ESVS Thoracic Ds PDF

Eur J Vasc Endovasc Surg (2017) 53, 4e52
Editor’s Choice e Management of Descending Thoracic Aorta Diseases

Clinical Practice Guidelines of the European Society for Vascular Surgery (ESVS)
V. Riambau a, D. Böckler a, J. Brunkwall a, P. Cao a, R. Chiesa a, G. Coppi a, M. Czerny a, G. Fraedrich a, S. Haulon a, M.J. Jacobs a,
M.L. Lachat a, F.L. Moll a, C. Setacci a, P.R. Taylor a, M. Thompson a, S. Trimarchi a, H.J. Verhagen a, E.L. Verhoeven a,
ESVS Guidelines Committee b P. Kolh, G.J. de Borst, N. Chakfé, E.S. Debus, R.J. Hinchliffe, S. Kakkos, I. Koncar, J.S. Lindholt,
M. Vega de Ceniga, F. Vermassen, F. Verzini,
Document Reviewers c P. Kolh, J.H. Black III, R. Busund, M. Björck, M. Dake, F. Dick, H. Eggebrecht, A. Evangelista,
M. Grabenwöger, R. Milner, A.R. Naylor, J.-B. Ricco, H. Rousseau, J. Schmidli
Keywords: Clinical practice, Descending thoracic aorta, Descending thoracic aortic management, Guideline,
Recommendations, Thoracic aorta abnormalities, Thoracic aorta diseases, Thoracic aorta disorders,
Thoraco-abdominal aorta
TABLE OF CONTENTS
1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
1.1. Purpose . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
1.2. Methodology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
2. General Aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
2.1. The normal descending thoracic aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
2.2. Epidemiology of descending thoracic aortic disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
2.3. Diagnostic methods in descending thoracic aortic disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.1. Medical history and physical examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.2. Plain radiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.3. Transthoracic echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.4. Transoesophageal echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.5. Computed tomographic angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2.3.6. Magnetic resonance imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
2.3.7. Positron emission tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
2.3.8. Intra-vascular ultrasonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
2.4. Neurological complications: prevention and management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
2.4.1. Spinal cord function monitoring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
2.4.2. Prevention of spinal cord ischaemia in open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
2.4.3. Prevention of spinal cord ischaemia in thoracic endovascular repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
2.4.4. Prevention of stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
3. Specific Thoracic Aortic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
3.1. Acute thoracic aortic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
3.1.1. Acute type B aortic dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
3.1.1.1. Definition, risk factors, and clinical presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
3.1.1.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
3.1.1.2.1. Medical management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
3.1.1.2.2. Endovascular repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
3.1.1.2.3. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
3.1.2. Intramural haematoma and penetrating aortic ulcer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
3.1.2.1. Definition and natural history . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
3.1.2.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
a
Writing Committee: Vincent Riambau* (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy),
Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat
(Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy),
Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
b
ESVS Guidelines Committee: Philippe Kolh (Chair) (Belgium), Gert Jan de Borst (Co-Chair) (Netherlands), Nabil Chakfé (France), Eike Sebastian Debus
(Germany), Robert J. Hinchliffe (United Kingdom), Stavros Kakkos (Greece and United Kingdom), Igor Koncar (Serbia), Jes S. Lindholt (Denmark), Melina Vega
de Ceniga (Spain), Frank Vermassen (Belgium), Fabio Verzini (Italy).
c
Document Reviewers: Philippe Kolh (Review Coordinator) (Belgium), James H. Black, III (United States), Rolf Busund (Norway), Martin Björck (Sweden),
Michael Dake (United States), Florian Dick (Switzerland), Holger Eggebrecht (Germany), Arturo Evangelista (Spain), Martin Grabenwöger (Austria), Ross Milner
(United States), A. Ross Naylor (United Kingdom), Jean-Baptiste Ricco (France), Hervé Rousseau (France), Jürg Schmidli (Switzerland).
* [email protected]
1078-5884/Ó 2016 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ejvs.2016.06.005
ESVS Guidelines Descending Thoracic Aorta 5
3.1.3. Ruptured aneurysm of the descending thoracic aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . 18

3.1.3.1. Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
3.1.3.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
3.1.3.2.1. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
3.1.4. Blunt traumatic thoracic aortic injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
3.1.4.1. Definition and diagnostic testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
3.1.4.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
3.1.4.2.1. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3.2. Chronic type B aortic dissections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3.2.1. Definition and natural history . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3.2.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
3.2.2.1. Indications for repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
3.2.2.2. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23
3.2.2.3. Endovascular repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24
3.3. Descending thoracic aortic aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
3.3.1. Definition and natural history . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
3.3.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
3.3.2.2. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
3.4. Thoraco-abdominal aortic aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
3.4.1. Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
3.4.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
3.4.2.2. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
3.5. Inflammatory diseases of the descending thoracic aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
3.5.1. Takayasu arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
3.5.2. Giant cell arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
3.5.3. Behçet disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
3.5.4. Other inflammatory aortitides . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
3.6. Coarctation of the thoracic aorta in adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
3.6.1. Definition and diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
3.6.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
3.6.2.2. Open repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
3.7. Miscellanea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
3.7.1. Genetic syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
3.7.1.1. Marfan syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
3.7.1.2. Loeys-Dietz syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
3.7.1.3. Ehlers-Danlos syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
3.7.1.4. Turner syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
3.7.1.5. Familial thoracic aortic disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
3.7.2. Aortic tumours . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
3.7.3. Floating thrombus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
4. Surveillance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
5. Gaps in the Evidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
5.1. General aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
5.2. Acute type B dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
5.3. Thoracic aortic rupture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.4. Blunt traumatic thoracic aortic injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.5. Chronic type B dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.6. Descending thoracic aortic aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.7. Thoraco-abdominal aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.8. Inflammatory diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.9. Coarctation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
5.10. Miscellanea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
5.11. Surveillance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Appendix. Recommended additional references . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
6 V. Riambau et al.
ABBREVIATIONS AND ACRONYMS LDS Loeys-Dietz Syndrome

AAA Abdominal Aortic Aneurysm LHB Left Heart Bypass
AAS Acute Aortic Syndromes LSA Left Subclavian Artery
AAST American Association for Surgery of Trauma MAP Mean Arterial Pressure
AD Aortic Dissection MEP Motor Evoked Potentials
ATBAD Acute Type B Aortic Dissection MFS Marfan Syndrome
CA Coeliac Artery MRA Magnetic Resonance Angiography
CAD Coronary Artery Disease MRI Magnetic Resonance Imaging
CCA Common Carotid Artery NSF Nephrogenic Systemic Fibrosis
COPD Chronic Obstructive Pulmonary Disease OR Open Repair
CSF Cerebrospinal Fluid PAU Penetrating Aortic Ulcer
CT Computed Tomography PET Positron Emission Tomography
CTA Computed Tomographic Angiography PMR Polymyalgia Rheumatica
CTBAD Chronic Type B Aortic Dissection RCT Randomized Clinical Trial
DSA Digital Subtraction Angiography SCI Spinal Cord Ischaemia
DTA Descending Thoracic Aorta SMA Superior Mesenteric Artery
DTAA Descending Thoracic Aortic Aneurysm SSEP Somatosensory Evoked Potentials
ECG Electrocardiogram TA Takayasu Arteritis
EDS Ehlers-Danlos Syndrome TAAA Thoraco-Abdominal Aortic Aneurysm
EJVES European Journal of Vascular and Endovascular TAI Thoracic Aortic Injury
Surgery TBAD Type B Aortic Dissection
ESR Erythrocyte Sedimentation Rate TEVAR Thoracic Endovascular Repair
ESVS European Society for Vascular Surgery TL True Lumen
FL False Lumen TOE Transoesophageal Echocardiography
GCA Giant Cells Arteritis TS Turner Syndrome
IMH Intramural Haematoma TTE Transthoracic Echocardiography
IRAD International Registry of Aortic Dissection WC Writing Committee
IVUS Intravascular Ultrasonography
1. INTRODUCTION document to avoid potential inter-specialty conflict. The

cost analysis of different treatments was also excluded
1.1. Purpose because of differences in financial management and
The European Society for Vascular Surgery (ESVS) appointed differing health systems across Europe. Primarily infectious
the Descending Thoracic Aorta (DTA) Writing Committee or mycotic disease processes were also considered outside
(WC) to produce the current clinical practice guidelines of the scope of this document because of their low inci-
document for surgeons and other physicians who are dence and poor outcomes.
involved in the overall care of patients with DTA disorders. All disorders originating in the DTA from the left sub-
The goal of these guidelines is to summarize and evaluate all clavian artery (LSA) origin to the diaphragm were consid-
current available evidence to assist physicians in selecting the ered for these guidelines. Pathology involving the thoraco-
best management strategies for all DTA pathologies. How- abdominal segment of the aorta was also included.
ever, each respective physician must make the ultimate de-
cision regarding the particular care of an individual patient.
The present guidelines document aims to improve deci- 1.2. Methodology
sion making and decrease variability in the vascular surgical The DTA WC was formed by members of the ESVS from
care of patients presenting with pathology of the DTA. different European countries, various academic and private
Unfortunately, robust evidence from prospective and ran- hospitals, and by both vascular surgeons and endovascular
domized studies is not available for management of most specialists, to maximize the applicability of the final guide-
DTA diseases. Consequently, the recommendations in these lines document. The DTA Guidelines Committee met in
guidelines are entirely based on level B and C evidence. November 2011 for the first time to discuss the purpose,
Nevertheless, when managing DTA pathology, it is clinically contents, methods, and timeline of the following
helpful to have access to the most recent and best available recommendations.
clinical and experimental knowledge to determine the cur- The DTA WC performed a systematic English literature
rent standard of care. search in the MEDLINE, EMBASE, and COCHRANE Library
The DTA WC intentionally agreed to exclude pathology of databases for each of the different topics that are discussed
the ascending aorta and aortic arch from the current and reviewed. The latest literature search was performed in
Table 1. Levels of evidence.1 For each recommendation, two members of the WC

assessed the strength of a recommendation and the
Level of Evidence A Data derived from mulple
randomized clinical trials or meta-
quality of supporting evidence independently. A full
analyses master copy of the manuscript with all recommendations
Level of Evidence B Data derived from a single randomized
was electronically circulated and approved by all WC
clinical trial or large non-randomized members. Recommendations that required consensus
studies were discussed and voted on by email among all members
Level of Evidence C Consensus of opinion of the experts of the WC.
and/or small studies, retrospecve This system permits strong recommendations, supported
studies, or registries
by low or very low quality evidence from downgraded RCTs
or observational studies, only when a general consensus is
December 2015. With regard to evidence gathered, the achieved among the WC members and reviewers.
following eligibility criteria were applied: Two members of the WC prepared each part of the
guidelines document. An internal review process was per-
Only peer reviewed published literature was considered formed before the manuscript was sent to independent
Published abstracts or congress proceedings were external reviewers. External reviewers made critical sug-
excluded gestions, comments, and corrections on all preliminary
Randomized clinical trials (RCT) as well as meta-analyses versions of this guideline. In addition, each member
and systematic reviews were assessed with priority participated in the consensus process concerning conflict-
Non-randomized clinical trials and non-controlled studies ing recommendations. The final document was reviewed
were included and approved by the ESVS Guidelines Committee and
Well conducted observational studies (cohort and case submitted to the European Journal of Vascular and Endo-
control studies) were included vascular Surgery (EJVES). Further updated guidelines doc-
Previous guidelines, position papers, and published uments on DTA management will be provided periodically
consensus documents were included as part of the by the ESVS when new evidence and/or new clinical
review process when new evidence was absent practice arise in this field.
We minimized the use of reports from a single medical To optimize the implementation of the current docu-
device or from pharmaceutical companies in order to ment, the length of the guideline has been kept as short as
reduce the risk of bias across studies. possible to facilitate access to guideline information.
Following this decision, the “References” list has been
A grading system was adopted based on the European limited to the most relevant references related to these
Society of Cardiology (ESC) guidelines methodology.1 The guidelines. Nevertheless, an Appendix of recommended
level of evidence classification provides information about additional references, also reviewed by the WC, has been
the study characteristics supporting the recommendation added for further information for readers. This clinical
and expert consensus, according to the categories shown in guidelines document was constructed as a guide, not a
Table 1. document of rules, allowing for flexibility with various pa-
The recommendation grade indicates the strength of a tient presentations. The resulting clinical practice guidelines
recommendation. Definitions of the classes of recommen- provide recommendations for the clinical care of patients
dation are shown in Table 2. with thoracic aortic diseases including pre-, peri-, and post-
operative care.
Table 2. Grades of strength of recommendations according to the Conflicts of interest of each WC member were collected
ESC grading system.1 prior to the writing process. These conflicts were assessed
Classes of DefiniƟon
and accepted by each member of the WC and are reported
recommendaƟon on the ESVS website. In addition, the WC agreed that all
intellectual work should be expressed without any inter-
Class I Evidence and/or general agreement that a ference beyond the honesty and professionalism of all
given treatment or procedure is beneficial, members and assistants during the writing process.
useful, effec ve. It should be performed
Class II Conflic ng evidence and/or a divergence of
2. GENERAL ASPECTS
opinion about the usefulness/efficacy of the
given treatment or procedure 2.1. The normal descending thoracic aorta
Class IIa Weight of evidence/opinion is in favour of
usefulness/efficacy. It should be considered The DTA originates from the isthmus, the region of the
thoracic aorta between the origin of the LSA and the
Class IIb Usefulness/efficacy is less well established by
evidence/opinion. It may be considered ductus arteriosus. The descending thoracic aorta runs in a
Class III Evidence or general agreement that the given
left para-spinal location until its distal segment, where it
treatment or procedure is not passes anteriorly through the diaphragmatic aortic hiatus
useful/effec ve, and in some cases may be and inferiorly into the abdomen. Important aortic side
harmful. It is not recommended branches originating from the descending thoracic aorta
8 V. Riambau et al.
include the intercostal arteries, spinal arteries, and bron- recent prospective analysis of 30,412 middle aged men and
chial arteries. The normal diameter of the mid-descending women from Malmö, Sweden with a 20 year follow up re-
aorta ranges from 24 to 29 mm in men and 24 to 26 mm in ported an incidence of acute aortic dissection of 15 per
women, whereas the normal diameter at the level of the 100,000 patient years.5 This increase is probably caused by
diaphragm is 24 to 27 mm in men and 23 to 24 mm in the increasing age of the population and improving diag-
women. Aortic diameter is influenced by age and body nostic modalities. The exact incidence remains unknown,
mass index.2,3 but PAU has been diagnosed with increasing frequency
The aortic wall is composed of three layers: the intima, because of the widespread use of advanced cross sectional
media, and adventitia. The intima, the innermost layer, imaging techniques. In symptomatic patients suspected of
consists of an endothelial monolayer and an internal AAS, the prevalence of PAU is 2.3e7.6% and the lesion is
elastic lamina. Because it is in direct contact with blood, localised in the DTA in 90% of patients.6 IMH may be
the function of the intima is to prevent thrombosis and related to PAU, accounting for 5e20% of patients with AAS
atherosclerosis. Its anti-thrombotic and anti-atheroscle- and more commonly involving the DTA (60%) than the
rotic function can be reduced by risk factors, such as ascending aorta.7
smoking, hypertension, hyperlipidaemia, diabetes, and Trauma is the leading cause of death during the first four
direct trauma, each making patients more prone to aortic decades of life, accounting for more than 250,000 deaths
disease. every year in the European Union alone.8 Blunt aortic injury
The media consists of concentric layers of elastin, is the second leading cause of death in these patients, and
collagen, and smooth muscle cells. These components are although it occurs in less than 1% of all motor vehicle ac-
responsible for aortic wall elasticity, which accommodates cidents, it accounts for 16% of all traumatic deaths.9
the changes in stroke volume during the cardiac cycle, Concerning ruptured descending thoracic aortic aneu-
converts pulsatile inflow into a smoother outflow (Wind- rysm (DTAA), a Swedish population study reported an
kessel function), and maintains the integrity of the aortic incidence of 5 per 100,000 person-years. The mean age of
wall. Congenital or hereditary disorders (e.g. bicuspid aortic patients in this cohort was 70 years for men and 72 years
valve, Marfan syndrome [MFS], Ehlers-Danlos syndrome for women.10
[EDS]), risk factors (hypertension, atherosclerosis, and Aneurysmal dilatation of the DTA is a degenerative dis-
trauma) all influence aortic wall function. These conditions ease with an estimated incidence of 6e10.4 per 100,000
can stiffen the aortic wall, decreasing its ability to accom- person-years. The incidence seems to be increasing with
modate the stroke volume, resulting in systemic hyperten- ageing of the general population and continually improving
sion, or weakening of the aortic wall, leading to dilatation or diagnostic modalities.11
dissection. Aortitis is a relatively uncommon disorder with a broad
The adventitia is the outermost layer of the aortic wall spectrum of clinical features. The most common autoim-
and is composed mainly of collagen fibres, external elastic mune disorders affecting the DTA are Takayasu’s arteritis
lamina, and small vessels (the vasa vasorum), which pro- (TA), giant cell arteritis (GCA), and Behçet’s disease. The
vide the blood supply to the aortic wall and surrounding best estimates of the incidence of TA suggest that two or
nerves. three cases occur each year per million people.12 There is a
9:1 female to male predominance. Although the disease has
2.2. Epidemiology of descending thoracic aortic disease a worldwide distribution, it appears to occur more
DTA diseases consist of a broad spectrum of degenerative, frequently in Asian women.
structural, acquired, genetic, and traumatic disorders. The GCA is the most common type of vasculitis observed in
true incidence of descending thoracic aortic pathology re- patients older than 50 years. Predominantly observed in
mains unclear. Epidemiological studies are sparse and it is populations of Scandinavian descent, it has a reported
likely that many DTA related deaths are attributed to other prevalence that varies between 1 and 30 per 100,000
cardiovascular diseases, such as cardiac arrest, myocardial people.13 The male to female ratio is around 2.5 to 1 and is
infarction, cerebrovascular accidents, or abdominal aneu- highly dependent on geographic and genetic parameters.14
rysm rupture. Therefore, the overall incidence of DTA dis- Behçet’s disease has been observed most commonly
ease is likely to be underestimated. along the classic Silk Route, with a peak prevalence in
The pathophysiology of thoracic aortic diseases is Turkey of 80e370 per 100,000 people, compared with 1e3
believed to be multifactorial, resulting both from genetic per million people in the Western world. Presentation is
susceptibility and environmental exposure. Therefore, the typically in the third to fifth decade of life, and both genders
incidence of the different thoracic aortic diseases can vary are affected equally.15
significantly among different population groups. Coarctation of the aorta is a congenital cardiovascular
Acute aortic syndromes (AAS) consist of three interre- defect, most commonly occurring at the level of the
lated diseases: aortic dissection, penetrating aortic ulcer isthmus and accounts for 5e8% of all congenital heart
(PAU), and intramural haematoma (IMH). Type B aortic defects. The overall incidence ranges between 20 and 60
dissection (TBAD) most commonly affects male patients and per 100,000 person-years, with a slight male predominance.
has an incidence between 2.9 and 4.0 per 100,000 person- Patients with Turner syndrome (TS) are more commonly
years.4 The incidence of TBAD seems to be increasing. A affected.16
2.3. Diagnostic methods in descending thoracic aortic in the diagnosis of DTA pathology remains limited. In cases of
disease examination limitations or inconclusive diagnosis, the use of
2.3.1. Medical history and physical examination. A additional imaging modalities is recommended.19 There are
comprehensive medical and family history, assessment of currently no specific studies to validate the usefulness of TTE
symptoms, and careful physical examination including blood for diagnosis of DTA pathology.
pressure measurement and electrocardiography (ECG), are 2.3.4. Transoesophageal echocardiography. TOE can visu-
required in all patients suspected of thoracic aortic disease. alize the DTA from the LSA to the coeliac artery (CA). This
Patients should be examined for suspicious clinical signs diagnostic test is generally used as a second line imaging
such as aortic regurgitation, cardiac murmur, pericardial modality and is useful to differentiate between AD, IMH,
rub, signs of tamponade, and an expansile abdominal aorta. and PAU. With a reported sensitivity of 98% and a specificity
The diagnosis of thoracic aortic disease is based on imaging of 95%, TOE is an accurate diagnostic tool for aortic disease,
and the choice of imaging modality should be based on the providing functional information in both the pre- and intra-
patient’s condition and the availability of different imaging operative settings.19 The semi-invasive nature of TOE has
modalities.17 rare procedure related risks, but it can cause patient
2.3.2. Plain radiography. Postero-anterior and lateral chest discomfort, requires sedation, and is contraindicated in the
radiographs can be used to diagnose calcification within presence of oesophageal pathologies. In the majority of
atheromatous lesions, left pleural effusions, aortic enlarge- cases, computed tomographic angiography (CTA) scanning
ment, and anomalous aortic contours in asymptomatic or is performed as the first imaging modality, providing all
symptomatic patients. A left pleural effusion can indicate a required information. TOE may be used in specific circum-
frank rupture, an exudate from inflammation of the adven- stances as a second line option.
titia in AAS, or, less commonly, inflammatory aortic disease.
The International Registry of Acute Aortic Dissection (IRAD) 2.3.5. Computed tomographic angiography. CTA offers a
showed that 21% of patients with a type B aortic dissection detailed visualisation of the entire aorta and its surrounding
presented with a normal chest X-ray, and a meta-analysis structures. It can distinguish different aortic pathologies and is
demonstrated a sensitivity for an abnormal chest X-ray of quick and widely available. Over the last two decades, CTA has
90% for TBAD.18 Although chest X-rays might be used in very become more sophisticated and is more readily available,
low risk patients to exclude thoracic aortic diseases, these with an increase in the number of scanners, the use of
potentially lethal diseases require a conclusive diagnosis retrospective and prospective ECG-gated techniques, and
with the use of multiplanar imaging techniques such as through advances in post-processing software. These ad-
computed tomographic angiography (CTA). vances have resulted in motion free images with better res-
olution, reduced scanning times, and better visualisation.20
RecommendaƟon 1 Class Level of evidence References
All pa ents with clinical suspicion of thoracic aor c disease and I C 17,18
abnormal chest radiograph should undergo computed tomographic
angiography for diagnosis confirma on.
2.3.3. Transthoracic echocardiography. The use of TTE to Current CTA scanners have a sensitivity of up to 100%
assess the DTA is limited by structures in the thorax that and a specificity of 98e99%, with slight variations between
weaken or distort the ultrasound signal and compromise im- different DTA pathologies.21 Imaging should include a non-
age quality. Via a suprasternal, subcostal, or parasternal view, contrast study, followed by an early and late phase contrast
small parts of the DTA can be visualized, while in the case of a study and should examine the part of the body between the
pleural effusion, the back of the patient can be used for thoracic inlet and the common femoral arteries. This range
transthoracic imaging.19 The major advantage of TTE is that it provides complete information with three dimensional
is non-invasive and can be used to visualize the ascending reconstruction. In addition, dynamic images can further
aorta, aortic arch, and supra-aortic vessels. In addition, the assist in surgical planning or endovascular interventions.
abdominal aorta can be visualized to check for abdominal Consequently, CTA has replaced digital subtraction aortog-
extension of aortic dissection (AD). During TTE, all planes raphy as the “gold standard” for aortic imaging. Important
should be used to assess the extent of aortic disease and to disadvantages of CTA include the use of nephrotoxic
exclude additional aortic or cardiac involvement. Because of contrast agents and the exposure of patients to ionizing
its non-invasive nature and wide availability, TTE is increasingly radiation. In patients at increased risk of contrast induced
used in the emergency department of community hospitals to nephropathy, circulating volume expansion with either
screen patients suspected of having one or other acute aortic isotonic sodium chloride or sodium bicarbonate solutions is
syndrome, such as type A dissection. However, the value of TTE recommended.22
10 V. Riambau et al.
Mul detector computed tomographic angiography from thoracic IIa C 20,21

