultrasonography is an excellenttool for screening and surveillance. Considered the 'goldstandard' in the past was aortography. CT and MRI haveemerged as the current 'gold standards' in the pre-operativeand post-operative evaluation of AAAs.

7.2.4.3 Screening abdominal aortic aneurysm in high-riskpopulations. The grim prognosis of ruptured AAA (mortality>60–70%) contrasts with the excellent survival rate (>95%)after planned AAA operation. Opportunistic screening is themost appealing in a situation for cardiologists duringechocardiography, since abdominal aorta imaging can beperformed using the same probe.

7.2.5 Management of small abdominal aortic aneurysmsThe definition of 'small' AAA varies as 30–49 mm or 30–54 mm,the upper limit depending on the threshold set for interven-tion. In this document, 'small' AAA encompasses situationswhere endovascular or surgical intervention is not yetconsidered.

7.2.5.1 Management of risk factors. Smoking was the mostimportant predictor of future aortic aneurysm outcomes.Intense isometric exercise is usually discouraged.

7.2.5.2 Medical therapy. Studies suggested potential benefitsof beta-blockers. Statins and ACE-inhibitors should be consid-ered in these patients, to reduce risk of cardiovascular disease.The use of antiplatelet therapy has been suggested to reducecomplication rates in AAA.

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7.2.5.3 Follow-up of small abdominal aortic aneurysm. Thosewith an aorta diameter <25 mm can be considered to be at verylow risk of large AAA within the following 10 years, while aninitial aorta of 26–29 mm merits a new assessment after 4years. Intervals of 3, 2, and 1 year(s) can be safely proposed forAAAs of 30–39, 40–44 and 45–54 mm diameter, respectively.Women with 45 mm AAA had a risk of rupture equivalent tomen with a 55 mm AAA.

Recommendations on the management of asympto-matic patients with enlarged aorta or abdominal aorticaneurysm.


In patients with abdominal aortic diameter of25–29 mm, new ultrasound imaging shouldbe considered 4 years later.

IIa B

Surveillance is indicated and safe in patientswith AAA with a maximum diameter of<55 mm and slow (<10 mm/year) growth.c

I A

In patients with small (30–55 mm) AAAs, thefollowing time interval

for imaging should be considered:c

� every 3 years for AAA of 30–39 mm diameter.� every 2 years for AAA of 40–44 mm diameter.� every year for AAA >45 mmd diameter.

IIa B

Smoking cessation is recommended to slowgrowth of the AAA.

I B

To reduce aortic complications in patients withsmall AAAs, the use of statins and ACEinhibitors may be considered.

IIb B

AAA repair is indicated if:� AAA diameter exceeds 55 mm.e

� Aneurysm growth exceeds 10 mm/year.

I B

If a large aneurysm is anatomically suitable forEVAR, either open or endovascular aorticrepair is recommended in patients withacceptable surgical risk.

I A

If a large aneurysm is anatomically unsuitablefor EVAR, open aortic repair is recommended.

I C

In patients with asymptomatic AAA who areunfit for open repair, EVAR, along with bestmedical treatment, may be considered.f

IIb B

a Class of recommendation.b Level of evidence.c With <1% risk of rupture between two AAA imaging assess-ments.d This interval maybe shortened in women or in the case of rapidgrowth between previous assessments.e Individual decision for operative aneurysm correction shouldalso be influenced by the patient's gender. At a given size, AAAs inwomen are up to four times as likely to rupture under surveillance,thus aortic repair can be discussed at a lower threshold of probably50 mm. The patient's life expectancy should also be consideredprior to decision for intervention.f Since only aneurysm-related and not all-cause mortality isimproved, informed patient choice is to be taken into account.AAA = abdominal aortic aneurysm; ACE = angiotensin-convertingenzyme; EVAR = endovascular aortic repair.

Recommendations for abdominal aortic aneurysmscreening.


Population screening for AAA with ultrasound:� is recommended in all men >65 years of age. I A� may be considered in women >65 years ofage with history of current/past smoking.

Ib C

� is not recommended in female nonsmokerswithout familial history.

III C

Targeted screening for AAA with ultrasoundshould be considered in first-degree siblingsof a patient with AAA.

IIa B

Opportunistic screening for AAA during TTE:� should be considered in all men > 65 years ofage.

IIa B

� may be considered in women >65 years witha history of current/past smoking.