inlet to common femoral arteries should be considered as the first
line diagnos c modality for descending thoracic aor c pathology
RecommendaƟon 3
For the diagnosis of descending thoracic aor c disease, IIa C 19
transoesophageal echocardiography should be considered as a
second line imaging modality when computed tomography is
unavailable, contraindicated, or inconclusive
RecommendaƟon 4
For pa ents at increased risk of contrast induced nephropathy, IIa C 22

volume expansion with either isotonic sodium chloride or sodium
bicarbonate solu ons should be considered before contrast
administra on
2.3.6. Magnetic resonance imaging. MRI has an excellent radiation exposure is a major disadvantage of PET/CT (when
diagnostic capability that is comparable with CTA and TOE compared with PETor CT examinations alone), as the effective
and can be used for both pre-operative planning and follow radiation dose is a combination of the dose from both scans.27
up.23 A major advantage of MRI is that it offers multiplanar
evaluation with good differentiation between different soft 2.3.8. Intra-vascular ultrasonography. IVUS permits 360
tissues. Moreover, MRI can provide additional dynamic visualisation of the aortic wall. It can be very helpful in
imaging regarding entry tear flow or arterial vessel confirming intimal defects when CTA and digital subtraction
involvement.23 Although contrast is not usually required, angiography (DSA) are inconclusive in the diagnosis of aortic
the use of gadolinium enhances the quality of MRI. MRI can injuries. IVUS is an operator and experience-dependent
be used to visualize the aortic wall in detail and is, there- invasive procedure, and a complete evaluation of the
fore, more commonly used in patients with aortic wall pa- aorta using IVUS can be time consuming. In some centres,
thologies such as IMH or aortitis. Furthermore, MRI does IVUS is routinely used as an adjuvant imaging technique
not require ionizing radiation and offers an alternative for during endovascular repair (see also Section 3.1.4.1).
patients with renal insufficiency in some circumstances. Table 3 compares different imaging diagnostic tests for DTA
Important disadvantages of MRI include its limited according to their features and performance.
availability and longer scanning times, which makes it less
suitable for critically ill or unstable patients. In addition, the 2.4. Neurological complications: prevention and
ability of MRI to detect calcification is decreased (compared management
with CTA), and artefacts from respiration or metallic im-
plants reduce image quality. Post-gadolinium nephrogenic The most feared and devastating complications associated
systemic fibrosis (NSF) is a rare, but devastating, side-effect with management of DTA disease are spinal cord ischaemia
in patients with impaired renal function. Using alternative (SCI), resulting in paraparesis or paraplegia, and cerebral
imaging modalities or using the lowest possible amount of embolism, resulting in stroke.28 SCI can develop immedi-
gadolinium may prevent NSF.24 ately after surgery or with a delayed presentation.
Although SCI rates as high as 20% have been reported, the
2.3.7. Positron emission tomography. PET is a nuclear im- incidence probably ranges between 2% and 6%.29 The
aging modality that is based on the detection of increased incidence of paraplegia is related to the duration and de-
metabolic activity in inflammatory cells, resulting in increased gree of spinal ischaemia resulting from an abrupt cessation
uptake of a gammagraphic tracer, most commonly fluo- of blood flow to the spinal cord and from a reperfusion
rodeoxyglucose ([18F] FDG). PET can be used for the diagnosis injury mediated by biochemical mediators.30 The presence
of aortitis and in the assessment of the extent and activity of of stroke after DTA surgery seems to be similar to aortic
any inflammatory disease. The diagnostic value of FDG-PET arch repair and often has an embolic aetiology, with an
differs among the various inflammatory aortic diseases with incidence up to 8% in elective cases or higher in the
a reported sensitivity ranging between 83% and 100% and emergency setting.31 Classic predictors for these compli-
specificity between 77% and 100%.25 Development of PET in cations are diverse and include increasing patient age,
combination with computed tomography (CT) scanners has previous carotid artery disease, aortic cross-clamp time, the
made it possible to combine functional and anatomic imaging, extent of the aortic resection, aortic rupture, concurrent
thereby making it possible for PET findings to be correlated aneurysm disease of ascending aorta and/or aortic arch,
with adjacent anatomical features. Although the availability of emergency surgery, the use and duration of hypothermic
PET and PET/CT is limited, this modality may be used for arrest, peri-operative hypotension, and pre-operative renal
diagnosis and follow up of aortitis.26 Increased patient dysfunction.28,31
Table 3. Comparison of different imaging modalities for DTA diagnosis.17

Characteristics TTE TOE CTA MRI PET IVUS
Non-invasiveness þþþ þþ þ
Ease of use þþþ þþ þþþ þþ þþ þ
Availability þþþ þþ þþþ þþ þ þ
Portability/bedside use þþþ þþþ þ/
Interventional guidance use þ þþþ þþþ
Safe for serial examinations þþþ þþ þþ þþþ þþ
Wall contour assessment þ þþþ þþþ þþ þþ þþ
Supra-aortic vessel status evaluation þþ þ þþþ þþþ þþ þþ
Aortic valve status evaluation þþþ þþþ
Pericardial effusion detection þþþ þþþ þþþ þþþ þþ
Inflammation/Infection detection þ þþ þþ þþþ þ
Number of signs (þ or ) represents the estimated potential value.
TTE ¼ transthoracic echocardiography; TOE ¼ transoesophageal echocardiography; CTA ¼ computed tomographic angiography;
MRI ¼ magnetic resonance imaging; PET ¼ positron emission tomography; IVUS ¼ intra-vascular ultrasonography.
Several methods for spinal cord and brain protection important vessels is relevant for success.35 Revascularisa-
have been used to reduce the incidence of neurological tion of the artery of Adamkiewicz and the intercostal ar-
complications. These methods are described in the teries of T11/T12 has been reported, although not widely
following sections. implemented. Pre-operative angiography has been used to
identify the most important vessels, but as angiography it-
2.4.1. Spinal cord function monitoring. During thoracic self can induce SCI (resulting from embolisation), other
aortic surgery, the function of the spinal cord can be directly modalities such as magnetic resonance angiography (MRA)
monitored with use of somatosensory evoked potentials and CTA have become more popular.36
(SSEP) or motor evoked potentials (MEP).30 SSEP monitoring Cerebrospinal fluid (CSF) pressure increases with aortic
records stimulation of the posterior tibial nerve by elec- cross-clamping, and will eventually exceed the venous pres-
trodes placed on the scalp, and this technique is sensitive sure, compromising venous outflow, leading to spinal cord
for detecting spinal cord ischaemia. Clinical studies have malperfusion and secondary SCI. Randomized controlled
shown that SSEP can be used both to identify dominant trials and meta-analyses have shown that CSF drainage has a
intercostal arteries and to determine if these vessels require role in the prevention of paraplegia and paraparesis, with a
re-implantation.32 However, SSEP does not record motor risk reduction up to 75% (OR 0.48, 95% CI 0.25e0.92), but
function because perfusion of the anterior corticospinal additional studies are recommended.29,37
tract is not assessed. Therefore, SSEP can be associated with During aortic cross-clamping, prophylactic CSF drainage
delayed detection of ischaemia, thereby reducing its spec- aims to maintain CSF pressure at 10 mm Hg intra-
ificity.30 MEP, on the other hand, stimulates the cortico- operatively and for 48e72 hours after completion of the
spinal tract transcranially or via the spinal cord directly. This aneurysm repair. In fact, delayed and late onset of neuro-
technique has demonstrated increased sensitivity in pre- logical deficit are also described and potentially worsened
dicting SCI compared with SSEP.33,34 Major disadvantages of by haemodynamic instability.35
MEP are the influence of anaesthetic agents on the po- Recent studies have shown that CSF drainage combined
tential amplitude and the resistance of axonal conduction to with intrathecal papaverine solution can be beneficial.38 Po-
ischaemia, which makes the response of MEP to ischaemia tential complications related to CSF drainage occur in fewer
generally slow.33 Institutional experience and availability of than 5% of cases, and include meningitis, epidural haema-
these techniques should determine their use. toma, subdural haematoma, and CSF leakage syndrome.37e39
During open thoracic or thoraco-abdominal aor c repair, IIb C 30,33

peri-opera ve monitoring of motor and/or somatosensory evoked
poten als may be considered to predict spinal cord ischaemia
2.4.2. Prevention of spinal cord ischaemia in open repair. The use of left heart bypass (LHB) prevents heart failure
Surgical techniques have been developed over the years to and maintains distal aortic perfusion and CSF pressures as
prevent SCI. Techniques like the Crawford inlay, single clamp close to baseline as possible, and thereby reduces the risk of
repair, sequential aortic clamping, and others have been post-operative paraplegia and paraparesis in patients un-
introduced with varying results. As revascularisation of all of dergoing repair of type I and type II thoraco-abdominal
the intercostal arteries is not feasible, the identification of aortic aneurysm (TAAA)40 (see also Section 3.4.). Extra-
corporeal circulation allows sequential aortic clamping, prevention of ischaemia, which suggests that numerous
maintaining retrograde perfusion of all of the vital organs agents may protect the spinal cord from transient
and the spinal cord. These techniques have been shown to ischaemia.43
be beneficial in patients with TAAA, reducing the incidence Other pharmacological agents such as glucocorticoste-
of SCI by perfusion of the distal aorta from 11.2% to 4.5%.40 roids may be beneficial in the prevention of SCI, although
Another important advantage of circulatory bypass is that it high quality evidence is lacking.44 Other options include
permits the induction of systemic hypothermia for addi- intrathecal hypothermia, but this requires an open lam-
tional neurological protection. Distal perfusion is often inectomy or closed epidural infusion system. These tech-
combined with CSF drainage or hypothermia, and has niques are not fully applicable in current clinical practice
proven beneficial in reducing the incidence of SCI.35,38,41 (see also Sections 3.1, 3.2, 3.3, and 3.4 for specific details).
Cerebrospinal fluid drainage has a role in the preven on of IIa B 29,37

paraplegia and paraparesis and should be considered during
extensive open repair of the descending thoracic aorta
RecommendaƟon 7
To prevent spinal cord ischaemia, left heart bypass, allowing distal IIa C 40
perfusion, during open type I and II thoraco-abdominal aor c
aneurysm repair should be considered
RecommendaƟon 8
During extensive open descending thoracic aorta repair, moderate IIb C 41,42
hypothermia around 32 °C may be considered to prevent spinal
cord ischaemia
RecommendaƟon 9
Systemic cooling to less than 32 °C in combination with cerebrospinal III B 39,42

fluid drainage is not recommended during open descending thoracic
aor c repair as it can increase the risk of subdural bleeding
Induced systemic hypothermia by cardiopulmonary bypass 2.4.3. Prevention of spinal cord ischaemia in thoracic
and intermittent cardiac arrest might be an effective endovascular repair. TEVAR has been associated with a
method to reduce the risk of SCI.41 Moderate systemic reduced incidence of neurological complications compared
hypothermia to temperatures of 32 C improves outcome with open DTAA repair, but the risk of paraplegia or para-
without significant risks for the patient.41,42 However, the paresis ranges from 2.5% up to 8% and remains a
systemic anticoagulation required in these patients in- concern.44e46 Prior abdominal aorta aneurysm (AAA) repair,
creases the risk of bleeding, and, in such conditions, CSF prolonged hypotension, severe atherosclerosis of the
drainage and intrathecal manoeuvres can be thoracic aorta, occlusion of the left subclavian artery and/or
dangerous.39,41,42 During the post-operative period, the hypogastric arteries, and extensive coverage of the thoracic
maintenance of mean arterial pressure (MAP) between 80 aorta by the endograft are all associated with an increased
and 100 mm Hg has been suggested by high volume incidence of SCI.44e49 Therefore, spinal cord protection
centers.35 (including CSF drainage) should be considered in patients
After a period of ischaemia caused by cross-clamping, with previous AAA repair or in patients who require
reperfusion injury can occur. Biochemical mediators, espe- extensive aortic repairs, as the benefit of CSF drainage is
cially iron-related free radicals, induce cellular damage. greatest in patients at the highest risk for spinal cord
Free radical scavengers have been studied in the injury.45e47
Pa ents with planned extensive thoracic aorta coverage (>200 mm) IIa C 45–47
or previous AAA repair have a high risk for spinal cord ischemia and
prophylactic cerebrospinal fluid drainage should be considered in
endovascular thoracic aorta repair.
2.4.4. Prevention of stroke. Intra-operative embolism is the most common site for the proximal intimal tear in ATBAD is
main cause of stroke in patients undergoing surgical repair located just distal to the origin of the left subclavian artery.
of the DTA, emphasizing the importance of precautions In 90% of cases, ATBAD has a secondary tear that allows
during the operative period. blood to re-enter the TL at what is known as the re-entry
Debris from aortic atheroma/thrombus can result in an site.51,52
embolic ischaemic stroke and the release of debris should The Stanford and DeBakey classifications are most
be prevented during surgery. Reducing manipulation of the commonly used to describe ATBAD (Fig. 1). DeBakey clas-
DTA to a strict minimum40 and TOE mapping of the sites of sified AD, based on the origin of the intimal tear and the
cannulation are techniques that can minimize the risk of extent of the dissection. The Stanford classification is based
embolisation.19 When clamping is neither possible nor on the involvement of the ascending aorta.53 A type A
recommended, deep hypothermic circulatory arrest must dissection is defined by involvement of the ascending aorta
be instituted.42 In patients with atrial fibrillation, the use of and type B by the absence of ascending aortic involvement.
arteriovenous femoro-femoral bypass or deep hypothermic There is no consensus about the classification of arch AD
arrest and the avoidance of cannulation of the left atrium without involvement of the ascending aorta.54 Type B dis-
with possible manipulation of thrombus may be a useful sections originate distal to the ostium of the left subclavian
alternative.40,42 artery. This classification is comparable with a DeBakey type
During endovascular repair, guidewires within the aortic III (including type IIIb), in which the dissection extends into
arch have the potential to mobilize debris and guidewire the abdominal aorta. ATBAD accounts for 30e40% of all
manipulation should be reduced to the minimum. Accord- dissections.52,54e56 AD is defined as “acute dissection”
ing to a recent retrospective, multicentre analysis of se- within 14 days of the onset of symptoms. Thereafter, it is
lective LSA revascularisation in the treatment of multiple defined as sub-acute between 2 weeks and 3 months and
DTA pathologies,48 coverage of the LSA in patients treated chronic after 3 months (see also Section 3.2).
with TEVAR was associated with a higher incidence of stroke Any condition that increases intimal shear stress or
and paraplegia. Therefore, LSA revascularisation should be decreases arterial wall strength is considered to be a risk
considered in the elective setting.44e46,49,50 In the acute factor. Systemic hypertension is present in almost 80% of
setting, revascularisation should be performed based on the ATBAD patients.51 Increasing age and atherosclerosis are
patient’s anatomy (left mammary to coronary bypass, or other important risk factors,57 as are congenital bicuspid
dominant cerebral blood supply from the left vertebral ar- or uni-commissural aortic valves,58 cocaine abuse,59
tery) during emergency repair of the DTA (see also Sections pregnancy,60 strenuous activities, and severe emotional
3.1 and 3.3). stress.61
In elec ve thoracic endogra ing cases when it is planned to IIa C 44

inten onally cover the le subclavian artery, in pa ents at risk of
neurological complica ons, preven ve le subclavian artery
revascularisa on should be considered
3. SPECIFIC THORACIC AORTIC DISORDERS

3.1. Acute thoracic aortic syndrome
AAS covers a heterogeneous group of patients with similar
anatomic and clinical characteristics. This group includes AD,
IMH, and PAU.51 Traumatic aortic transection and ruptured
DTAA also may be included. The most common symptom is
chest pain. Histopathologically, all of these entities involve
disruption of the media of the aorta, with bleeding between
the layers of the aorta or transmurally in the case of rupture.
Each condition can progress to another one of the group
and each pathology may coexist in the same patient.
3.1.1. Acute type B aortic dissection.
3.1.1.1. Definition, risk factors, and clinical presentation.
Acute type B aortic dissection (ATBAD) is the result of a tear
in the intimal arterial layer, which allows blood to propagate
within the medial layer. This creates a flap, which divides Figure 1. Schematic drawings of De Bakey (I, II, IIIa and IIIb) and
the aorta into a true lumen (TL), and a false lumen (FL). The Stanford (A and B) combined classifications for aortic dissection.
Table 4. Main clinical presentations of ATBAD. mortality.60 Although most patients present with these
Signs and symptoms Incidence, % symptoms, some may have a non-specific presentation or
Acute excruciating chest or 80 even no symptoms, which can delay the diagnosis.68,69 For
interscapular pain that reason, physicians must be familiar with an atypical
Chest pain 79 presentation and a low threshold for diagnostic imaging
Back pain 64 should be maintained.
Abdominal pain 43 The most severe ATBAD complications include aortic
Syncope 4
rupture and occlusion of arterial branches, with conse-
Pulse deficits 9
quent loss of arterial perfusion. Aortic rupture is associated
Hypotension/shock 4
Visceral ischaemia 7 with a high mortality, irrespective of the type of treat-
Renal ischaemia 15 ment,52,70e73 whereas patients with impending rupture
Limb ischaemia 9 who have no haemodynamic complications have better
Recurrent pain, refractory 18 outcomes.56,70
pain, or refractory hypertension Renal and/or visceral ischaemia may be difficult to detect.
Spinal cord ischaemia 3 Renal ischaemia can increase creatinine levels and potentially
induce refractory hypertension.Treatment of renal ischaemia
is important to prevent permanent renal insufficiency and
Another important risk factor is a positive family history refractory hypertension which is likely to result in a more
of thoracic aortic diseases. ATBAD has a prevalence of 13e rapid expansion of the affected aorta. Visceral ischaemia was
22% in patients who have a first degree relative with a the third most common cause of death (after aortic rupture
history of DTAA or AD.62 The “familial thoracic aorta and and tamponade) in AD patients in the IRAD study,55 and can
dissection syndrome” is related to several identified gene result from malperfusion or systemic hypotension.55,56,60
mutations, including fibrillin-1 (FBN1), transforming growth Serum lactate levels are elevated when the ischaemic injury
factor-b1 (TGFBR1), transforming growth factor-b2 progresses or becomes irreversible; therefore early diagnosis
(TGFRB2), a-actin 2 (ACTA 2), and myosin heavy chain 11 is essential. ATBAD patients who present with or who
(MYH11), increasing insight into the genetic pathology of develop recurrent abdominal pain should undergo repeat
the disease.63,64 cross sectional imaging. In this situation, there should be a
Structural weakness of the aortic wall is associated with low threshold for undertaking laparoscopic inspection of the
multiple connective tissue disorders such as MFS, EDS, and peritoneal cavity.74 Gastrointestinal haemorrhage is a rare
Loeys-Dietz syndrome (LDS) (see also Section 3.7). In pa- complication and every patient presenting with bleeding and
tients younger than 40 years of age, who present with abdominal pain should be suspected of having mesenteric
aortic dissection, about 50% will have MFS or a related ischaemia.75
genetic disorder. This group of patients, as well as those Acute limb ischaemia may present with paralysis of one
with a family history of thoracic aortic diseases, should or both lower limbs. Because of the dynamic nature of the
receive genetic counselling.62 The aortic diameter is not dissection membrane, the presence of palpable peripheral
closely related to ATBAD, although an increased risk of pulses may be misleading.76
ATBAD has been observed in patients with a dilated DTA.65 Paraplegia/paraparesis represents a catastrophic, but
The mean diameter in ATBAD has been reported as rare, complication of ATBAD secondary to SCI. Other com-
41 mm.66 Indeed, many cases of ATBAD can occur in pa- plications include refractory pain and refractory hyperten-
tients with normal diameter aortas.66,67 sion, which are both indirect signs of impending rupture
The clinical presentation of patients presenting with and are associated with increased in hospital mortality.77
ATBAD can be diverse and may mimic a wide range of other Complicated ATBAD is defined as the presence of rapid
disorders. The classical presentation is acute onset excru- aortic expansion, aortic rupture and/or hypotension/shock,
ciating chest or interscapular pain, which is present in about visceral, renal, or limb ischaemia, paraplegia/paraparesis,
80% of ATBAD patients.55,60 The main clinical signs and peri-aortic haematoma, recurrent or refractory pain, and
symptoms are reported in Table 4. The IRAD showed that refractory hypertension despite adequate medical therapy.
hypotension (OR 23.8, 95% CI 10.31e54.94, p < .0001), In hospital survival in complicated ATBAD patients
absence of chest/back pain (OR 3.5, 95% CI 1.3e9.52, treated conservatively is about 50%.55,56,60,65,70e72,78
p ¼ 0.01), and branch vessel involvement (OR 2.9, 95% CI Hypotension/shock and visceral ischaemia are considered
1.21e6.99, p ¼ 0.02) are predictors of in hospital the most important predictors of in hospital death.56,60
Recommendaon 12 Class Level of evidence References
Paents with acute type B aorc dissecon who develop new or I C 78

recurrent abdominal pain and where there is any suspicion of
visceral, renal and/or limb malperfusion should undergo repeat
CT imaging
Complicated ATBAD patients with severe comorbidities Pain relief is also an important component of optimal
(e.g. ischaemic heart disease, chronic pulmonary disease, or medical therapy, as persisting pain may indicate progression
malignancy) may not benefit from invasive management of the dissection or impending rupture, requiring additional
and should be evaluated individually.65,70,78 therapy.77
ATBAD may also present without complications in almost During hospitalisation, all IV medications should be
50% of cases. This cohort is defined as uncomplicated converted into oral agents, and long-term blood pressure
ATBAD.55,78 Despite the absence of complications at the regulation with adequate surveillance is mandatory.
Medical therapy should always be part of the treatment of paents I C 55,56,60,65,70,77–79

with acute type B dissecon
Recommendaon 14
In paents with acute type B aorc dissecon, β-blockers should be IIa C 79,83,84
considered as the first line of medical therapy
Recommendaon 15
In paents with acute type B aorc dissecon who do not respond or IIb C 79,83
are intolerant of β-blockers, calcium channel antagonists and/or
renin-angiotensin inhibitors may be considered as alternaves or
complementaries
time of presentation, these patients have an in hospital 3.1.1.2.2. Endovascular repair. TEVAR has developed as the
mortality of 3e10%.55,78 In the presence of complications first line therapeutic option in patients with complicated
(such as visceral, renal or limb ischaemia, and/or aortic ATBAD.85e95 The aim of endovascular repair for treating
rupture), mortality rises to 20% by day 2 and 25% by day 30. impending aortic rupture or malperfusion is to cover the
Like type A dissection, advanced age, rupture, shock, and primary entry tear and to reduce blood pressure within the
malperfusion are important predictors of increased early FL.90,95 Reduction in FL perfusion can prevent extension of the
mortality.60,79 dissection, which may lead to FL thrombosis, aortic remod-
3.1.1.2. Management. The aims of treating ATBAD are to elling, and aortic wall stabilisation.
maintain or restore perfusion of the vital organs and to Although there are no randomized controlled trials, there
prevent both progression of the dissection and aortic is increasing evidence that in complicated ATBAD, emergency
rupture. Therefore, it is important to make a risk assessment and urgent interventions have been beneficial.55,60,77,86,87 In
at an early stage to determine the merits of medical, these settings, TEVAR has shown a substantial advantage
endovascular, or surgical intervention. over OR in terms of early mortality.87e89,92e94
3.1.1.2.1. Medical management. Medical therapy with Currently, there are three meta-analyses available that
antihypertensive agents is widely accepted to be the first report short- and mid-term results in complicated ATBAD
line treatment in uncomplicated ATBAD patients.52,54e56, patients treated with TEVAR. Technical success ranged from
60,65,70,77e79
Medical management is based on blood 95% to 99%, hospital mortality ranged from 2.6% to 9.8%, and
pressure reduction to limit aortic wall stress and to reduce neurological complications ranged from 0.6% to 3.1%.6,96,97
the force of left ventricular ejection.72 The goal is to A prospective, multicentre European clinical registry
reduce systolic blood pressure between 100 and 120 mm showed a 30 day mortality of 8%, a stroke rate of 8%, and a
Hg and, when attainable, the heart rate below 60 beats/ SCI rate of 2% in 50 ATBAD patients.98 The initial results of a
min.54 In ATBAD, initial medical therapy consists of b- single arm multicentre study for endovascular repair of
blockers.55,79,80 In patients who do not respond to b- complicated AD, using a composite device design (PETTICOAT
blockers or who do not tolerate the drug, calcium channel technique), which includes an uncovered infra-
antagonists and/or renin-angiotensin inhibitors can be diaphragmatic aortic stent in addition to a standard TEVAR,
used as alternatives.80e83 b-blockers and calcium channel showed a 1 year mortality of 10%. Stroke, transient ischaemic
blockers have been associated with improved long-term attack, or progression of dissection occurred in 7.5%, 2.5%,
survival in ATBAD patients.83 Recent data have shown and 5% of patients, respectively.99 Another study confirmed
that angiotensin II type 1 receptor blockers have similar these findings with an in hospital mortality of 4%, 40%, and
positive effects to atenolol in terms of growth reduction 33% in TEVAR, OR, and medically treated patients, respec-
of the aortic root in children and young adults with tively.87 However, there is no evidence that extended
MFS.81,82 Other alternative therapies include sodium coverage of the DTA is needed to restore distal perfusion.
nitroprusside, A1-adrenergic, and non-selective Among complicated ATBAD patients, those presenting
b blockers.84 with visceral malperfusion experience the poorest
outcomes. Although visceral vessel patency following TEVAR use of proximal balloon dilatation, proximal bare metal stents,
is maintained in up to 97% of patients, the 30 day mortality and with rigid non-compliant devices.107 To facilitate the pa-
is high (17e34%), as are aortic related complications during tient selection process, important anatomical and clinical
the chronic stage.67,89 features that characterize the aortic dissection pathology
These patients seem to benefit from aortic balloon were recently summarized in a new categorisation scheme
fenestration and branch stenting.100e102 A dynamic (DISSECT), which may be helpful in making the decision of
obstruction can be managed by increasing FL outflow with whether or not to intervene.108
fenestration of the intimal flap, while a static obstruction or Endovascular repair of ATBAD can be technically chal-
ostial disruption should be treated by stenting of the mal- lenging and optimal results can be obtained in high volume
perfused branch vessel.73,100e102 centres with considerable endovascular experience and a
TEVAR has also been advocated in the treatment of un- multidisciplinary approach to the treatment of aortic dis-
complicated ATBAD, to prevent long-term aortic dilatation eases.6,100 Chronic post-TEVAR aortic dilatation may be
and rupture. IRAD reported reduced mortality at 5 years in observed in patients with persistent and patent FL.87,92,109
ATBAD patients treated by TEVAR, compared with those Therefore, life long clinical and imaging surveillance are
managed medically.103 ADSORB, the only randomized con- advised in this situation6 (see also Section 4.).
In pa ents with complicated acute type B aor c dissec on, I C 6,85–89,92–94,96–99,

endovascular repair with thoracic endogra ing should be the first 103,109
line interven on
RecommendaƟon 17
In complicated acute type B aor c dissec on, endovascular IIa C 73,100–102

fenestra on should be considered to treat malperfusion
RecommendaƟon 18
To prevent aor c complica ons in uncomplicated acute type B aor c IIb B 104
dissec on, early thoracic endogra ing may be considered selec vely
trol trial in patients with uncomplicated ATBAD, was not 3.1.1.2.3. Open repair. The aim of OR in the treatment of
sufficiently powered for mortality at 1 year follow up. This ATBAD is to replace the DTA with a graft and excise the
trial did, however, show higher rates of FL thrombosis in intimal tear, to restore peripheral perfusion and treat or
patients randomized to TEVAR, and FL thrombosis is asso- prevent aortic rupture. Partial cardiopulmonary bypass has
ciated with fewer late complications and increased aortic been widely used and hypothermic circulatory arrest has
remodelling following repair of ATBAD.104 been adopted for cerebral protection in a subset of patients
Although TEVAR results in this setting are favourable, who were managed by OR approaches.110e112 There are no
endovascular related complications can be devastating and randomized controlled trials available to compare the
may require revision with OR.105 Stroke is reported to occur in different OR techniques and the level of evidence sup-
3e10% of patients undergoing TEVAR because of manipula- porting various techniques is low. In patients presenting
tion of catheters in the arch and ascending aorta, and occurs with complications such as imminent rupture, rapid
more commonly in patients with severe arch atheroscle- expansion, or malperfusion syndromes, classic OR carries a
rosis.44 Although rare in ATBAD patients, SCI is related to the significant risk of morbidity, including irreversible spinal
extent of aortic coverage, history of previous aortic surgery injury and post-operative death.110e112 Surgical aortic
and the presence of hypotension at initial presentation.47 fenestration or extra-anatomical bypass has been used for
Arm ischaemia, paraparesis, and paraplegia may occur from treating complicated ATBAD, but with the introduction of
LSA or intercostal artery occlusion, which may require revas- minimally invasive techniques, this procedure is only used
cularisation.49,50 (see Section 2.4.4). Other complications as an alternative when endovascular repair fails or is
(device or procedure related) can include aortic rupture contraindicated.113
during deployment, angulation, migration, or collapse of the Although the results of OR have improved over the last few
stent graft, false aneurysm formation at the proximal or distal decades,114e117 complications remain high, with in hospital
end of the stent graft, graft erosion, or stent frame frac- mortality rates ranging from 25% to 50%.70,71,115,116 Pre-
ture.106 A retrograde type A dissection is associated with operative conditions heavily influence outcomes following
devastating outcomes. TEVAR for aortic dissection is particu- surgical repair.71 Patients older than 70 years with hypoten-
larly prone to retrograde type A aortic dissection. The risk of sion or shock have less favourable outcomes, while those,
retrograde type A dissection seems to be increased with the normotensive at the time of surgery, have better outcomes.71
Complications affect 40e80% of patients undergoing open thickening of the aortic wall, extending in a longitudinal, non-
repair.115 Neurological complications such as paraplegia spiral fashion, is pathognomonic. The aortic lumen is pre-
(2.3e6.6%), paraparesis (0e3.1%), and stroke (2.7e6.6%) served and the luminal wall is curvilinear and smooth, as
are associated with the extent and duration of the opera- opposed to a rough, irregular border seen with aortic
tion.71,88,115,116 Benefits from extracorporeal circulation in atherosclerosis and PAU. IMH is generally a more localised
the reduction of neurological complications and renal process than classic AD, which typically propagates along the
ischaemia remain controversial.88,114,115 Other complications entire aorta to the iliac arteries.119
include myocardial ischaemia or infarction, respiratory fail- The natural history of type B IMH is similar to that of
ure, visceral ischaemia, sepsis, multiorgan failure, and limb ATBAD.7,51 Conservative medical treatment is used for iso-
ischaemia.71,88,114e116 lated uncomplicated type B IMH.120 Treatment with b-
In acute complicated type B aorc dissecon, open repair should be IIa C 94,113
considered as an alternave to endovascular therapy following
failure of endovascular management or where endovascular
intervenons are contraindicated
3.1.2. Intramural haematoma and penetrating aortic ulcer. blockers has a survival rate of 95%, compared with 67% for
3.1.2.1. Definition and natural history. Intramural haema- those treated without b-blockade.120 IMH, if associated with
toma (IMH) is defined as the presence of blood within the PAU, has a significantly worse prognosis with a higher risk of
aortic wall without intimal disruption or an identifiable expansion and rupture.51 Regression of acute IMH occurs in
entry point on imaging. IMH may be a precursor to both one third, progression in 20%, and up to 40% evolve into AD.7
classic AD and penetrating aortic ulcer (PAU). It is distin- Indications for treatment in type B IMH are refractory chest
guished from AD by the absence of an intimal flap and from pain, evidence of increasing size of the expanding haematoma,
PAU by the absence of any connection with the aortic aortic rupture, and a progressive pleural effusion.7,51,120
lumen.117 Most cases of IMH (50e85%) are located in the Similarly, there is also considerable controversy regarding
DTA. At least 5e15% of patients admitted with IMH have an the aetiology of PAU.51 PAU may result from progressive
AD. The exact pathophysiology underlying IMH remains erosion of atheromatous mural plaque with penetration of
controversial. One theory suggests that IMH is a conse- the elastic lamina. PAU may also develop in younger patients
quence of rupture of vasa vasorum in the medial layer of with intimal tears which remain localised and fail to progress
the aortic wall, which then causes a secondary tear into the to AD or IMH. PAU is more often present in the DTA and
aortic lumen. This process is typically associated with hy- occurs more often in older patients with arterial hyperten-
pertension and is initiated by aortic wall infarction. Another sion, hyperlipoproteinaemia, and aortic sclerosis. Compli-
theory suggests that IMH results following an intimal entry cated PAU involves degeneration towards pseudoaneurysm
tear, allowing blood from the aortic lumen to enter the formation, dissection, or rupture. Careful imaging is needed
aortic wall. The blood then thromboses within the intimal to evaluate both the diameter and depth of PAU.119 Although
layer so that no entry tears can be detected.117,118 Modern the specific growth rate is unknown, 20e30% of asymp-
imaging suggests that IMH, PAU, and AD can develop from tomatic PAUs show evidence of progression over time.119
each other and, therefore, they are likely to be variants of Symptomatic PAUs have an ominous natural history of
the same pathological process. IMH evolves longitudinally progression and rupture. Urgent repair is commonly rec-
between the medial layers and may progress, regress, or ommended in this setting.119,121 Currently, there is a lack of
remain unchanged.117 IMH has identical clinical manifesta- data concerning the natural history of patients with
tions and treatment principles to those of AD. The classifi- asymptomatic PAU. Progression with pseudoaneurysm for-
cation of IMH follows that of the Stanford classification of mation may occur in 15e50% of cases. The association
AD. Type A IMH involves the ascending aorta. Type B IMH is between aortic diameter and rupture risk remains unclear.
localised in the aortic arch and in the DTA.118 However, patients with a PAU that initially measures
Cross sectional imaging techniques (CTA, MRA) are used to >20 mm in diameter or >10 mm in depth have a high risk
differentiate between IMH, PAU, and AD. The characteristic of disease progression and should be considered candidates
finding of IMH on axial imaging is a thickening of the aortic for early endovascular repair.119,121
wall greater than 5 mm in an eccentric or concentric pattern. 3.1.2.2. Management. Patients presenting with uncompli-
Mural thrombus has an irregular luminal surface, narrows the cated type B IMH are primarily treated by medical therapy
lumen, and does not extend longitudinally as much as IMH. and intensive care monitoring, in line with the management
Discrimination between IMH and acute dissection with a of AD (see Section 3.1.1.2.1).122
thrombosed FL may be difficult. Unenhanced CT acquisition is Endovascular repair is currently indicated in symptomatic
crucial for the diagnosis of IMH. A high attenuation crescentic or complicated patients or in those with evolution towards
AD because of a higher risk of peri-operative morbidity and 3.1.3.2. Management. Rupture of the DTAA is an acute
the risk of rupture.121 Endovascular repair is associated with condition resulting in a high mortality. Most patients die
lower peri-operative morbidity and mortality than OR.123e125 before receiving treatment or do not survive treatment.
Nevertheless, the role of endovascular repair in patients with Survivors are at risk of multisystem organ failure and/or
type B IMH is debatable and identifying appropriate in- cerebral/spinal insult. Traditionally, DTAA rupture has been
dications for treatment is critical. Although the literature treated by OR but, in the last few decades, endovascular
provides no compelling guidelines for treatment, the WC repair has emerged as an alternative option in selected
recommends that treatment of IMH should follow similar patients. Symptomatic and ruptured DTAAs should be
guidelines as for the treatment of AD in the corresponding treated urgently because of the risk of imminent exsan-
segment of the aorta, especially if it is associated with an guination and death.
evolving PAU, expansion of IMH, intimal tear disruption, or 3.1.3.2.1. Open repair. Traditionally, ruptured DTAA has
peri-aortic haematoma or progression to AD.119 been managed by open repair. A recent meta-analysis of
Indications and the choice of treatment of PAU are similar 224 patients with ruptured DTAA demonstrated a 30 day
to IMH. High mortality rates have been reported for OR119; mortality of 33% for patients treated with OR.126 Major
therefore, endovascular repair is the preferred first line complications of myocardial infarction, stroke, and para-
treatment.123 According to the largest published series, plegia have been reported to occur in 11.1%, 10.2%, and
specific aortic diameter, neck length and depth of PAU were 5.5%, respectively. Another recent multicenter, retrospec-
not required for endovascular repair. Indications were pain tive review of 69 patients with ruptured DTAA published by
and increase of the total aortic diameter at the level of the the same authors demonstrated a composite outcome of
PAU.121 In the absence of RCT, the level of evidence for the death, stroke, or permanent paraplegia in 36.2%.127 These
treatment of PAU is low. There is currently no evidence based results were confirmed by a larger study, including data
treatment recommendation available to support treatment from the US Nationwide Inpatient Sample data on 559
of asymptomatic PAU beyond blood pressure control. patients with ruptured DTAA, with a reported mortality rate
Uncomplicateda type B intramural haematoma and penetrang I C 121,122