IIb C

a Class of recommendation.b Level of evidence.AAA = abdominal aortic aneurysm; TTE = transthoracic echocar-diography.

7.2.6 Abdominal aortic aneurysm repair7.2.6.1 Pre-operative cardiovascular evaluation. Coronary ar-tery disease is the leading cause of early mortality after surgeryfor AAA. Endovascular AAA repair procedures carry a lowerrisk (1–5%) than open surgery.

7.2.6.2 Aortic repair in asymptomatic abdominal aortic aneur-ysm. The management of AAA depends on aneurysm diame-ter. The aneurysm—until it reaches 55 mm or becomessymptomatic or fast growing (>10 mm/year)—is regarded asa safe strategy for intervention in patients with small AAAs.Diameter in women, intervention at a smaller diameter(>50 mm) may be justified.

7.2.6.3 Open aortic aneurysm repair. Open AAA repair hasbeen regarded as the default surgical intervention for AAA, butit carries a certain risk of mortality and morbidity. Outcomes ofopen ruptured AAA repair are much worse than those forelective AAA repair.

7.2.6.4 Endovascular aortic aneurysm repair. The greatestadvantage of EVAR is in its less invasive nature with lowermortality rates, at 1.4%, in recent studies. On the other hand,the long-term efficacy of EVAR remains a matter of concern.

7.2.6.5 Comparative considerations of abdominal aortic aneur-ysm management. Endovascular aortic repair is a validalternative to surgical repair of AAA; however, in patientswith more complex aortic anatomy open repair remains the

standard. For all other AAA aneurysms that are not suitable forEVAR, open repair remains the reference standard.

7.2.7 (Contained) rupture of abdominal aortic aneurysm7.2.7.1 Clinical presentation. The classic presentation of rup-tured AAA includes abdominal pain or back pain, hypotension,and abdominal pulsatile mass.

7.2.7.2 Diagnostic work-up. In the presence of free, rupturedAAA, massive periaortic bleeding involving the perirenal orpararenal spaces, as well as free fluid in the peritoneal space,allows for a straightforward diagnosis even with ultrasound.Computed tomography is the imaging method of choice in the

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evaluation of patients with suspected contained- or containedrupture of an AAA.

7.2.7.3 Treatment. The recently published results showed nosignificant difference between EVAR and open repair. Womenare exposed to higher periprocedural mortality in elective openand endovascular aneurysm repair.

7.2.8 Long-term prognosis and follow-up of aortic aneurysmrepairMost patients require a convalescence period of up to 3months after open AAA repair, after which quality-of-lifescores are similar for endovascular and open AAA repair, andeven slightly better for open repair at 1 year.

Recommendations on genetic testing in aortic diseases.


It is recommended to investigate first-degreerelatives (siblings and parents) of a subjectwith TAAD to identify a familial form inwhich relatives all have a 50% chance ofcarrying the family mutation/disease.

I C

Once a familial form of TAAD is highlysuspected, it is recommended to refer thepatient to a geneticist for family investigationand molecular testing.

I C

Variability of age of onset warrants screeningevery 5 years of 'healthy' at-risk relativesuntil diagnosis (clinical or molecular) isestablished or ruled out.

I C

In familial non-syndromic TAAD, screening foraneurysm should be considered, not only inthe thoracic aorta, but also throughout thearterial tree (including cerebral arteries).

a Class of recommendation.b Level of evidence.TAAD = thoracic aortic aneurysms and dissection.

Recommendations on management of patients withsymptomatic abdominal aortic aneurysm.


In patients with suspected rupture of AAA,immediate abdominal ultrasound or CT isrecommended.

I C

In case of ruptured AAA, emergency repair isindicated.

I C

In case of symptomatic but non-ruptured AAA,urgent repair is indicated.

I C

In case of symptomatic AAA anatomicallysuitable for EVAR, either open orendovascular aortic repair is recommended.c

I A

a Class of recommendation.b Level of evidence.c Depending on the expertise of the interventional team andpatient's level of risk.AAA = abdominal aortic aneurysm; CT = computed tomography;EVAR = endovascular aortic repair.

8 Genetic diseases affecting the aorta

Genetic diseases affecting the aorta are broadly split into twocategories: syndromic and non-syndromic, both essentiallydisplaying autosomal dominant transmission.

8.1 Chromosomal and inherited syndromic thoracic aorticaneurysms and dissection

8.1.1 Turner syndrome (TS)Turner syndrome (TS) is essentially caused by partial orcomplete monosomy of the X chromosome (karyotype 45X0).