aorc ulcer should be treated medically, and followed by serial
imaging surveillance
Recommendaon 21
Endovascular repair should be considered for complicateda type B IIa C 123–125

intramural haematoma
Recommendaon 22
Endovascular repair should be considered for complicatedb type B IIa C 119,121,123,124

penetrang aorc ulcer
a
Uncomplicated/complicated IMH means absence or presence of recurrent pain, expansion of the IMH, peri-aorc haematoma, and
inmal disrupon.
b
Complicated PAU means presence of recurrent pain or PAU that inially measures >20 mm in diameter or >10 mm in depth or
progression of total aorc diameter.
3.1.3. Ruptured aneurysm of the descending thoracic of 28.6%.128 These data confirm that OR for ruptured DTAA
aorta. is associated with high mortality and morbidity.
3.1.3.1. Definition. Most thoracic aortic aneurysms are 3.1.3.2.2. Endovascular repair. TEVAR has emerged as a
either located in the ascending aorta or the DTA, but either less invasive therapeutic option for the management of
type can extend into the aortic arch. Rupture risk correlates ruptured DTAA. No prospective, randomized study has
with aneurysm diameter. Aortic rupture is defined as compared stent grafting versus OR in the treatment of
disruption of all the layers of the aortic wall (intima, media, ruptured DTAA. However, the results of meta-analyses and
and adventitia). In the acute phase, active extravasation of multicentre studies suggest lower mortality and complica-
blood (as detected by contrast enhanced CTA, MRA, or tion rates following TEVAR.126e128
echocardiography), is pathognomonic for rupture. Gener- In a recent meta-analysis comparing endovascular repair
ally, DTAA rupture is contained by periaortic structures and OR for ruptured DTAA, the 30 day mortality was 19%
(pleura, pericardium) or intrathoracic organs (oesophagus, and 33%, respectively.126 Lower rates of myocardial infarc-
lungs, heart). tion (3.5%), stroke (4.1%), and paraplegia (3.1%) were noted
for endovascular repair. The composite outcome of death, The classic site of TAI is at the aortic isthmus in about
stroke, and myocardial infarction was 21.7% in the TEVAR 55e90% of patients admitted to hospital alive. Other re-
group compared with 36.2% in the OR group (odds ratio gions of the thoracic aorta are less often affected.132,133
0.49, 95% CI 0.24e0.97, p ¼ .044). By contrast, in the study Trauma to the distal segment of the thoracic aorta can be
using the US Nationwide Inpatient Sample data, TEVAR was associated with injury to the diaphragm and adjacent
not associated with a significantly lower mortality rate compression fractures of the thoracic spine.132
compared with OR (23.4% vs. 28.6%, respectively, After traumatic brain injury, TAI is the second most
p > .1).128 common cause of death in blunt trauma patients. The
The need for LSA revascularisation in challenging prox- morbidity and mortality of this injury are high, causing
imal aortic neck anatomy is controversial, especially in acute sudden death in 80e90% of cases.134 With improved rescue
cases. LSA coverage, to achieve a satisfactory proximal seal and rapid detection of TAI, patients who initially survive are
during TEVAR for ruptured DTAA, is reported in up to 38% more likely to undergo successful repair.
of cases. LSA revascularisation was not performed in most The damage incurred in TAI can be partial or circumfer-
of these cases.127,128 Comprehensive data regarding the ential. With more accurate diagnostic tools, the term “mini-
rationale for LSA coverage without revascularisation are mal aortic injury”, which implies the presence of a small
unavailable. In one study, subclavian artery bypass was intimal flap with minimal to no peri-aortic haematoma, has
performed in half of the cases (10/19 covered) before been introduced to describe a lesion that carries a low risk of
TEVAR.129 In cases in which the LSA is to be covered, prior rupture.135 A classification scheme for TAI has been pro-
revascularisation of the LSA in the emergency setting is posed: type I (intimal tear), type II (intramural haematoma),
recommended in patients with a left internal mammary type III (pseudoaneurysm), and type IV (rupture).136
artery to coronary artery bypass, or in those with a clearly About 2e8% of patients with an initially unrecognized
dominant left vertebral artery. In all other emergency pa- TAI may develop a chronic post-traumatic pseudoaneur-
tients, LSA coverage without revascularisation can be per- ysm.137 There are only a few reports on the natural history
formed49,129 (see also Section 2.4.4.). of this type of pseudoaneurysm. The largest series reporting
In cases with challenging distal thoracic aortic anatomy, 413 patients was published in 1982.138 Up to 85% under-
the CA can be selectively covered. Angiography of the su- went surgical repair, and one third of the remaining patients
perior mesenteric artery (SMA) after balloon occlusion of died from their untreated lesions within 20 years of the
the CA can visualize adequate collateral circulation, how- initial trauma. Other authors report the development of late
ever even with proven collateralisation, ischaemic compli- symptoms in about 50%, with aneurysmal expansion in
cations can occur following CA coverage.130 Endovascular about 20%, and death secondary to aortic rupture in 20%
parallel or “periscope” endograft techniques, can poten- within 15 years of the injury.139
tially be used to maintain perfusion through the coeliac Clinical presentation ranges from non-specific symptoms
artery.131 Physician modified fenestrated endografts have to thoracic or interscapular pain. Signs of chest wall injury,
also been used to maintain perfusion to the CA. The pseudo-coarctation syndrome, a systolic murmur, or para-
development of off the shelf fenestrated grafts may provide plegia can be present. The risk assessment for TAI begins
new options to treat acute DTA pathology involving visceral with a high index of suspicion based on the mechanism of
branches (see also Section 3.3.2.3.). injury. Abdominal injury, thoracic injury, hypotension, and
In pa ents with ruptured descending thoracic aor c aneurysm, I B 127

endovascular repair should be the first treatment op on when the
anatomy is appropriate
RecommendaƟon 24
In emergency ruptured descending thoracic aor c aneurysm in I C 49

pa ents with a patent le mammary to coronary bypass or with a
dominant or single le vertebral artery, le subclavian artery
revascularisa on should be performed prior to le subclavian artery
coverage
3.1.4. Blunt traumatic thoracic aortic injury. lack of restraint in motor vehicle accidents have been
3.1.4.1. Definition and diagnostic testing. Blunt traumatic identified as clinical predictors of TAI. The greater the blunt
thoracic aortic injury (TAI) most often occurs after sudden trauma force the higher the index of suspicion for TAI
deceleration as a result of head on or side impact collisions, should be.140
usually in high speed motor vehicle accidents or falls from Initial plain chest X-rays have a significant false negative
great heights. rate in patients with TAI. Consequently, once aortic
transection is suspected, computed tomography evaluation 3.1.4.2. Management. The appropriate timing of any oper-
is recommended.141 CTA has replaced angiography as the ation in patients with TAI remains controversial. In 1997, the
first line diagnostic test for TAI.142 CTA is quick and repro- American Association for the Surgery of Trauma (AAST) pub-
ducible, with a near 100% sensitivity and specificity in lished a multicentre study reporting that about 9% of patients
identifying TAI. In addition, CTA is ideal for evaluating non- reaching the hospital haemodynamically stable, progressed to
arterial trauma such as brain, spinal, pelvic, splenic, liver, free rupture. The majority of these ruptures occurred within
and kidney damage in patients with multiple injuries. CTA 24 hours.146 For this reason, immediate treatment of TAI was
findings, associated with TAI, include mediastinal haema- considered the standard of care for many years.
toma, haemothorax, pseudoaneurysm formation, variation More recent studies147,148 have shown a reduction in
of aortic contour, and presence of intimal flap and paralysis and mortality with delayed treatment. In 2008, a
thrombus. DSA now has a very limited role in TAI prospective, observational, multicentre study by the AAST
detection.142 demonstrated similar complication rates, but a significantly
With the increasing use of TEVAR, IVUS has been advo- higher mortality in patients who underwent early repair
cated as having an important role in the evaluation of when compared with delayed repair. The study concluded
selected patients with suspected TAI.143 Most of the patients that patients with TAI and associated major injuries are
who undergo TEVAR for TAI are young.144,145 These patients more likely to benefit from delayed intervention.149
have a healthy and elastic aortic wall, with increased aortic This conclusion is consistent with an extensive review of
pulsatile compliance. Aortic diameter varies in these patients the literature150 including 139 studies and 7,768 patients,
by 10% (up to 18%) between systole and diastole.145 In which did not show a significant difference in mortality
contrast to CTA, IVUS permits a dynamic real time evaluation between early (within 24 hours) and delayed (after
of aortic diameters during the cardiac cycle. Furthermore, 24 hours) repair, suggesting that repair can be delayed if
IVUS can easily detect the origin of side branches, allowing other extensive injuries require stabilisation. These con-
less use of contrast with DSA. Once the stent graft is clusions are consistent with the guidelines of The Society of
deployed, IVUS can be used to evaluate adequate expansion Vascular Surgery, which suggest urgent repair (within
of the stent, excluding infolding complications. 24 hours) barring other serious concomitant non-aortic
TOE has a limited role and low sensitivity in routine injuries, or repair immediately after other injuries have
screening for TAI and should not be performed at the been treated but prior to hospital discharge.151
expense of prompt evaluation of other coexisting injuries. The type of aortic injury is also a determining factor in
However, DSA and TOE might be used together when CTA is the timing of intervention. Patients with a 15 mm or larger
not available or is equivocal.143 TOE can also be used intra- arch haematoma were significantly more likely to die from
operatively to monitor myocardial function and fluid man- TAI than those with a smaller haematoma. By contrast,
agement. In addition, TOE has gained an important role in minimal aortic injuries that present with an intimal tear and
the delivery of stent grafts in TEVAR143 (see also Section occur in 10% of patients with TAI may be managed
2.3.4.). expectantly with serial imaging for surveillance.135,151
Computed tomographic angiography should be performed for the I C 142

detec on of trauma c aor c injury
RecommendaƟon 26
In trauma c thoracic aor c injury, transoesophageal IIa C 142,143

echocardiography or intravascular ultrasonography should be
considered for intra-opera ve sizing and for the deployment of
stent gra s
Pa ents with free rupture of a blunt trauma c thoracic aor c injury I C 136,144,149
or a large peri-aor c haematoma (≥15 mm) should undergo
emergency repair
RecommendaƟon 28
In cases of blunt trauma c thoracic aor c injury without large IIa C 144,149,150
haematoma, delayed interven on should be considered to priori ze
treatment of associated life threatening injuries
3.1.4.2.1. Open repair. OR of a TAI at the classic isthmus an associated mortality of 12.9%. Similar advantages were
location requires exposure of the aorta through a left fourth found in a systematic review in terms of survival and a
interspace thoracotomy with single right lung ventilation to decreased incidence of paraplegia with TEVAR, when
improve surgical exposure. The aorta is clamped at the compared with OR. This review reported an early endoleak
origin of the LSA and distal to the vessel injury. Until the rate of 4.2% and a stent collapse rate of 1.2%. The latter
mid-1980s, most of these procedures were completed with complication was fatal in 30% of cases.160
an expeditious clamp-and-sew technique. Although there Most TAI occur in young patients, who are more likely to
are isolated reports of reasonable outcomes,152 meta- have acute curvature of the aortic arch. This anatomical
analyses of this technique have reported a mortality of variant may limit the optimal apposition of the stent graft
16e31% and a paraplegia rate of 5e19%.147,148,153 to the inner aortic curvature, leading to “bird beaking” and
Various methods of distal aortic perfusion have been used an increased risk of endoleak and stent collapse.163 The
to protect the spinal cord. Early techniques used heparin- average diameter of the thoracic aorta, proximal and distal
bonded intraluminal shunts (passive perfusion).153 Meta- to the rupture site, is 19 mm in patients with trauma.
analyses and large-cohort studies of active versus passive Excessive oversizing (>20%) has been associated with an
perfusion showed a decrease in the rate of post-operative increased risk of device collapse.163 With respect to pre-
paraplegia from 19% to 3% and a mortality reduction from operative CTA measurements, stent grafts should be
30% to 12% in favour of active perfusion with partial car- oversized by about 10%. More aggressive oversizing may
diopulmonary bypass154 (see also Section 2.4.2.). be applied in gravely hypotensive patients, but not
Optimal peri-operative patient management with a skilled exceeding 20%. Hypotension may lead to inadvertent
trauma team is mandatory to establish the correct timing and undersizing of the stent graft with a consequent increased
treatment priorities. Aggressive fluid administration that may risk of migration, endoleak, and/or graft collapse once
exacerbate bleeding, coagulopathy, and rupture should be resuscitation is fully achieved.164 Where possible, delayed
avoided. MAP should not exceed 80 mm Hg.136 Hypotension TEVAR may allow complete resuscitation. In these cases,
(systolic blood pressure 90 mm Hg), and peri-aortic hae- serial CTA imaging can provide more reliable data
matoma size (15 mm) were found to be independent risk regarding the actual aortic measurements for stent graft
factors for early mortality in patients with TAI.144 sizing.164
In patients suffering severe brain injury with elevated The necessity of LSA coverage during TEVAR for TAI was
intracranial pressure and haemorrhage or significant lung reported in 30% of cases in recent reviews.150,160 As in
injury, delayed repair of TAI is preferable to avoid further other pathological situations, in the emergency setting LSA
brain or respiratory deterioration.133 revascularisation is recommended in patients with left in-
3.1.4.2.2. Endovascular repair. The first TEVAR procedure ternal mammary artery to coronary artery bypass, and
for a post-traumatic aortic pseudoaneurysm was performed when the presence of a dominant left vertebral artery as-
in 1987 by Nicolai Volodos in Ukraine.155 Since then, TEVAR sures a better posterior cerebral perfusion compared with
has become the “first choice treatment” for TAI for many the right49,129 (see also Sections 2.4.2. and 3.1.3.2.2.).
authors because of good early and late re- A separate consideration should be applied to chronic
sults.126,129,136,147,149,152,156e161 Data extracted from the post-traumatic pseudoaneurysms, which have different
Nationwide Inpatient Sample in 2006 showed a significant anatomical characteristics from degenerative aneurysms, in
change from OR to TEVAR in the USA.162 A recent non- that they are typically localised, calcified saccular lesions
systematic review of the literature159 identified 62 retro- located just distal to the LSA, making them attractive targets
spective reviews or studies and six meta-analyses. A sub- for endovascular repair.165
group analysis of these papers showed that the TEVAR The indications for late surgical intervention after aortic
population was older, whereas patients in the OR group were injury are not the same as those for other types of DTAA
more often unstable. Compared to OR, TEVAR showed a (see also Section 3.3.).
significantly reduced mortality (9.7% vs. 27.7%; p < .001) and Follow up strategies after TEVAR for TAI deserve a special
a trend towards reduced paralysis (0.4% vs. 2.9%). OR of TAI mention. A combination of multi-view chest X-rays and
was associated with a reduced stroke rate (0.4% vs. 2.3%). MRA, rather than CTA, may be considered166 (see also
Combining these neurological complications (paralysis and Section 4.).
In pa ents with trauma c thoracic aorta injury and suitable anatomy, I C 157,159,160
endovascular repair should be performed as the first op on
stroke), the complication rate was comparable between the 3.2. Chronic type B aortic dissections
two groups. Overall, the endoleak incidence was 5.2% (type I 3.2.1. Definition and natural history. Historically, TBAD is
proximal in almost all cases). There was a 1% migration rate in defined as “chronic” when 14 days have elapsed from the
this cohort and stent collapse occurred in 2.5% of cases with acute event. This temporal classification is based on the fact
that 70% of deaths from aortic dissection occur within 2 Long-term survival after uncomplicated type B dissec-
weeks of onset.167 The risk of death following dissection tions remains relatively poor. An analysis of the IRAD
remains high during the first 3 months and, in recent years, database has shown that late mortality is high in patients
it has been suggested that a separate category of dissection, discharged from the hospital after ATBAD, approaching 25%
termed sub-acute (between 2 weeks and 3 months of the within 3 years.85 Most deaths in patients with CTBAD are
initial dissection), should be included. Additionally, the po- related to comorbid conditions.92
tential for endograft-stimulated aortic remodelling within
this time period is higher.98 3.2.2. Management. Aggressive medical therapy and close
In general, chronic type B aortic dissection (CTBAD) also surveillance of the aorta remains the cornerstone of
includes patients with a residual TBAD after repair of a type A management to reduce the risk of late mortality and
dissection (De Bakey type I dissection).168 Aortic related aortic complications. In a non-randomized, observational
complications may occur in 20e50% of patients with study, improved survival was shown in patients treated
CTBAD.169 Overall, it is estimated that approximately 20e40% with b-blockers in the chronic phase of AD. This study
of patients with CTBAD develop enlargement of the FL that reported an 80% freedom from aortic events at a mean
warrants treatment and approximately 25% of DTAA or TAAA of 4.2 years in patients on b-blockers, in comparison with
are associated with AD.170 Aside from aneurysmal degenera- 47% freedom from aortic events in patients treated with
tion, aortic related complications include recurrent dissection, other antihypertensive agents. The efficacy of other
retrograde dissection, and rupture of the FL. The expansion antihypertensive drugs has not been specifically demon-
rate of the chronically dissected aorta is not well known but strated in patients with CTBAD, although these drugs
ranges between 1 and 7 mm per year.171 Hypertension, an have a role in maintaining appropriate blood pressure
aortic diameter of 40 mm or more in the acute phase, chronic (systolic below 130 mm Hg and diastolic below 85 mm
obstructive pulmonary disease, and patency of the FL have Hg).80
been recognized as risk factors for late aneurysmal degener- Several studies have suggested that 40e70% of late
ation.169 In addition, the presence of an entry tear larger than deaths in patients with CTBAD are caused by comorbid
10 mm in diameter or located in the arch or proximal DTA has diseases, mainly heart disease and stroke,177 emphasizing
been recognized as a predictor of late mortality and the that cardiovascular risk factors should be thoroughly
consequent need for aortic repair.168 Moreover, in patients assessed and treated in this group. Interestingly, cigarette
treated for type A dissection with a patent distal FL, a large smoking does not seem to affect aortic expansion and
area of the FL (>70% of the total aortic area) is considered to rupture rates,178 although its detrimental role on cardio-
be a predictor of aneurysmal degeneration.202 vascular risk is well established.

In pa ents with chronic aor c dissec on, effec ve an hypertensive I C 80,83
therapy should be given to reduce the risk of aor c related death
RecommendaƟon 31
In pa ents with chronic dissec on, measures to reduce cardiovascular I C 177,179
risk (such as treatment of hyperlipidaemia, an -platelet therapy,
management of hypertension, and smoking cessa on) should be
implemented to reduce the incidence of late cardiovascular death
RecommendaƟon 32
Long-term medical treatment with β-blockers should be given to I C 80

pa ents with chronic uncomplicated aor c dissec on as they reduce
the progression of aor c dilata on, the incidence of subsequent
hospital admission, and the need for late dissec on related aor c
procedures
Conversely, FL thrombosis has been associated with 3.2.2.1. Indications for repair. The main goal of aortic repair
a slower growth rate.172,173 Interestingly, partial distal FL in patients with CTBAD is to avoid aneurysmal degeneration
thrombosis has been shown to be an independent and rupture of the aorta. The presence of malperfusion also
predictor of mortality after hospital discharge in patients mandates repair. Clinical and radiological factors may indi-
with a CTBAD.174 The responsible mechanism may be cate the need for treatment.
increased FL pressure resulting from a large entry tear New onset symptoms of AAS, such as lumbar back pain not
without outflow.175 In addition, an increased risk of rupture caused by other conditions, hypotension, a new peripheral
in aneurysms resulting from AD has been observed when pulse deficit or blood pressure differential, new focal neuro-
compared with degenerative aneurysms,176 although there logical deficit, or signs of end-organ malperfusion in patients
is insufficient evidence to support treatment at a lower with chronic dissection, mandate immediate evaluation to
diameter. exclude rupture or progression of the dissection.171
In asymptomatic patients, the maximum aneurysm of the intimal flap. This can lead to longer operation
diameter remains the most important indicator for treat- times with increased risk of bleeding, SCI, and renal
ment. A large aortic diameter is associated with increased failure.183
rupture risk, and several authors have shown that an aortic Aneurysms caused by chronic dissection are generally
diameter between 50 and 60 mm is associated with rupture more extensive than degenerative aneurysms and also
in almost 20% of cases.92,97,167,179 It should be acknowl- develop in younger patients. Several extracorporeal circu-
edged that there is a paucity of literature describing the lation methods and adjunctive measures for end organ and
In pa ents with chronic aor c dissec on and acute aor c symptoms, IIa C 171
emergency repair should be considered if malperfusion, rupture, or
progression of dissec on is confirmed on imaging
RecommendaƟon 34a
In pa ents with chronic aor c dissec on, a descending thoracic aor c IIb C 167,176
diameter between 56 and 59 mm may be considered as an indica on
for treatment in pa ents at reasonable surgical risk
RecommendaƟon 34b
In pa ents with chronic aor c dissec on, a descending thoracic aor c IIa C 97,167,176
diameter greater than 60 mm should be considered as an indica on
for treatment in pa ents at reasonable surgical risk
RecommendaƟon 35
In pa ents with chronic aor c dissec on and thoraco-abdominal IIa C 167,176

extension, an aor c diameter greater than 60 mm should be
considered as an indica on for treatment in pa ents at reasonable
surgical risk
aortic rupture risk in relation to aortic diameters in patients spinal cord protection have been described. Differing
with CTBAD. opinions exist regarding the use of these adjunctive
3.2.2.2. Open repair. Despite the lack of data regarding methods (see also Section 2.4.).
comparison between open and endovascular repair, OR In early reports on OR treatment, chronic dissection was
remains the standard treatment in low surgical risk patients a risk factor for paraplegia with an incidence approaching
with CTBAD because of an improvement in surgical results 30e35%.184 Indeed, these patients seem to have a different
over the last 20 years.41,54,180 It should be noted that pre-operative risk profile compared with those with
although several authors specifically report low surgical risk degenerative aneurysms, as they are more likely to present
as a prerequisite for OR, the definition of surgical risk in in an emergency/urgent fashion, have a connective tissue
thoracic aortic surgery is difficult. The most commonly used disorder, and be Crawford type I and II TAAA.183 With the
cardiac surgery risk models (European System for Cardiac introduction of surgical adjuncts, the negative predictive
Operative Risk Evaluation - EuroSCORE, Department of value of chronic dissection on paraplegia has been reduced
Veterans Affairs Continuous Improvement in Cardiac Sur- and, at present, is not considered a predictor of SCI in
gery Program - CICSP) report contradictory results when thoraco-abdominal aneurysm repair185 (see also Sections
applied to thoracic aortic surgery. In particular, a Japanese 2.4. and 3.4.2.2.).
study showed that the logistic EuroSCORE was able to In numerous contemporary, single centre series of pa-
reliably predict in hospital mortality in 327 consecutive tients with complex aortic pathologies treated by OR, re-
thoracic aortic procedures.181 Conversely, an American ported mortality rates range from 6% to 11%, with
study reported that both the EuroSCORE and the CICSP paraplegia/paraparesis being reported in 3.6e
greatly overestimated operative mortality rates in a series 12%.41,180,183e185 In contrast to high volume centres, na-
of 100 patients treated in a tertiary centre182 (see also tional registries and community based outcome analyses
Section 3.4.2.2.). suggest that the mortality for the overall treatment of dis-
The mainstays of surgical repair of chronic dissection sections by OR techniques is significantly higher and may
are similar to those of thoracic aortic aneurysms or exceed 20%.186 These data reinforce the recommendation
TAAA. Patients with chronic dissections typically require for centralisation of OR for chronic type B dissection, as well
a more complex operative repair because of the presence as for TAAA (see also Section 3.4.2.2.).
Open repair of aneurysmal or symptoma c chronic type B aor c IIa C 41,180

dissec on in pa ents with low surgical risk should be considered in
dedicated centres with low complica on rates
RecommendaƟon 37
In pa ents with chronic type B dissec on undergoing opera ve repair, IIa C 41,183
intra-procedural cerebrospinal fluid drainage, le heart bypass, and
moderate hypothermia should be considered to reduce procedural
mortality and spinal cord injury.
3.2.2.3. Endovascular repair. Since 1999, endovascular repair The advent of thoracic endografting has opened up a
has been used increasingly in the treatment of CTBAD.90 The potential therapeutic avenue for patients with uncompli-
goal of endovascular repair is to seal the proximal entry tear, cated sub-acute type B thoracic dissection. Without inter-
facilitate aortic remodelling to achieve thrombosis of the FL, ventional therapy, a substantial proportion of these patients
and thereby reduce the likelihood of aortic rupture in the mid- will go on to develop a large aneurysmal chronic dissection
to long-term. The technique of endovascular repair involves that may require therapy.169 Treatment of chronic dissec-
placement of a stent graft over the primary entry tear in the tions is difficult and from an endovascular perspective, re-
DTA, usually via a trans-femoral route. quires extensive thoracic coverage because of poor aortic
There are limited data comparing endovascular with OR for plasticity. It has been hypothesized that a subset of patients
CTBAD. A published series of 24 patients undergoing repair of with sub-acute dissection (between 2 and 12 weeks after
chronic dissection, showed mortality in the endovascular the index process), could be identified on the basis of
group of 0% compared with 33% in the open group.90 National morphological and physiological characteristics that pre-
registry data support an improved long-term outcome in pa- dispose to formation of aneurysmal chronic dissection.
tients undergoing endovascular repair of AD in comparison These patients might then be preferentially treated in the
with OR.186 More recently, larger series have supported the sub-acute phase.
concept that endovascular repair of CTBAD is associated with Patients with sub-acute TBAD have a low endovascular
lower morbidity. A recent systematic review of TEVAR for repair mortality rate and a significantly greater aortic plas-
chronic AD detailed 567 cases. In this review, the early (30 day ticity than patients with chronic dissections. Although
or in hospital) mortality was 3.2%, the incidence of stroke attractive as a hypothesis, the morphological features that
0.82%, and SCI occurred in 0.43%.187 predict late expansion are not fully defined189 and further
Endovascular repair of chronic dissection appears to be research is needed in this field.
associated with lower mortality and morbidity rates than The INSTEAD trial randomized patients with sub-acute
for AD, a difference attributable to the high incidence of and chronic dissections to early endovascular repair or
emergency procedures in the acute setting. There is now a best medical management and surveillance. Although
reasonably robust body of evidence to support the asser- aortic remodelling was greater in the endovascular group,
tion that endovascular repair of chronic dissections may be there were no statistically significant differences between
achieved with mortality rates below 5% and a low inci- the groups regarding 2 year mortality. Later follow up re-
dence of neurological complications, even in complicated sults have been published recently.190 It has been
cases.88,188 demonstrated that TEVAR, in addition to optimal medical
In the previously mentioned systematic review, the me- treatment, was associated with improved 5 year aorta
dian length of follow up was 26 months. The all cause specific survival and delayed disease progression. The au-
mortality rate was 9.2%. The mid-term aortic related mor- thors concluded that in cases of stable CTBAD with suitable
tality from those studies was 4.2%. The most common anatomy, preemptive TEVAR should be considered to
delayed complication was the development of aneurysms of improve late outcomes. A proportion of patients with sub-
the distal aorta or continuation of FL perfusion with aneu- acute dissection may, therefore, benefit from early endo-
rysmal dilatation (7.8%). Delayed aortic rupture was re- vascular repair,190 especially in those at risk of further
ported in 3.0%. No studies reported whether rupture aortic complications.168,169,172e174,189
occurred within or distal to the stented segment, or One issue that requires clarification is whether endo-
following persistent FL perfusion. Rates of complete FL vascular repair is effective in reducing aortic related death
thrombosis ranged from 38% to 100% (median 86%) in in the mid- to long-term. OR series demonstrate a high early
studies with a median follow up of 17 months. Studies aortic related mortality, but effective prevention of aortic
reporting mid-term follow up of aortic morphology related death in the long term.180 Data regarding preven-
observed a reduction of the FL diameter in 79% of patients, tion of aortic related death are less robust for endovascular
FL expansion occurred in 15%, and TL expansion in 66%. techniques. A series of 76 patients reported 12 late deaths.
Data from this review should be interpreted carefully Only one was defined as aortic related (rupture of an un-
because of the poor quality of the studies included.187 treated ascending dissection).191
The ability of the aorta to remodel after endovascular segment of the aorta between the LSA origin and the dia-
repair of acute and chronic dissections has been studied. phragm (see also Section 2.1. for normal values). Histo-
Following coverage of the primary entry tear in ATBAD, pathologically, it is characterized by medial degeneration,
there is rapid aortic remodelling with an increase in with disruption and loss of elastic fibres and increased
diameter of the TL and a reduction in FL diameter.91,192 deposition of proteoglycans, with or without
By contrast, in chronic dissections, there is less aortic atherosclerosis.
remodelling and the integrity of the endovascular repair At any given increased size of various aortic segments,
is determined by the ability of the endovascular proce- the expansion rate is greatest in the DTA. There are many
dure to induce FL thrombosis. In treating chronic dis- other factors that affect the expansion rate apart from
sections, it has been shown that FL thrombosis occurs diameter and anatomical location including smoking,
more often in patients with limited dissection and that FL intraluminal thrombus, chronic obstructive pulmonary dis-
thrombosis occurs more effectively in the stented part of ease (COPD), and vascular disease.195 Once diagnosed, the
the DTA.191 prognosis of large degenerative aneurysms (more than
These findings suggest that the extent of coverage of the 60 mm in diameter) is poor if not treated, with a 3 year
DTA is directly related to the extent of FL thrombosis. survival of approximately 20%.196
Although SCI rates after TEVAR for chronic dissection are
lower than those after TEVAR for thoracic aneurysms, 3.3.2. Management. The rate of expansion is important in
increased aortic coverage may increase the risk of SCI. To deciding the frequency of surveillance in DTAA. Risk factors
reduce SCI rates, spinal cord perfusion should be main- for rupture include maximum aneurysm diameter, age,
tained whenever possible through collateral channels, gender, active smoking status, diastolic hypertension, and
especially from the LSA. Irrespective of the extent of aortic aneurysm related pain.9,176,196,197
coverage, the dissected portion of the aorta below the 3.3.2.1. Indications for repair. An initial diameter of 60 mm
diaphragm remains untreated, and there is evidence to carries an annual risk of rupture of 10%. For DTAA, there is a
suggest that this region may dilate over time.91 The role of threshold of 70 mm at which the risk of rupture suddenly
bare stents in the treatment of chronic dissection, and the escalates.127 Intervention in aneurysms below 55 mm may
effect that these stents have on aortic remodeling, remain not afford a survival benefit, although a randomized
undefined at the present time.99 controlled trial is necessary to evaluate the possible benefit
The available literature regarding TEVAR for chronic of repair in the small aneurysm group.9 This threshold can
dissection in patients with MFS is sparse.193 Early technical be reduced to 50e55 mm for women or in the setting of
feasibility has been demonstrated, but long-term outcomes connective tissue disorders (see also Section 3.7.1.).54,88
remain uncertain. The fragility of the aorta in MFS and other 3.3.2.2. Open repair. Indications for OR of a DTAA are
associated conditions poses a problem for proximal aortic limited to fit patients (see also Section 3.3.2.3.) unsuitable
fixation and may lead to the development of complications for TEVAR such as those with:
(see also Section 3.7.1.1.).
Recently, new endovascular techniques have been tested 1) Absence of adequate arterial access197 or a
to induce FL thrombosis in chronic CTBAD. Despite the contraindication to aortic/iliac conduit graft placement
feasibility and the early usefulness that have been (presence of hostile abdomen, severe aorto-iliac
demonstrated in selected patients in highly experienced disease). The use of an aortic/iliac sidearm graft is
centres, it is too early for widespread adoption and for a required in approximately 15% of cases.54
general recommendation statement.194 2) Absence of proximal or distal landing zones.
In pa ents with moderate to high surgical risk or with contraindica ons IIa C 187
to open repair, endovascular repair of complicated chronic type B
aor c dissec ons should be considered in dedicated centres
RecommendaƟon 39
In pa ents at risk of further aor c complica ons with suitable anatomy IIa B 190
for endogra ing, endovascular repair of uncomplicated chronic type B
aor c dissec ons should be considered in the sub-acute phase, in
dedicated centres
3.3. Descending thoracic aortic aneurysms 3) DTA associated with a connective tissue disorder such
3.3.1. Definition and natural history. Descending thoracic as MFS, or DTA in young, healthy patients without
aortic aneurysms (DTAA) are defined as any aortic dilatation major contraindications for OR.54,88
with at least a 50% increase in diameter located in any 4) Prohibitively high risk of neurological deficit post-TEVAR
caused by full extent DTAA in patients with previous
abdominal aortic surgery, reducing pelvic circulation and The risk of post-operative renal failure is higher in pa-
occlusion of the lumbar arteries.45,198e200 tients with impaired renal function. This scenario can be
5) Symptoms related to compression by a large DTAA of successfully managed by pre-operative hydration and
adjacent structures such as the thoracic vertebral bodies adequate renal perfusion during the peri- and post-
(chronic pain syndrome), trachea or left mainstem operative period.206
bronchus (dyspnea), or oesophagus (dysphagia).201 There is robust evidence showing that the risk of SCI,
The extension and the location of the disease affect the mesenteric and renal ischaemia is related to the duration of
surgical decision making process in terms of type of incision cross clamping, and that cross clamp time represents the
and use of peri-operative adjunctive measures. A classifi- most important predictor of post-operative neurological
cation has been proposed to define the risk of SCI during deficit.28,31,114,206 Methods of extracorporeal circulation
DTAA repair. Such a classification distinguishes three types include LHB and cardiocirculatory arrest. This more
of DTAA: type A, which involves the proximal DTA and ends aggressive approach can prove useful in the presence of
at the level of T6; type B, which involves the distal portion rupture or when proximal aortic clamping entails a high
of the DTA starting at the level of T6; and type C, which risk.207 However, in a retrospective analysis of 387
affects the entire DTA202 (see Fig. 2). consecutive DTAAs, no significant paraplegia risk reduction
Pre-operative planning should include a CTA or MRA eval- was found by applying LHB in DTAA OR114 (see also Section
uation of the spinal cord circulation including lower lumbar 2.4.2 for prevention of spinal cord ischaemia).
and pelvic arteries, which play a major role in spinal cord Outcomes following elective OR for DTAA have improved
blood supply in 16% and 8% of cases, respectively.198,199 over the past 25 years in some high volume cen-
The pre-operative assessments of cardiac, pulmonary, tres.114,208,209 However, these acceptable results were not
and renal function, as well as carotid and peripheral arterial corroborated in an American National Inpatient Sample
occlusive disease are essential to minimize the risks of administrative database, presumably reflecting results ach-
mortality and morbidity associated with DTAA repair. ieved outside centres of excellence. Mortality rates in
Additional studies such as echocardiography, cardiac cath- elective and emergency cases were 10% and 45%, respec-
eterisation, and, in selected cases, Holter 24-hour electro- tively. Furthermore, spinal cord ischaemia has also been
cardiography monitoring, are usually requested for patients reported in the range of 11e15%.210
with a history of coronary or valvular heart disease. 3.3.2.3. Endovascular repair. Evidence comparing OR and
Symptomatic coronary artery disease (CAD) is always TEVAR in the treatment of DTAAs relies on systematic re-
treated aggressively before aortic surgery. The management views and meta-analysis of retrospective series and non-
of asymptomatic patients with CAD remains controversial randomized controlled or population based studies.211e215
given recent evidence suggesting a more conservative The available results decisively favour a less invasive
approach203 compared with older data.204 In the evaluation approach in terms of mortality, morbidity, and length of
of such cases, the extent of the planned procedure and the hospital stay. These results were, however, tempered by
severity of the CAD determine the pre-operative strategy. several less impressive results, such as long-term survival.213
A history of smoking and the presence of COPD increase In a meta-analysis of 17 studies with 517 elective pa-
the risk of post-operative respiratory failure. Pulmonary tients treated by OR and 538 patients treated by TEVAR,
function tests and arterial blood gas analyses should be the endovascular cohort demonstrated a lower 30 day
performed in all patients.205 mortality rate (5.57% vs. 16.5%). TEVAR was also associated
Figure 2. Classification of DTAA according to the extension.202