8.1.2 Marfan syndromeIt is the most frequent heritable connective tissue disorder.

8.1.3 Ehlers-Danlos syndrome Type IVEhlers-Danlos syndrome Type IV (EDSIV) is a rare, autosomal,dominant connective tissue disorder caused by mutations inthe COL3A1 gene coding for Type III procollagen.

8.1.4 Loeys-Dietz syndromeLoeys-Dietz syndrome (LDS) is an autosomal dominant aorticaneurysm syndrome combining the triad of arterial tortuosity

and aneurysms throughout the arterial tree, hypertelorism,and bifid uvula.

8.1.5 Arterial tortuosity syndromeArterial tortuosity syndrome characterized by arterial tortu-osity, elongation, stenosis, and aneurysm of the large- andmiddle-sized arteries, arterial tortuosity syndrome (ATS) is avery rare autosomal recessive disease.

8.1.6 Aneurysms-osteoarthritis syndromeAneurysms-osteoarthritis syndrome (AOS) is a new syndromicTAAD that accounts for approximately 2% of familial TAAD.

8.1.7 Non-syndromic familial thoracic aortic aneurysms anddissectionIn these patients, familial aggregation with an affected first-degree relative is found in up to 19% of cases.

8.1.8 Genetics and heritability of abdominal aortic aneurysmThe proportion of patients with AAA who have first-degreerelatives with the disease is usually low although it does varybetween 1% and 29%.

8.2 Aortic diseases associated with bicuspid aortic valve

8.2.1 Epidemiology8.2.1.1 Bicuspid aortic valve (BAV). BAV is the most commoncongenital cardiac defect, with a prevalence at birth of 1–2%.

8.2.1.2 Ascending aorta growth in bicuspid valves. Aorticdilation, defined as an aorta diameter of >40 mm irrespectiveof body surface area, or of >27.5 mm/m2, is frequentlyassociated with BAV. BAV is a risk factor for dissection andrupture. Average annual changes in the ascending aorta inpatients with BAV may vary from 0.2 to 1.2 mm/year.

8.2.1.3 Aortic dissection. The prevalence of BAV ranges from2 to 9% in Type A AD and 3% in Type B AD.

(Continued )


Serial measurement of the aortic root andascending aorta is indicated in everypatient with BAV, with an intervaldepending on aortic size, increase in sizeand family history.

I C

In the case of a diameter of the aortic root orthe ascending aorta > 45 mm or anincrease >3 mm/year measured byechocardiography, annual measurementof aortic diameter is indicated.

I C

In the case of aortic diameter >50 mm or anincrease >3 mm/year measured byechocardiography, confirmation of themeasurement is indicated, using anotherimaging modality (CT or MRI).

I C

In cases of BAV, surgery of the ascendingaorta is indicated in case of:

� aortic root or ascending aortic diameter>55 mm.� aortic root or ascending aortic diameter>50 mm in the presence of other riskfactors.c

� aortic root or ascending aortic diameter>45 mm when surgical aortic valvereplacement is scheduled.

I C

Beta-blockers may be considered in patientswith BAV and dilated aortic root >40 mm.

IIb C

Because of familial occurrence, screening offirst-degree relatives should beconsidered.

IIa C

In patients with any elastopathy or BAVwith dilated aortic root (> 40 mm),isometric exercise with a high static load(e.g. weightlifting) is not indicated andshould be discouraged.

III C

a Class of recommendation.b Level of evidence.c Coarctation of the aorta, systemic hypertension, family history ofdissection, or increase in aortic diameter >3 mm/year (on repeatedmeasurements using the same imaging technique, measured atthe same aortic level, with side-by-side comparison and confirmedby another technique).BAV = bicuspid aortic valve; CT = computed tomography; MRI = -magnetic resonance imaging; TTE = transthoracic echocardiogra-phy.

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8.2.1.4 Bicuspid aortic valve and coarctation. Data on theprevalence of aortic coarctation in BAV are scarce; one reportstates it as 7%.

8.2.2 Natural historyMean progression is reported to be 1–2 mm/year. Rapidprogression of >5 mm/year and larger diameters are associat-ed with increased risk of AD or rupture A higher gradientacross a stenotic BAV. The re-operation for an aortic root witha diameter of 40–50 mm during the valve replacement is rarelynecessary after a follow-up of >10 years.