Computed tomographic angiography or magne c resonance I C 198,199

angiography should be performed for pa ents planned for descending
thoracic aor c repair, to define the extent of the disease and to
iden fy the poten al risk of the procedure related to the main
intercostal arteries
RecommendaƟon 41
In candidates for open surgical repair of descending thoracic aor c IIa C 204
aneurysms with a past medical history of coronary artery disease,
addi onal diagnos c inves ga ons should be considered to define the
severity of the underlying heart disease
RecommendaƟon 42
Revascularisa on of symptoma c or severe asymptoma c coronary IIa C 203
artery disease should be considered before open descending thoracic
aorta surgery
RecommendaƟon 43
Stra fica on of peri-opera ve risk associated with pulmonary, renal, IIa C 31,205,206
and cerebrovascular studies should be considered
RecommendaƟon 44a
Open repair may be considered for fit pa ents, with a descending IIb C 88,197
thoracic aorta between 56 and 59 mm in diameter, who are unsuitable
for endovascular repair
RecommendaƟon 44b
Open repair should be considered for fit pa ents, with a descending IIa C 88,197
thoracic aorta exceeding 60 mm in diameter, who are unsuitable for
endovascular repair
RecommendaƟon 45
To reduce the incidence of postopera ve paraplegia, le heart bypass is III C 114

not recommended for pa ents undergoing open repair for descending
thoracic aneurysm
with a significant reduction in peri-operative mortality in the development of SCI.216e218 In the presence of one or
(pooled OR [odds ratio] 0.36, 95% CI 0.228e0.578; more such risk factors, CSF drainage should be performed
p < .0001). Major neurological complications occurred in to reduce the risk of SCI post TEVAR (see also Section
26 TEVAR patients (5.4%) and 67 OR patients (14%). 2.4.3.).
Endovascular repair was associated with a reduced risk of Intentional coverage of the LSA during TEVAR to provide
paraplegia (pooled OR [odds ratio] 0.33, 95% CI 0.18e0.63, adequate proximal sealing and fixation has been reported in
p < .0007). The risk of stroke was not affected by TEVAR 10e50% of cases. A robust body of evidence, based on
(pooled OR [odds ratio] 0.77, 95% CI 0.38e1.59, p ¼ .48). observational studies, shows that this increases the risk of
TEVAR had no impact on the major re-intervention rate at stroke, SCI, and upper limb ischaemia.44e50 An additional
48 months (pooled OR [odds ratio] 0.91, 95% CI 0.610e meta-analysis reported an almost doubled rate of cerebro-
1.619), which was 8.4% versus 7% for OR and TEVAR, vascular events (4.7% vs. 2.7%, OR [odds ratio] 2.28, 95% CI
respectively.212 1.24e4.09) and SCI (2.8% vs. 2.3%, OR [odds ratio] 2.39,
SCI can occur after TEVAR. A recent observational study 95% CI 1.30e4.39, p ¼ .005), when the LSA was covered219
on 424 DTAA treated by TEVAR reported 12 cases of SCI (see also Sections 2.4.3. and 2.4.4.).
(2.8%).216 Several reports confirm that specific anatomical An inadequate distal landing zone of less than 15 mm length
conditions may represent potential risk factors for post- and a proximal neck diameter exceeding 40 mm are contra-
operative SCI. These include planned coverage of the indications for TEVAR with currently available devices.215,220
thoracic aorta over more than 200 mm or involving the level These anatomical limitations occurred more commonly than
T8-T12, previous abdominal aortic surgery with ligation of the rate of 4.4% previously reported in a multicentre review of
lumbar arteries, an occluded hypogastric artery, and, in the TEVAR.220 In spite of these data, safe coverage of the CA has
emergency setting the need for LSA coverage not amenable been described to achieve acceptable distal sealing and fixa-
to revascularisation. Chronic renal failure and a peri- tion.221 CTA is suitable to assess anatomical details of the distal
operative MAP lower than 70 mm Hg may also play a role DTA and the proximal abdominal aorta; however, selective
angiography of the CA and SMA remains the most reliable test entire abdominal aorta, with or without extension to the iliac
to predict the role of potential collateral circulation following arteries. A type V TAAA starts below the sixth intercostal space
CA coverage during TEVAR.221 and tapers just above the renal arteries (Fig. 3).222
Contraindications to CA coverage include the presence of Using this classification, an international consensus has
a common coeliaco-mesenteric trunk, the absence of been achieved on describing the extent of aneurysmal
adequate angiographic evidence of collateral circulation, disease. Both in open and endovascular repair, this classi-
poor portal vein perfusion, and any previous conventional fication provides an indication of the extent of the proce-
or endovascular procedure that may have compromised the dure, the likely technical challenges involved in any repair,
collateral circulation.221 and the potential post-operative risks. For example, a type
An alternative solution to CA coverage in certain situa- IV TAAA can technically be performed by laparotomy only,
tions is a scallop-designed endograft or the use of a “snorkel whereas all the other types require a thoraco-abdominal
or chimney technique”. The use of these techniques is incision, (partial) incision of the diaphragm, and tempo-
promising, although supported by only few data. rary collapse of the left lung, reflecting the potential for
RecommendaƟon 46a Class Level of evidence References

In fit and unfit pa ents with favourable anatomy, endovascular repair IIb B 88,211–215
may be considered for descending thoracic aorta aneurysms between
56 and 59 mm diameter
RecommendaƟon 46b
In fit and unfit pa ents with favourable anatomy, endovascular repair IIa B 88,211–215
should be considered for descending thoracic aorta aneurysms >60
mm diameter
3.4. Thoraco-abdominal aortic aneurysms substantial differences in surgically induced trauma be-
3.4.1. Definition. Crawford first described the extent of tween these two exposures. Furthermore, the five types are
thoraco-abdominal aortic aneurysms (TAAA) and his classifi- each associated with different end organ complications
cation has found general acceptance. Type I TAAAs start at the such as paraplegia, renal failure, and visceral ischaemia.
level of the LSA, or at least proximal to the level of the sixth
vertebra (T6) and affect the entire DTA, involve the visceral 3.4.2. Management.
arteries and end at the renal arteries.The aneurysm can involve 3.4.2.1. Indications for repair. It is extremely important to
the origin of the LSA or even involve the distal aortic arch. Type assess the risk of any intervention against the risk of
II TAAAs start at the same level as a type I, but involve the DTA rupture. Extensive pre-operative risk analysis is required as
and the entire abdominal aorta. In many patients, especially an essential part of the decision making process, especially
those suffering from connective tissue diseases and post- in patients with comorbidities. General consensus exists
dissection aneurysms, the iliac arteries are aneurysmal as regarding surgical repair of TAAA for low to moderate sur-
well. Type III TAAAs start more distal than type II aneurysms, gical risk patients with TAAA larger than 60 mm (less for
usually at the level of T6, and extend distally as in a type II.Type patients with connective tissue disorders), rapid growth
IV TAAAs start at the level of the diaphragm and involve the (>10 mm/year), or with symptoms.112,223
Figure 3. Crawford TAAA classification modified by Safi.222

RecommendaƟon 47 Class Level References

Open or endovascular repair should be considered for pa ents at low IIa C 223
to moderate surgical risk, with an atherosclero c or degenera ve
thoraco-abdominal aor c aneurysm of 60 mm or larger diameter,
rapid aneurysm enlargement (>10 mm/year), or aneurysm related
symptoms
3.4.2.2. Open repair. Open TAAA repair is a major aortic of efficacy, compelling data exist to support the use of CSF
intervention and is associated with post-operative compli- drainage along with methods of aortic perfusion distal to
cations including myocardial infarction, respiratory failure, the cross-clamp.230 A multimodal approach including CSF
renal insufficiency, stroke, paraplegia, and death. To esti- drainage, maintenance of adequate MAP, LHB, and revas-
mate the risks of OR, pre-operative cardiopulmonary and cularisation of intercostal arteries seems to be effective in
renal assessment is required. Patients with degenerative reducing the risk of SCI.232
TAAA are usually older than patients with post-dissection or The majority of publications on the results of open TAAA
connective tissue disease aneurysms. A substantial number repair originate from individual series, coming from highly
(34e40%) will suffer from CAD, and compromised cardiac specialized centers.30,225,227 The results of these experienced
function is a strong predictor of post-operative mortality.224 centres report mortality rates varying between 5% and 15%.
In patients with COPD and in smokers, pulmonary function Major complications include respiratory failure (up to 60%),
is significantly reduced in 23e36%, leading to oxygenation neurological deficits (3e18%), and renal failure (3e15%).
problems during peri-operative single-lung ventilation, Outside highly specialized centres, mortality and morbidity
prolonged post-operative ventilatory support, and pneu- rates are almost doubled. A United States state-wide data-
monia.225 Pre-operative spirometry and blood gas analysis base showed 30 day and 1 year mortality rates after elective
can detect pulmonary compromise, and appropriate respi- TAAA repair of 19% and 31%, respectively.233 These results
ratory training programmes and medical treatment can be indicate that these complex procedures should be performed
initiated to improve lung function. Pre-existing renal insuf- only in specialized centres. Indeed, adequate staff support
ficiency is also a predictor of post-operative renal failure and hospital and surgeon volume significantly influence post-
and mortality and should be taken into account when operative survival. Another study234 clearly demonstrated
considering OR.226 that less experience correlated with increased post-operative
In OR, proximal aortic cross-clamping causes increased mortality: in 1,542 operated patients, a significant difference
pre- and after-load on the heart. In addition, all organs and (p < .001) in mortality was determined between low volume
tissues distal to the clamp will be excluded from the cir- hospitals (27.4%) and high volume hospitals (15%). Also, low
culation, including the spinal cord, visceral organs, kidneys, volume surgeons had significantly (p < .001) higher mortality
and legs. The application of extracorporeal circulation with rates (25.6%) compared with high volume surgeons (11.0%).
distal aortic perfusion has reduced ischaemic complications. These volume outcome relationships were summarized in a
Several techniques for extracorporeal circulation can be systematic review.235
used, including LHB by means of a left atriofemoral bypass 3.4.2.3. Endovascular repair. Despite improvements in peri-
circuit or femoral veno-arterial bypass40,227 (see also Sec- operative care and various surgical adjuncts, cardiopulmo-
tions 2.4.2. and 3.3.2.2.). nary, renal, and neurological complications such as SCI are
During TAAA repair, the CA and the SMA should be still common problems after open TAAA repair. Thus,
perfused with blood and both renal arteries should be alternative managements have been sought, with two ap-
perfused with either cold crystalloid or blood to reduce proaches currently under evaluation.
end-organ ischaemia.228 Despite protective measures such In the first, defined as the “hybrid approach”, visceral
as antegrade mesenteric perfusion, or retrograde perfusion perfusion was safeguarded by means of an OR extra-
with sequential clamping, mucosal damage can still occur anatomic bypass, followed by endovascular exclusion of
and inflammatory reactions are induced.229 the entire aneurysm. This approach has the advantage of
Risk factors for the development of SCI include prolonged limiting exposure to a laparotomy while avoiding a tho-
aortic cross clamp time, extensive aneurysmal disease, racotomy, although, this remains a considerable under-
aortic dissection, emergency surgery, internal iliac artery taking in unfit patients. The issue of a single stage versus
exclusion, and previous abdominal aortic procedures. two stage strategy has been debated, with higher mor-
Several strategies to limit neurological complications tality and renal failure rates associated with one stage
include distal aortic perfusion, intercostal artery reimplan- procedures and an increased risk of death resulting from
tation,37 CSF drainage,29,37,230 spinal cord cooling,231 and rupture while awaiting the second stage.236 Early results,
assessment of spinal cord function peri-operatively30 (see in terms of mortality, SCI, and morbidity rates, generated
also Section 2.4.2.). considerable enthusiasm.237 Recent data on 5 year
In two recent reviews, all available strategies to prevent patency rates were also encouraging.238 Currently, the
SCI were summarized.37,230 Despite lacking definitive proof hybrid technique is usually restricted to patients with no

In pa ents planned for open or endovascular thoraco-abdominal aor c I C 224–226
aneurysm repair, pre-opera ve cardiac, pulmonary, and renal func on
should be assessed to es mate surgical risks and ini ate therapy to
improve the pre-opera ve status
RecommendaƟon 49
In open type I, II, and III thoraco-abdominal aor c aneurysm surgery, IIa C 227,228
extra-corporeal techniques allowing distal aor c and organ perfusion
should be considered to reduce ischaemic complica ons, especially in
extensive aneurysms requiring prolonged cross clamping me
RecommendaƟon 50
In pa ents with extensive thoraco-abdominal aor c aneurysm (type I, IIa B 29,37,230
II, III) undergoing open repair, cerebrospinal fluid drainage should be
considered as a measure to decrease the risk of neurological deficit
RecommendaƟon 51
An integrated approach with op misa on of mean and distal aor c IIa C 232
arterial pressure, moderate hypothermia, neuromonitoring, and
reimplanta on of intercostal arteries should be considered to protect
the spinal cord during thoraco-abdominal aor c aneurysm surgery
RecommendaƟon 52
Centralisa on of open repair of thoraco-abdominal aor c aneurysm in IIb C 234,235
dedicated high volume centres may be considered
other reasonable options, such as emergency cases or high endovascular repair appears to be durable with accept-
risk patients with anatomy unfavourable for a branched able secondary re-intervention rates. One year survival
endograft. rates between 76% and 87% were reported and were
The second technique (currently in evolution) is a total largely dependent on patient selection.239e243
endovascular repair using specifically constructed branched The pre-operative clinical characteristics of the patients
modular aortic grafts. The rationale for this approach grew out investigated in these series may mean there is a negative
of the promising initial experience with fenestrated grafts, bias towards treating patients with greater comorbidities.
which were developed to treat juxta-renal aneurysms by Therefore, the outcome of TAAA patients managed with a
endovascular means.239 Preservation of visceral flow is ach- minimally invasive approach, although associated with
ieved by means of either fenestrations or branches or a positive results, still has a consistent rate of morbidity and
combination of both on the component deployed in the region mortality. Patients undergoing these procedures, most of
of the visceral arteries. The aortic anatomy is still a limiting whom have been rejected for OR, remain vulnerable in the
factor, but the progress in imaging and device conception has post-operative period. The relatively significant risk of in
begun to overcome this challenge. Initial reports of the tech- hospital death with advancing age and chronic renal failure
nique have all been from single centre studies and are non- underscores the importance of excluding patients with poor
randomized.240e242 Many patients are deemed unfit for OR of physiological status and limited life expectancy. By contrast,
TAAA, particularly older patients, and this cohort represents the encouraging mid-term results suggest that this tech-
the group that have endovascular TAAA repair. nique has a role in relatively unfit patients, especially when
The published 30 day mortality rates range from 5.2% to performed in high volume centres.239e243
9.7% in the larger series (2.3%e2.6% for type IV TAAA).240e
243
Rates of SCI and cardiopulmonary and renal morbidities
appear to be largely in concordance with rates observed 3.5. Inflammatory diseases of the descending thoracic
following OR.240e243 The largest series of endovascular aorta
repair of TAAA (406 patients) reported similar paraplegia 3.5.1. Takayasu arteritis. Takayasu arteritis (TA), also known
risks between endovascular and OR (4.3% endovascular as pulseless disease, is a rare form of large vessel granu-
repair vs. 7.5% OR, respectively, p ¼ .08).241 The SCI risk lomatous vasculitis with massive intimal fibrosis and
was associated with COPD, procedure duration, extent of vascular narrowing, or destruction of the elastic fibres and
the aortic disease, and prior aortic surgery. Clinical aneurysm formation.244
improvement after SCI has been reported.240 The pathogenesis of TA is poorly understood. Some evi-
As in open procedures, patients with type I, II, or III dence suggests that a viral/bacterial infection of some kind,
TAAAs have a higher risk of post-operative SCI and death, in a person with other predisposing factors (such as genetic
when compared with type IV. In the short- to mid-term, disorders), may lead to this disease.245

For thoraco-abdominal aneurysm repair, in pa ents unfit for open IIa C 239–243
repair, an endovascular procedure should be considered
RecommendaƟon 54
For thoraco-abdominal aneurysm repair in pa ents unfit for open IIa C 237,238
repair with aor c anatomy unfavourable for a branched/fenestrated
endogra , a hybrid approach should be considered
RecommendaƟon 55
Centralisa on of endovascular repair of thoraco-abdominal aor c IIb C 242

aneurysm in dedicated high volume centres may be considered
The American College of Rheumatology proposed the tapered over several weeks to a dose that is more tolerable
following criteria for diagnosing TA: for the patient, with respect to the decrease of C-reactive
protein and erythrocyte sedimentation rate (ESR). For life-
1) Age of onset younger than 40 years. long treatment, methotrexate, azathioprine, and even
2) Intermittent claudication. cyclophosphamide are sometimes used in addition to
3) Diminished brachial artery pulse. prednisone.
4) Subclavian artery or aortic bruit. There are few data in the literature to identify TA pa-
5) Systolic blood pressure variation greater than 10 mm Hg tients who require operation. Evidence for surgical or
between arms, and endovascular repair of thoracic aortic disease in patients
6) Angiographic (CT, MR) evidence of aortic or aortic with TA is extremely limited. It seems acceptable to main-
branch vessel stenosis. tain the same indications as in non-inflammatory disorders,
based on the risk of rupture related to aneurysm dimension,
The presence of three or more of these six criteria and the presence of secondary organ vascular insufficiency.
demonstrated a high sensitivity and specificity for the In an 8 year study including 11 patients with TA (n ¼ 3),
disease.245 GCA (n ¼ 2), and MFS (n ¼ 6), endovascular repair of
The clinical manifestation of TA is typically described in complicated aortic aneurysms proved safe and feasible.246
two phases: 1) a systemic “acute” phase, and 2) a “chronic” However long-term durability in this younger group of pa-
phase. These two phases are not always distinct and patients who have an ongoing risk of arterial degeneration
tients may have features of both phases at the same time. remains to be determined.
In the systemic phase, patients have symptoms and signs of
an active inflammatory disease. These may include 3.5.2. Giant cell arteritis. Giant cell arteritis (GCA), also
“constitutional symptoms” (fever, fatigue, weight loss), known as temporal arteritis or cranial arteritis,247 is a sys-
arthritis, and non-specific aches and pains. This phase is temic, inflammatory, vascular syndrome that affects the
succeeded by the chronic phase, typical for symptoms aorta and its secondary and tertiary branches.
presenting in affected organs. Patients may experience Although arteritis is a basis for aneurysms, dissections,
claudication of upper and/or lower limbs, symptoms and stenotic lesions of the aorta and its major branches,
associated with cerebrovascular insufficiency (dizziness on patients with GCA experienced similar mortality rates to the
standing up, headaches, visual problems, transient ischae- general population of similar age and gender.248
mic attack, or stroke), hypertension secondary to renal The aetiology of GCA is unknown, but the pathogenesis is
artery involvement, or aortic regurgitation caused by similar to TA and involves a chronic inflammatory process,
aortitis. which releases several cytokines, including T-lymphocyte
The thoracic aorta itself may be involved in the inflam- products interferon-gamma (IFN-gamma) and interleukin
matory process and develop either aneurysms or stenoses. (IL)-2. The T-cell clonal expansion suggests a specific anti-
The diagnosis is confirmed by CTA or MRA, which show genic response, which is still unclear (viral, bacterial, or
stenosis and dilation of the aorta, its branches, or both. other). Concentric intimal hyperplasia is an important un-
Thickening of the aortic wall detected by MRI or ultraso- derlying pathologic lesion in GCA.249
nography can precede the angiographic changes. Clinical presentation varies from fever of unknown origin
Treatment of TA is based principally on corticosteroids. to constitutional symptoms. Arterial lesions may be wide-
The great majority of patients respond to prednisone, spread and the variable expression of GCA can be analyzed
which is effective for the systemic symptoms and can according to the anatomical pattern of the arteries affected.
impede further progression of the vasculitis. The usual Polymyalgia rheumatica (PMR) and GCA may represent two
starting dose is approximately 1 mg/kg of body weight per parts of a single disease spectrum, with GCA at the more
day. Because of the significant side effects of long-term severe end. The two processes share certain constitutional
high dose prednisone use, the high starting dose is symptoms, including fatigue, weight loss, and fever.
The following are criteria for GCA defined by the Amer- to aneurysm dimension, and the presence of secondary organ
ican College of Rheumatology in 1990247: 1) age 50 years or vascular insufficiency related to fibrotic stenosis.
older; 2) new onset localised headache; 3) temporal artery
tenderness or decreased temporal artery pulse; 4) ESR of at 3.5.3. Behçet disease. This syndrome carries the name of
least 50 mm/h; and 5) abnormal arterial biopsy specimen the Turkish dermatologist Hulusi Behçet, who, in 1937,
characterized by mononuclear infiltration or granulomatous described a syndrome of recurrent aphthous ulcers, genital
inflammation. The presence of at least three criteria yields a ulcers, and uveitis.256 Vascular manifestations may vary and
diagnostic sensitivity of 93.5% and specificity of 91.2% when depend on the type and location of the vessel involved. Any
compared with patients who were clinically classified as large or small artery, vein, or organ may be involved in an
having the disease. In the case of localisation of disease to unpredictable combination. The most common vascular
the temporal artery, biopsy is diagnostic in the majority of complaints are secondary to venous thrombosis, often of
cases, although its sensitivity declines with the initiation of the superficial veins.
steroid therapy.250 Surgical and endovascular repair of aneurysm in Behçet
A population based study over 50 years in patients with disease has been described as feasible. After surgical repair,
GCA revealed a 31% cumulative frequency of large artery a high recurrence rate of anastomotic false aneurysm has
complications, including aortic aneurysm or aortic dissec- also been described, caused by the friability of the diseased
tion in 18%, and/or large artery stenosis in 11%.251 Presence arteries. Moreover, any surgery may exacerbate Behçet
of thoracic aortic dissection was associated with markedly disease, so patients must be prescribed immunosuppressive
increased mortality (median survival of only 1.6 years after therapy before and after the surgical intervention.257
diagnosis of GCA).252 Because pseudoaneurysms may develop at arterial punc-
A comparative study suggested that a steroid regimen, ture sites and veins may be quickly thrombosed after in-
starting with 30e40 mg/day and tapering to 10 mg/day jection of contrast material, angiography and venography
within 6 months and to 5e7.5 mg/day within 1 year, is should be avoided whenever possible.258
effective and better tolerated in the patient population, than 3.5.4. Other inflammatory aortitides. Other chronic dis-
the two higher dose regimens (40e60 or >60 mg/day).253 eases with potential inflammatory involvement of the
In refractory cases, the use of immunosuppressants, such as thoracic aorta include rheumatoid arthritis, sarcoidosis,
methotrexate (15e25 mg/week) or azathioprine (2 mg/kg/ Cogan syndrome, Kawasaki disease, ankylosing spondylitis,
day), as potentially steroid-sparing disease controlling drugs, systemic lupus erythematosus, and Wegener’s gran-
has been considered and has shown conflicting results.254 The ulomatosis.259 Some types of aortitides remain idiopathic.
newer tumour necrosis factor antagonists are being evaluated Cardiovascular involvement in these patients is variable,
in clinical trials, but initial studies of infliximab did not show consisting of aortic valve regurgitation, aneurysm forma-
promising results.254 Encouraging results have been reported tion, dissection or stenosis of the aorta or its initial
from the use of low dose aspirin for prevention of visual loss branches. Localisation of the inflammatory disease at the
and stroke in patients with GCA.255 level of the aortic root with consequent aortic valve
Indications for surgical or endovascular repair in patients regurgitation is the most common cardiovascular compli-
with GCA are similar to non-inflammatory disorders, and the cation in these patients. The involvement of the DTA is
decision in each patient is based on the risk of rupture related extremely rare but potentially more prone to rapid growth

Cor costeroids should be the first line of treatment in pa ents with I C 253,254
inflammatory diseases associated with thoracic aor c disease
RecommendaƟon 57
For a pa ent with an inflammatory disease of the descending thoracic IIa C 253
aorta, tapering the star ng cor costeroid dose over several weeks to
a dose that is acceptable for the pa ent, with respect to the decrease
of inflammatory markers, should be considered to avoid significant
side effects of long-term high dose cor costeroid use
RecommendaƟon 58
Selec ve repair should be considered for pa ents with thoracic IIa C 246,257
inflammatory disease based on the risk of rupture related to aneurysm
size, and the presence of secondary end organ ischaemia
RecommendaƟon 59
In pa ents with an inflammatory disease of the descending thoracic IIa C 257
aorta undergoing surgical interven on, applica on of a higher dose of
cor costeroids and/or immunosuppressive agents before and a er
the interven on should be considered
and rupture.260 In particular, systemic lupus erythematosus CAD, or complications associated with valvular or cerebral
has been suggested as possibly associated with thoracic AD, disease.264
especially in patients with persisting inflammation while on After intervention, hypertension resolves in two thirds
chronic steroid therapy.260 of patients and the majority of them will benefit from
improved ventricular function, reduced ventricular mass
3.6. Coarctation of the thoracic aorta in adults and improved ascending aortic distensibility.265 Inter-
3.6.1. Definition and diagnosis. Aortic coarctation is a vention should be balanced between the anticipated
congenital defect characterized by stricture of the aortic benefits and the patient’s life expectancy, surgical risk
lumen typically located at the region of the ligamentum and comorbidities. In adults the pathological changes
arteriosum. This narrowing is usually localised, but may resulting from aortic coarctation may no longer be
involve other segments of the aorta such as the arch and, reversible.265,266
less often, the abdominal aorta. There is significant variation 3.6.2.1. Indications for repair. Treatment is indicated for
in the degree of stenosis, extent of disease, and clinical symptomatic patients and should be considered for
manifestations. Generally, an extensive collateral circulation asymptomatic patients with a trans-coarctation gradient
is present in adults. >20 mm Hg or persistent upper limb hypertension (systolic
Men are more often affected (2:1).261 Clinical manifesta- pressure >140 mm Hg or diastolic pressure >90 mm Hg).
tions include upper limb hypertension, weak and delayed These thresholds are based on expert opinion only, but are
femoral pulses, and lower limb claudication. A thoracic bruit supported by clinical evidence suggesting that intervention
may be present. In advanced cases, heart failure or intra- before large gradients are present may confer a higher
cranial haemorrhage commonly develop. When the aortic chance of reversing left ventricular dysfunction and pre-
gradient is less pronounced or collaterals are well developed, venting irreversible loss of aortic wall compliance.265,266
symptoms may be absent or mild. Other cardiovascular ab- There are two potential invasive treatment strategies,
normalities include ventricular septal defects, bicuspid aortic open and endovascular surgery; however, given the rarity of
valve, sub-aortic stenosis, aortic medial disease, aortic arch patients requiring invasive treatment, there are no ran-
hypoplasia, patent ductus arteriosus, and intracranial aneu- domized trials comparing these two options. Case series
rysms.262 Adult patients most commonly present with and non-randomized studies suggest marginally increased
discrete forms of the disease, which may remain occult peri-operative morbidity rates with OR, and greater recur-
during childhood and have no association with congenital rence rates after endovascular repair.265e267 Regardless of
cardiac abnormalities, or present with recurrence after treatment strategy, centralisation of treatment into expert
intervention. There are currently no data on the exact inci- centres should be considered.
dence of coarctation manifesting in adulthood. 3.6.2.2. Open repair. OR is the classical method of treatment
Both CTA and MRI provide anatomical evidence of for aortic coarctation. The most common techniques include
coarctation and are the investigations of choice for defini- resection and end to end anastomosis, or an aortic interpo-
tive diagnosis. The aortic gradient may be directly measured sition graft. Alternative techniques have been proposed, such
by catheterisation, or indirectly estimated using Doppler as patch or subclavian flap aortoplasty, or ascending to
echocardiography or cardiovascular MR.263 descending aortic bypass with or without valve replacement,
While TTE is usually diagnostic in children, it is often but are generally only performed in expert centres.267
inconclusive in adults. However, TOE is the method of Operative mortality after OR is generally very low (<1%).
choice to evaluate cardiac sequelae such as ventricular Complications of OR include rebound hypertension, phrenic
hypertrophy and left ventricular dysfunction, and associa- and recurrent laryngeal nerve injury, recurrence of coarcta-
tion with congenital cardiac abnormalities. CTA is a good tion, and aneurysm or pseudo-aneurysm formation at the
alternative to MRA, despite absence of functional infor- patch site. Rarely, paraplegia and arm claudication (only if a
mation. Diagnostic DSA and invasive gradient measure- subclavian flap is used) may occur.267
ments can be reserved for cases where non-invasive 3.6.2.3. Endovascular repair. Anatomical suitability, with a
methods fail to provide the necessary information for de- combination of good access vessels, and a short and
cision making. crossable lesion, is a necessary requirement for endovas-
In adult pa ents with aor c coarcta on, magne c resonance IIa C 263
angiography should be considered the imaging technique of choice for
anatomical characterisa on, inves ga on for associated cardiovascular
abnormali es, and to es mate aor c flow gradients
3.6.2. Management. Without treatment, mortality is 25% at cular repair. Operative mortality following endovascular
age 20, 50% at 32, and 75% at 46.264 Most patients die as a repair is very low (<1%). Rarely, aortic rupture (1e2% after
result of heart failure, acute aortic syndromes, premature simple balloon dilatation), retrograde or antegrade

Open or endovascular repair should be performed in pa ents with I C 265
coarcta on of the thoracic aorta and clinical manifesta ons resul ng
from le ventricular dysfunc on, severe hypertension or lower limb
ischaemia
RecommendaƟon 62
Pa ents with coarcta on of the thoracic aorta without clinical IIa C 265
symptoms, but with a significant aor c gradient at rest (>20 mm Hg)
and/or proximal systemic hypertension (>140/90 mm Hg), should be
considered for open or endovascular repair
dissection (<1%), stroke, or access problems may occur. gene encoding transforming growth factor beta receptor 2
Such data make endovascular repair a valid alternative to (TGFb R2), which has already been reported in LDS.272
OR in native coarctations. Late complications of endovas- A diagnosis of MFS can be established by clinical eval-
cular repair include re-coarctation, which occurs more uation alone in the majority of cases. In 1998, the Ghent
often compared to OR and aneurysm formation (0e12% criteria specified the characteristics of the phenotype and
after simple balloon dilatation).266e268 In adults, stent genotype that can be assessed through history, physical
placement appears to reduce late complications (particu- examination, imaging, and molecular genetic testing.270
larly aneurysm formation) and recurrence.267 Although More recently, an international expert panel has estab-
there is no current evidence to justify preferential use of lished a revised nosology, which puts more weight on
covered, straight, or tapered stents, these should be readily aortic root aneurysm (or dissection) and ectopia lentis.
available to expeditiously treat aortic rupture following Then, in the absence of a family history, the diagnosis of
angioplasty.268 Provided anatomical suitability, an MFS can be made in the case of aortic root enlargement
endovascular-first approach may be beneficial for the and the presence of ectopia lentis; in the case of aortic
treatment of re-coarctation.269 root enlargement and the presence of 7 or more points of

For adult pa ents with aor c coarcta on, centralisa on of treatment IIb C 266–268
to expert centres may be considered
RecommendaƟon 64
In anatomically suitable pa ents with na ve thoracic aor c coarcta on, IIa C 266,267
endovascular repair should be considered as an alterna ve to open
repair
3.7. Miscellanea a systemic score; and in the case of aortic root enlarge-
Genetic, congenital, and neoplastic pathologies can involve ment and a probably causal FBN1 mutation. Finally, the
the DTA. occurrence of ectopia lentis together with an FBN1 muta-
tion known to be associated with aortic dilatation is also
3.7.1. Genetic syndromes. sufficient to make the diagnosis. Together with a family
3.7.1.1. Marfan syndrome. Marfan syndrome (MFS) is a history, ectopia lentis or aortic root enlargement or a sys-
heritable connective tissue disorder. A mutation of the temic score 7 are sufficient to make the diagnosis in
fibrillin-1 (FBN-1) gene on chromosome 15 has been found adults.270
in patients with MFS. The sensitivity of molecular genetic testing is substantial
Estimates indicate that approximately 1 in 3,000e5,000 yet imperfect for unidentified reasons. It may be justified by
individuals have MFS.270 The mutation is inherited as a atypical location or character of FBN1 pathogenic variants in
dominant trait, so each parent with the condition has a 50% some individuals or to locus heterogeneity.273
risk of passing the genetic defect on to any child. Around MFS has a range of expressions, from mild to severe. The
one in four patients has no family history, and the syndrome most serious complications are defects of the heart valves
is the result of a spontaneous mutation. and aorta. The most severe cardiovascular complication is
Recently a second type of the disorder (MFS type 2, acute type A aortic dissection, but type B dissection is also
MFS2) has been identified.271 These mutations are in the common. AAA is rare.
TTE measurement of the aortic root is very important for pregnancy well, with good maternal and foetal outcomes,
the diagnosis and follow up of aortic complications in pa- they should be advised regarding the risk of pregnancy to
tients with MFS.274 Generalized aortic root dilatation is a both mother and foetus. Patients who have aortic root
potent marker of an increased risk of subsequent aortic dilatation >40 mm are recommended to avoid
complications in MFS, while dilatation limited to the sinuses pregnancy.277
of Valsalva without including the aortic arch has a less 3.7.1.2. Loeys-Dietz syndrome. Loeys-Dietz syndrome (LDS)
malignant prognosis. After diagnosis of aortic root/ is an autosomal dominant genetic syndrome caused by
ascending aortic dilatation is made, regular two dimensional mutations in the genes encoding transforming growth
TTE is recommended at 6 months and then annually if no factor beta receptor 1 (TGFb R1) or 2 (TGFb R2).278 Given
further progression of the disease is demonstrated. that this syndrome was only recently described, its inci-
Some studies have shown that prophylactic beta- dence is currently unknown. Four main characteristics are
adrenergic blockade is effective in slowing the rate of commonly seen in individuals with LDS: 1) arterial tortu-
aortic dilatation and reducing the development of aortic osity (twisting or spiralled arteries), most often occurring in
complications in some patients with MFS.270 Some ran- the vessels of the neck; 2) hypertelorism (widely spaced
domized studies revealed that treatment with an angio- eyes); 3) bifid (split) or broad uvula; and 4) aneurysms
tensin II receptor blocker may decrease aortic diameters in (although most often observed in the aortic root leading to
adult patients with MFS.275 However, more recent data did AD, can also be found in other arteries throughout the
not show any advantages for angiotensin II receptor body).
blockers in comparison with atenolol among children and Cardiovascular involvement in LDS has been reported as
young adults with MFS and large aortic root dilatation. A very aggressive with aneurysm formation and dissection/
significant decrease in the degree of aortic root dilatation, rupture at a young age. Aortic root aneurysms are common
relative to body surface area, was observed with both and often lead to AD. Aneurysms of the ascending or DTA
treatments.81,82 are less often seen and are seldom isolated. In the initial
It is now essential to await results of ongoing large, clinical series,279 mean age at first dissection was 26 years,
collaborative, randomized controlled trials276 with clinical dissection occurred at aortic diameters as small as 39 mm
end points to assess the novel medical treatments in and the median age of death was 37 years.
MFS. Currently suggested criteria for elective intervention for
Determination of the size threshold in MFS for surgical asymptomatic aneurysms in adults with LDS include an
repair of thoracic aneurysm is not easy. Surgery is aortic diameter >40 mm for the aortic root and abdominal
generally recommended for a dilatation 50 mm, which aorta,280 >50 mm for the DTA, and/or rapid expansion
is a lower threshold in comparison with other patients (>5 mm/year) regardless of location. Considering that
with thoracic aortic dilatation.54,274 Other factors that dissection has been reported in aneurysms <40 mm in
may significantly influence timing of aortic surgery diameter, even these criteria might not eliminate the risk of
include: 1) rate of growth >2 mm per year; 2) presence dissection or death, and earlier interventions might be
of significant aortic regurgitation; and 3) family history of indicated, depending on family history or an evaluation of
dissection, especially if dissection occurred at aortic the risks and benefits of surgery. A strategy of early and
diameter 50 mm. In cases with borderline indication aggressive surgical intervention seems to be justified,
(diameter 45 mm), a well defined increase in aortic especially given the typically young age of these patients,
diameter >3 mm/year (on repeated measurements using the generalized tolerance of surgery, and the rarity of
the same imaging technique, measured at the same complications related to tissue fragility.281 A definite
aortic level with side by side comparison and confirmed threshold diameter for intervention in cases of TAA has not
by another technique) may be an indication for surgical been established and the matter requires further investi-
treatment. gation with larger series.
Surgery to the aortic root and thoracic and thoraco- 3.7.1.3. Ehlers-Danlos syndrome. Ehlers-Danlos syndrome
abdominal aorta has resulted in significantly prolonged (EDS) is a group of disorders that share common features
life expectancy in many patients. The evidence for thoracic including easy bruising, joint hypermobility, skin hyper-
endovascular repair in MFS is much more limited, with only elasticity or laxity, and weakness of tissues, including
small series and registry or case reports including a het- arteries.
erogeneous group of patients with only short-term follow In the vascular type of EDS (formerly called type IV),
up.193 More recently, particularly in patients with an spontaneous rupture of arteries and bowel are serious
increased surgical risk because of redo sternotomy or tho- manifestations that can lead to death. It is primarily
racotomy, a gradual move to endovascular repair has been inherited as an autosomal dominant genetic disorder.
observed, but this approach cannot be recommended for Arterial rupture may be preceded by aneurysm formation,
routine use in patients with MFS. arteriovenous fistulae, or dissection but may also occur
Women with MFS are at high risk of aortic dissection in spontaneously. The anatomic locations of arterial rupture
pregnancy even in the absence of pre-conception aortic are the thorax and abdomen (50%), head and neck (25%),
root/ascending aorta dilatation. Although women with and extremities (25%). Such complications are dramatic and
aortic root dilatation of <40 mm usually tolerate often unexpected, presenting as sudden death, stroke and
its neurological sequelae, acute abdomen, retroperitoneal should be repeated every 5e10 years, or earlier if clinically
bleeding, and/or shock. The average age for the first major indicated by pregnancy or by known risk factors for AD such
arterial or gastrointestinal complication is 23 years, and the as hypertension, bicuspid aortic valve, or coarctation.288
life expectancy is only 48 years.282 A recent study investigating the effect of supra-
Surveillance may include periodic arterial screening by physiological doses of growth hormone to increase adult
MRI or computed tomographic imaging with or without height in patients with TS, which may have direct effects on
contrast. A conservative approach is usually recom- the cardiovascular system, revealed that this regimen
mended for vascular type of EDS; however, urgent sur- neither seems to affect ascending or DTA diameter beyond
gery may be required to treat potentially fatal the increase related to the larger body size nor does it
complications. disproportionately affect cardiac dimensions.289
From a pharmacological point of view, a recent multi- 3.7.1.5. Familial thoracic aortic disease. A family pattern of
centre, randomized and blinded study suggested that cel- transmission of familial thoracic aortic aneurysm and
iprolol, a b(1)-adrenoceptor antagonist with a b(2)- dissection has been described recently.62 Studies have
adrenoceptor agonist action, prevents dissections and confirmed the genetic nature of DTAAs in a large popula-
arterial ruptures in patients with vascular EDS.283 tion of affected patients and families, with an inherited
A number of patients develop post-operative anastomotic pattern for DTAA present in up to 21.5% of non-Marfan
pseudoaneurysms despite utilisation of a number of pre- patients. Patterns of inheritance and phenotypic features
cautions including delicate and atraumatic handling of tis- among family clusters have also been identified.62 These
sues, and sewing of anastomoses with pledgeted sutures.284 statistics underestimate the true prevalence of familial
At present, knowledge of the use of stents to treat aneurysm disease because many family members may
vascular complications of EDS is insufficient. Novel ap- harbour unknown aneurysms. For this reason it is quite
proaches using endovascular repair with coil embolisation likely that the true rate of inheritance of DTAA is even
have also been attempted, with good results in selected higher than 20%.
cases of ruptured pseudoaneurysms, visceral aneurysms, Although certain chromosomal loci have been identi-
and carotid-cavernous fistulas. However, because of the risk fied, complete identification of the genes leading to this
of arterial rupture following arterial puncture, indications familial non-syndromic form of DTAA is still in the early
for endovascular aortic repair remain to be determined.285 phase of investigation.290 Some families have not mapped
Maternal mortality during pregnancy in the vascular type of to either of these loci suggesting that additional loci may
EDS is around 12%, because of uterine or vascular rupture, contribute to the constellation of genes responsible for
particularly during the last trimester. To minimise the risks aneurysmal predisposition. The predominant mode of in-
related to contractions and achieve haemostasis, a Caesarean heritance is autosomal dominant, with reduced gene
section should be carried out before the onset of labour.286 penetrance in women. Variable expressivity and multiple
3.7.1.4. Turner syndrome. Turner syndrome (TS) is a genetic anatomic locations of familial arterial aneurysms have
disorder of female development with cardinal features of been observed.
short stature and congenital cardiovascular defects. It is a Patients with familial DTAA are significantly younger at
chromosomal condition in which all or part of one of the sex the time of presentation than patients with sporadic TAAA,
chromosomes is absent. Analysis of cytogenetic screening but not as young as patients with MFS. The aortic growth
studies indicates that TS occurs in w1/200 conceptions but rate has been reported to be 2.1 mm/year, which is faster
only 1/2000 live female births, with congenital cardiovascular when compared with sporadic DTAA patient growth
defects leading to a high rate of foetal death.287 (1.6 mm/year), and MFS (1 mm/year).
An epidemiological study of Danish and Swedish records The aggressive clinical course of this disease underscores
found the median age of onset of AD or rupture in TS to be the importance of appropriate screening in extended rela-
35 years, with a projected annual incidence of approxi- tives of patients with familial non-syndromic DTAA. Sec-
mately 15 cases/100,000 for individuals <20 years of age, ondary prevention strategies employed for patients with
73e78 cases/100,000 for women 20e40 years old, and MFS including angiotensin converting enzyme inhibitor
w50/100,000 for older women with TS. Of note, the inci- administration, should also be evaluated for patients with
dence of AD in the general Danish population was 6/ early onset familial DTAA.
100,000 in the same study.287 All first degree relatives of patients considered to be part of
Patients who develop AD have aortic valve disease and/ a familial aneurysm disorder may be considered for screening
or coarctation in 10e25% of cases. While apparently all TS imaging to evaluate for DTA and AAA at the age of 50, with
patients younger than 20 years have some structural repeat surveillance at regular intervals (e.g. 2e3 years).
congenital heart disease, about 10% of adults with TS that
have died from aortic dissection have no evidence of major 3.7.2. Aortic tumours. Primary tumours of the aorta are
structural defects on autopsy.288 rare and are usually malignant. The diagnosis may be
Evidence for regular screening for aortic disease in complicated as aortic tumours can clinically mimic het-
women with TS is not available. A baseline cardiovascular erogeneous conditions such as aneurysm, dissection, aortic
evaluation should be obtained at the time of diagnosis and coarctation, floating thrombus, intrathoracic, abdominal or
includes MRI in addition to echocardiography. These studies retroperitoneal malignancies, and atherosclerotic