8.2.3 PathophysiologyNotch 1 gene mutations are associated with BAV.

8.2.4 Diagnosis8.2.4.1 Clinical presentation. BAV, with stenosis or regurgita-tion, can give rise to complaints and clinical signs. A smallsubset of patients with BAV (<15%), almost exclusively youngmen, presents predominantly with aortic root dilation withoutsubstantial valvular stenosis or regurgitation.

8.2.4.2 Imaging. There are no specific comments regardingimaging of the aorta in this setting.

8.2.4.3 Screening in relatives. Screening of first-degree rela-tives may be considered because of BAV's strong familialassociation.

8.2.4.4 Follow-up. The patient with BAV, the aortic root andascending aorta should be visualized with TTE alone orassociated with another imaging modality, preferably MRI.In cases of an increase in diameter >3 mm/year or adiameter of 45 mm, annual follow-up of the ascending aortais advised.

8.2.5 TreatmentIt is common clinical practice to advise beta-blocker therapy.The indication for surgical treatment of aortic dilation in BAV issimilar to that for other causes of dilation. When surgery isindicated for BAV, stenosis or regurgitation, aortic rootreplacement should be considered if the root is larger than45 mm in diameter.

8.2.6 PrognosisThe risk of dissection and rupture increases with the diameterof the aorta, with a sharp increase at a diameter of 60 mm.

Recommendations for the management of aortic rootdilation in patients with bicuspid aortic valve.


Patients with known BAV should undergoan initial TTE to assess the diametersof the aortic root and ascendingaorta.

I C

Cardiac MRI or CT is indicated in patientswith BAV when the morphology of theaortic root and the ascending aorta cannotbe accurately assessed by TTE.

I C

8.3 Coarctation of the aorta

8.3.1 BackgroundCoarctation of the aorta is typically located at the area of ductusarteriosus insertion.

8.3.2 Diagnostic work-upClinical features include upper body systolic hypertension,lower body hypotension, and a blood pressure gradientbetween the upper and lower extremities. Echocardiographyprovides information regarding site, structure, and extent ofcoarctation of the aorta, LVH and aortic and supra-aortic vesseldiameters. MRI and CT are the preferred non-invasivetechniques to evaluate the entire aorta in adults. Cardiaccatheterization indicates a haemodynamically significantcoarctation of the aorta and angiography is still the 'gold

c o r e t v a s a 5 7 ( 2 0 1 5 ) e 2 9 7 – e 3 1 9 e317

standard' for evaluation of this condition at many centresbefore and after operative or interventional treatment.

8.3.3 Surgical or catheter interventional treatmentIn native coarctation of the aorta with appropriate anatomy,stenting has become the treatment of first choice in adults inmany centres.

Recommendations on management of aortic plaque.


In the presence of aortic atherosclerosis,general preventive measures to control riskfactors are indicated.

I C

In the case of aortic plaque detected during thediagnostic work-up after stroke or peripheralembolism, anticoagulation or antiplatelettherapy should be considered. The choicebetween the two strategies depends oncomorbidities and other indications for thesetreatments.

IIa C

Prophylactic surgery to remove high-risk aorticplaque is not recommended.

III C


Recommendations on interventions in coarctation of theaorta.


In all patients with a non-invasive pressuredifference >20 mm Hg between upper andlower limbs, regardless of symptoms but withupper limb hypertension (>140/90 mm Hg inadults), abnormal blood pressure responseduring exercise, or significant left ventricularhypertrophy, an intervention is indicated.

I C

Independent of the pressure gradient,hypertensive patients with >50% aorticnarrowing relative to the aortic diameter atthe diaphragm level (on MRI, CT, or invasiveangiography) should be considered forintervention.

IIa C

Independent of the pressure gradient andpresence of hypertension, patients with>50% aortic narrowing relative to the aorticdiameter at the diaphragm level (on MRI, CT,or invasive angiography) may be consideredfor intervention.

IIb C

a Class of recommendation.b Level of evidence.CT = computed tomography; MRI = magnetic resonance imaging.

9 Atherosclerotic lesions of the aorta

9.1 Thromboembolic aortic disease

Aortic plaques consist of the accumulation of lipids in theintima-media layer of the aorta. Secondary inflammation,fibrous tissue deposition, and surface erosions with subse-quent appearance of thrombus may cause either thrombotic oratherosclerotic embolism.

9.1.1 EpidemiologyAortic plaques are associated with cerebrovascular andperipheral embolic events. Aortic plaques ≥4 mm wereindependent predictors of recurrent brain infarction and anyvascular events.

9.1.2 DiagnosisTTE offers good imaging of the aortic root and proximalascending aorta. Multislice computed tomography can offerexcellent imaging of aortic atheromas. Magnetic resonanceimaging can give details on the composition of plaques.

9.1.3 Therapy9.1.3.1 Antithrombotics (antiplatelets vs. vitamin K antago-nists). Because of the thromboembolic risk, antiplatelettherapy or anticoagulation is considered.

9.1.3.2 Lipid-lowering agents. No randomized trials are avail-able to support the use of statins.

9.1.3.3 Surgical and interventional approach. There are limiteddata and no clear evidence to recommend prophylacticendarterectomy or aortic arch stenting for prevention of stroke.

9.2 Mobile aortic thrombosis

Mobile thrombi in the aorta of young patients without diffuseatherosclerosis have been reported since the regular use ofTOE in patients with cerebral or peripheral emboli.

9.3 Atherosclerotic aortic occlusion

Abdominal aortic occlusion is rare and results in a major threatof leg amputation or death. The diagnosis is mostly made withthe use of Doppler ultrasonography. CT or MRI yield moredetailed information that can guide the planning of treatment.Treatment may be bypass grafting or aorto-iliac endarterecto-my. Endovascular therapy has also been proposed.

9.4 Calcified aorta

Calcification occurs in the media, and the amount ofcalcification is directly associated with the extent of athero-sclerosis (porcelain aorta).

9.5 Coral reef aorta

'Coral reef' aorta is a very rare calcifying stenotic disease of thejuxta renal and suprarenal aorta.

10 Aortitis

10.1 Definition, types, and diagnosis

Aortitis is the general term used to define inflammation of theaortic wall. The most common causes of aortitis are non-infectious inflammatory vasculitis.

Recommendations for follow-up and management ofchronic aortic diseases.


Chronic aortic dissectionContrast CT or MRI is recommended, to confirmthe diagnosis of chronic AD.

I C

Initial close imaging surveillance of patients withchronic AD is indicated, to detect signs ofcomplications as soon as possible.

I C

In asymptomatic patients with chronicdissection of the ascending aorta, electivesurgery should be considered.c

IIa C

In patients with chronic AD, tight blood pressurecontrol <130/80 is indicated.

I C

Surgical repair or TEVAR is recommended forcomplicated Type B AD (aortic diameter>60 mm, >10 mm/year growth, malperfusion orrecurrent pain).

I C

Follow-up after endovascular treatment for aortic diseasesAfter TEVAR or EVAR, surveillance isrecommended after 1 month, 6 months, 12months, and then yearly. Shorter intervals canbe proposed in the event of abnormal findingsrequiring closer surveillance.

I C

CT is recommended as the first-choice imagingtechnique for follow-up after TEVAR or EVAR.

I C

If neither endoleak nor AAA sac enlargement isdocumented during first year after EVAR, thencolour DUS, with or without contrast agents,should be considered for annual postoperativesurveillance, with non-contrast CT imagingevery 5 years.

IIa C

For patients with TAA <45 mm, annual imagingis recommended; while in patients with TAA45 mm and <55 mm, imaging every 6 months isrecommended, unless the stability of thelesions is confirmed by serial imaging.

I C

For follow-up after (T)EVAR in young patients, MRIshould be preferred to CT for magneticresonance compatible stent grafts, to reduceradiation exposure.

IIa C

Long-term surveillance of open abdominal aorticrepair may be considered at loose (5-year)intervals using colour DUS or CT imaging.

IIb C

a Class of recommendation.b Level of evidence.c Pending comorbidities and perioperative risk.AAA = abdominal aortic aneurysm; AD = aortic dissection;CT = computed tomography; DUS = duplex ultrasonography;EVAR = endovascular aortic repair; MRI = magnetic resonance ima-ging; TAA = thoracic aortic aneurysm; TEVAR = thoracic endovas-cular aortic repair.

c o r e t v a s a 5 7 ( 2 0 1 5 ) e 2 9 7 – e 3 1 9e318

10.1.1 Giant cell arteritisGiant cell arteritis tends to affect the older population, moreoften by far in women than in men. Echocardiography, CT, orMRI are recommended. PET scanning has suggested thatsubclinical aortic inflammation is often present in patientswith GCA.

10.1.2 Takayasu arteritisTakayasu arteritis is a rare, large-vessel vasculitis of unknownaetiology, typically affecting young women. Echocardiogra-phy, MRI, and CT are useful in demonstrating homogeneouscircumferential thickening of the aortic wall with a uniformsmooth internal surface. Inflammation biomarkers are elevat-ed. Pentraxin-3 may have a better accuracy in differentiatingthe active- from the inactive phase of Takayasu arteritis.

10.2 Treatment

In non-infectious aortitis, corticosteroids are the standard initialtherapy. Nearly half of the patients require additional immuno-suppression. Second-line agents include methotrexate, azathi-oprine, and anti-tumour necrosis factor-alpha agents.

11 Aortic tumours

11.1 Primary malignant tumours of the aorta

Primary malignant tumours of the aorta are an extremely rare.The prognosis for aortic sarcomas is poor, with metastaticdisease leading to death in a short time in most patients.

12 Long-term follow-up of aortic diseases

Patients with aortic disease usually require life-long surveil-lance.

12.1 Chronic aortic dissection

Now accepted practice is to further divide the time course ofAD into acute (<14 days), sub-acute (15–90 days), and chronic(>90 days) phases. Patients with chronic AD also include thosepreviously operated for Type A AD, with persisting dissectionof the descending aorta. Patients with newly diagnosedchronic AD are often asymptomatic. Patients may also presentsymptoms related to the enlarging dissected aorta or chronicmalperfusion or acute chest pain indicating rupture. Inpatients with chronic, uncomplicated Type B AD, a primaryapproach with medical therapy and repetitive clinical andimaging follow-up is recommended. Patients with chronicType B AD that is complicated by progressive thoracic aorticenlargement (>10 mm/year), FL aneurysms (with total aorticdiameter >60 mm), malperfusion syndrome, or recurrent pain,require TEVAR or surgical treatment.

12.2 Follow-up after thoracic aortic intervention

Regular clinical follow-up is necessary, more frequently withinthe first year after diagnosis or intervention and then on a

yearly basis. After aortic surgery, less-strict imaging intervalsmay be sufficient.

12.3 Follow-up of patients after intervention forabdominal aortic aneurysm

Computed tomography is the first choice for follow-upimaging after EVAR; Duplex ultrasound is specific for thedetection of endoleaks after EVAR. Magnetic resonanceimaging has high diagnostic accuracy for detection ofendoleaks after EVAR. All patients should be provided withthe best current medical treatment protocol.

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13 Gaps in evidence

This Task Force emphasizes the need for scientific networkingand multicentre trials on several aspects of the managementof aortic diseases. The Task Force highlights, briefly, majorgaps in evidence that need further research as a priority.

r e f e r e n c e 1

[1] 2014 ESC Guidelines on the diagnosis and treatment of aorticdiseases. Document covering acute and chronic aorticdiseases of the thoracic and abdominal aorta of the adult.The Task Force for the Diagnosis and Treatment of AorticDiseases of the European Society of Cardiology (ESC),Authors/Task Force members: Raimund Erbel, Victor

1 For all other references see this [1] original full text ESC docu-ment.

Aboyans, Catherine Boileau, Eduardo Bossone, Roberto DiBartolomeo, Holger Eggebrecht, Arturo Evangelista, VolkmarFalk, Herbert Frank, Oliver Gaemperli, Martin Grabenwöger,Axel Haverich, Bernard Iung, Athanasios John Manolis,Folkert Meijboom, Christoph A. Nienaber, Marco Roffi, HervéRousseau, Udo Sechtem, Per Anton Sirnes, Regula S. vonAllmen, Christiaan J.M. Vrints, European Heart Journal 35(41) (2014) 2873–2926; ESC Guidelines on the diagnosis andtreatment of aortic diseases – web addenda, Authors/TaskForce members: Raimund Erbel, Victor Aboyans, CatherineBoileau, Eduardo Bossone, Roberto Di Bartolomeo, HolgerEggebrecht, Arturo Evangelista, Volkmar Falk, Herbert Frank,Oliver Gaemperli, Martin Grabenwöger, Axel Haverich,Bernard Iung, Athanasios John Manolis, Folkert Meijboom,Christoph A. Nienaber, Marco Roffi, Hervé Rousseau, UdoSechtem, Per Anton Sirnes, Regula S. von Allmen, ChristiaanJ.M. Vrints, European Eur Heart Journal (2014), http://dx.doi.org/10.1093/eurheartj/ehu281.

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