In pa ents with Marfan syndrome, trans-thoracic echocardiographic IIa C 274
measurement of the aor c root should be considered for the diagnosis
and follow up of aor c involvement
RecommendaƟon 66
In Marfan syndrome, use of prophylac c beta-adrenergic blockade and IIa C 82,270
of angiotensin II receptor blockers should be considered
RecommendaƟon 67
In pa ents with Ehlers-Danlos syndrome, treatment with celiprolol IIa B 283
should be considered to prevent dissec ons and arterial ruptures
RecommendaƟon 68
Pa ents with Loeys-Dietz and Ehlers-Danlos syndromes should be IIa C 284
considered for treatment in collabora on with highly specialized
referral centres
RecommendaƟon 69
All first degree rela ves of pa ents considered to be part of a familial IIb C 62
aneurysm disorder may be considered for screening with imaging for
descending thoracic aor c and abdominal aor c aneurysms at the age
of 50, repeated therea er at regular intervals
RecommendaƟon 70
Reproduc ve health counselling by gene c specialists should be I C 271,272,278,279
offered to pa ents diagnosed with any gene c connec ve ssue
disorder
RecommendaƟon 71
Women with a confirmed gene c connec ve ssue disorder should be I C 277,286,287
advised regarding the rela ve risks of pregnancy to both mother and
foetus
RecommendaƟon 72
In pa ents with gene c syndromes associated with thoracic aor c IIa C
aneurysm dilata on ≥50 mm, surgery should be considered
RecommendaƟon 73
In pa ents with a gene c syndrome and enlarged aor c diameter IIb C 281
<50 mm, surgery may be considered according to body surface area in
pa ents of small stature or for rapid progression or more aggressive
diseases or with a family history of dissec on
RecommendaƟon 74
In pa ents with connec ve ssue disorder, endovascular repair may be IIb C 193,285
considered in redo opera ons or in emergencies as bridging procedures
peripheral vascular disease. The diagnosis is most often Making a diagnosis is challenging. Aortic sarcomas can
made after surgery or at autopsy. Most of the cases easily be confused with aneurysmal disease or atheroscle-
described in the literature are sarcomas and malignant rotic aorto-iliac occlusive disease. The diagnosis may be
fibrous histiocytomas.291 Aortic angiosarcomas occur more based on a high index of suspicion and is generally
often in the DTA. confirmed by fluorodeoxyglucose/positron emission to-
A clinico-pathological classification of aortic tumours mography/CT (FDG PET/CT), which shows increased uptake
categorizes the lesions as intimal or mural (obstructive and at the location of the tumour,293 and biopsy.
non-obstructive).292 Intraluminal tumours often give rise to Evidence regarding the therapeutic management of
thromboembolism, whereas those arising from the media aortic tumours shows poor outcomes. Survival in patients
and adventitia form aggressive mass lesions, which may with malignant tumours has been very poor, whether or not
have intra- and extra-luminal extension. Clinical presenta- they tolerate surgical resection. Five year survival is re-
tion and imaging findings are often non-specific. Symptoms ported at 12% after surgical intervention, and only 8% after
are typically the result of tumour embolisation, local mass conservative treatment.294
effect, or luminal obstruction causing claudication or sec- Endovascular repair is not recommended. Stent grafts do
ondary hypertension in the case of renal stenosis.292 not preclude the growth and metastatic potential of
neoplastic lesions despite angiographic exclusion.295 Endo- thrombus has disappeared, as the long-term outcome re-
vascular exclusion can be offered only for compassionate mains uncertain.
use when repetitive tumour embolic events occur.
4. SURVEILLANCE
3.7.3. Floating thrombus. Floating thrombus is defined as
the presence of mobile thrombus in the DTA that may be a To prevent complications and ascertain therapeutic efficacy,
potential source of life threatening peripheral arterial it is suggested that all patients diagnosed with or treated
emboli to visceral organs or the lower limbs. This condition for DTA disease should undergo a strict programme of
may be related to a previously injured aortic wall (dissec- systematic surveillance. Successful management and out-
tion, atherosclerosis, or trauma). comes are dependent not only on the initial treatment, but
A floating thrombus in a healthy thoracic aorta is a rare also on adequate follow up and early diagnosis of compli-
event, and is commonly associated with an underlying cations. Although the therapeutic options differ among the
thrombophilia. In many cases the pathophysiology/aeti- various DTA diseases, patient surveillance follows a similar
ology remains unknown. protocol (Fig. 3A and 3B, Table 3). These surveillance in-
Prompt diagnosis and treatment are necessary to avoid tervals are based on several reported experiences, as no
severe complications. The diagnosis of floating thoracic definitive evidence exists.44,65,85,88,107,108,129,168,173,299
aortic thrombus is usually made after debilitating embolic All patients with DTA disease require aggressive man-
events. TOE represents the non-invasive study of choice agement of hypertension, secondary prevention of cardio-
to first exclude a cardioembolic source, while also vascular diseases, and close follow up to monitor the
providing a good view of the distal arch and DTA. CTA and evolution of the diseased aorta. In particular, conservatively
MRA can provide additional and more detailed treated patients are prone to develop progression of dis-
information.296,297 ease195 and OR patients can develop anastomotic pseu-
The treatment strategy for this disease remains contro- doaneurysm, new para-anastomotic aneurysms, graft
versial and is determined by the location and extension of infection or graft occlusion.299 TEVAR may result in stent
the thrombus, presence of complications, the patient’s graft related complications such as endoleak, stent graft
comorbidities, and the physician’s preferences. Options migration, or stent graft collapse.106,107 The primary
include anticoagulant therapy, aspiration thrombectomy, importance of the surveillance protocols is to prevent these
surgical thrombectomy, or endovascular repair by covered complications.
stent deployment.296 CTA is the modality most often used for follow up in DTA
If anticoagulation is selected, a size reduction of disease. ECG-gated CTAs have greatly improved imaging
thrombus should be observed in a short time and heparin quality by eliminating pulsation artefacts. This imaging mo-
treatment must be maintained or switched to oral anti- dality may identify subtle aortic characteristics such as the
coagulation until the thrombus is completely dissolved. primary intimal tear in TBAD and ulcerative plaques in PAU
When no reduction in thrombus size is observed, or if the (see also Section 2.3.5.). An excellent assessment of the vessel
thrombus appears mobile within the aortic lumen, a more wall and aortic lumen, including the surrounding anatomy is
aggressive treatment strategy should be recommended. The derived from multiplanar imaging, three dimensional recon-
benefits of an OR approach with thoracic aortic throm- struction and centre lumen line reconstruction.20,21
bectomy are debatable, as the operative risk is dispropor- However, cumulative lifetime radiation exposure caused
tionally high relative to the potential benefit. Moreover, the by multiple CTA examinations must be taken into account
risk of a medical treatment failure leading to repeated during planning of follow up. In addition, iodinated intra-
embolism is undetermined, with a few reports demon- venous contrast agents are associated with nephrotoxic
strating complete thrombus dissolution with anti- effects. MRI can be used to avoid radiation and iodinated
coagulation alone.297 On the other hand, early intervention contrast media.300 MRI compatible stent grafts are a pre-
with thoracic aortic thrombectomy has been shown to requisite, as the presence of stainless steel implants causes
prevent fatal recurrent embolic events.296 artefacts. In patients treated with thoracic aortic endog-
Less invasive surgical approaches have been proposed, rafts, conventional chest X-rays can show stent graft
including TOE guided trans-aortic and trans-femoral balloon migration, collapse, or fracture. This is especially true
thrombectomy, percutaneous catheter thrombo-aspiration, regarding follow up after TAI repair. Given the generally
and TOE guided thrombus exclusion using endovascular young age of this subgroup of patients, concerns arise with
covered stents.298 Endovascular exclusion of the thrombus regard to the cumulative radiation and iodinated contrast
from the systemic circulation by stent graft deployment is exposure required for long-term follow up. For these rea-
certainly an attractive approach in high risk surgical patients sons, MRA may also be used for surveillance when magnetic
with favourable anatomy, although the possibility of resonance compatible stent grafts are employed.166
thrombus fragmentation and dislodgement by catheter Therefore, it seems reasonable to adopt a combination of
manipulation should also be considered. a multi-view chest X-rays and MRA instead of CTA for long-
Follow up must include long-term anticoagulant therapy term imaging of these patients.151
and routine surveillance using TOE or CTA or MRI until all In patients with inflammatory disease, infectious/in-
flammatory activity can be visualized and followed using
nuclear imaging with 18-fluorodeoxyglucose (FDG) posi- should have the same surveillance programme as those
tron emission tomography (PET), or gallium 67 (67GA).301 with AD.
In suspected aortic graft infection, the diagnostic perfor- In patients with DTAAs or TAAAs, surveillance after initial
mance of FDG-PET is superior to that of CTA and is rec- detection is similar to that for other DTA diseases (Table 3).
ommended in certain situations.301 Because these In this setting, the level of evidence remains low, despite
modalities do not provide any morphological characteris- the higher incidence of aneurysms and experience reported
tics of the aorta, they need to be combined with CTA or in the literature. In conservatively managed patients, the
MRI. Additional oesophagoscopy and bronchoscopy may mean aortic growth is about 1 mm/year, and is more
be required in evaluation of aorto-bronchial and/or aorto- prominent in the DTA in which growth has been reported to
oesophageal fistulae. be 1.9 mm/year.65 Other factors influencing aortic growth
In patients with ATBAD managed medically, progressive are initial diameter and the presence of connective tissue
aneurysmal dilatation can develop over time.189 This dila- disorders. Surveillance of patients diagnosed with aneu-
tation may eventually lead to rupture and sudden death rysms will consist of imaging studies after 6 and 12 months,
during the chronic phase.173 In ATBAD, some predictors of and yearly thereafter. For small and stable aneurysms, the
aortic growth, like the entry tear size and partial FL surveillance interval can be reduced to every 2e3 years
thrombosis, are now identified.168,174 Patients with chronic (Table 5).
TBAD may develop progression of the initial dissection or a The surveillance of patients with thoracic aortic coarc-
secondary aortic dissection event like intramural haema- tation should include an annual clinical evaluation (blood
toma. In addition, in ATBAD patients treated with TEVAR, pressure in upper and lower limbs) by the cardiologist
distal perfusion of the FL is often observed, and its role combined with echocardiography and additional aortic
remains poorly understood.191 imaging when indicated. Although uncommon, pregnant
Therefore, routine surveillance should consist of physical patients with coarctation should be monitored carefully to
examination, blood pressure control, and echocardiography, prevent complications. After coarctation repair, by surgery,
and mainly be done with MRA or CTA, based on patient TEVAR, or balloon angioplasty, regular follow up exami-
characteristics (see also Table 3). nation is mandatory. Long-term complications may occur,
The natural history of both PAU and IMH is unclear especially when the intervention has been performed in
and patients with these conditions should have a close neonates. It is imperative to minimise radiation, as the
surveillance protocol. Morphological changes can develop patient population can be relatively young. MRI, which is
over time,7,12,121 and patients with IMH and PAU the most cost effective imaging modality, should be
Table 5. Suggested disease specific follow up protocol for conservative management.

Modalities Interval Indications for closer follow up or
potential intervention
ATBAD CT or MRI 3 m, 6 m, yearly Aortic growth >5 mm/year
In stable condition after 3 years Aortic diameter >50 mm
follow up, the interval can be
extended to 2e3 years
IMH CT or MRI 3 m, 6 m, yearly Extension of IMH
In stable condition after 3 years Presence of concomitant PAU
follow up, the interval can be Aortic growth >5 mm/year
extended to 2e3 years Aortic diameter >50 mm
PAU CT or MRI 3 m, 6 m, yearly
In stable condition after 3 years
Thoracic aortic aneurysm CT or MRI 6 m, 12 m, yearly Aortic growth >5 mm/year
Thoraco-abdominal aneurysm CT or MRI 6 m, 12 m, yearly Aortic growth >5 mm/year
Inflammatory disease CT or MRI, combined 3 m, 6 m, yearly
with PET
Blood tests: white cell
count, CRP
Thoracic coarctation CT or MRI On clinical indication
Aortic disease with connective CT or MRI 3 m, 6 m, yearly Aortic diameter >45 mm
tissue disorders Aortic growth >5 mm/year
adopted routinely as the follow up screening test of Randomized studies often yield conflicting results because
choice.302 More recently, a non-invasive method based on of technical evolution and growth in the participants’
computational fluid dynamics and MR imaging, has been experience. These overall guidelines include this section to
proposed to estimate the pre- and post-operative hae- identify the most relevant gaps in the evidence.
modynamics for both native and recurrent coarctation
patients.303 5.1. General aspects
In all patients with DTA diseases managed by OR, follow
up is similar (see Fig. 4). After a 6 month post-operative CT 5.1.1. The value of glucocorticosteroids, mannitol, and
scan, patients should have yearly CTA or MRI examinations. intrathecal techniques to prevent spinal cord
The interval between scans may be lengthened after 3 years ischaemia needs clinical data to be definitively
of complication-free follow up. adopted or rejected for clinical practice during OR of
TEVAR requires more intensive surveillance compared DTA.
with open treatment, because of potential stent graft 5.1.2. The value of MEP and SSEP in patients treated with
related complications such as endoleak, migration, and TEVAR is not clearly supported by clinical data.
collapse. Detection of type I and III endoleaks requires
prompt intervention, as does stent graft migration or 5.1.3. The optimal brain protection strategy in patients
collapse. Type II and IV endoleaks may be followed by serial requiring open or endovascular repair for thoracic
CTA/MRI187,304 (see Fig. 5). aortic disease needs to be defined.
Computed tomographic aortography should be considered IIa C 20

the first op on for imaging surveillance in the acute se ng
of pa ents with thoracic aor c diseases
RecommendaƟon 76
Magne c resonance imaging should be considered as an IIa C 300

imaging modality to follow up pa ents with aor c endogra s
RecommendaƟon 77
In pa ents diagnosed with type B aor c dissec on, IIa C 19,121,173,189

penetra ng aor c ulcer, or intramural haematoma, rou ne
surveillance should be considered including physical
examina on, echocardiography and imaging with magne c
resonance angiography or computed tomographic
aortography
RecommendaƟon 78
For pa ents treated with endogra s life-long imaging follow IIa C 187
up with magne c resonance angiography or computed
tomographic aortography should be considered
RecommendaƟon 79
Any early or late type I or III endoleak a er an endovascular I C 187,304

repair of the descending thoracic aorta should undergo
prompt interven on
RecommendaƟon 80
In pa ents with DTA pathology managed by open repair, I C 129, 299

follow up imaging a er 6 months with computed
tomography should be performed
5. GAPS IN THE EVIDENCE 5.2. Acute type B dissection

Robust evidence is still needed in many aspects of the
5.2.1. TEVAR indications for uncomplicated ATBAD need to
management of DTA pathologies. In numerous clinical
be elucidated by prospective and randomized
scenarios, adequate trials are lacking. In addition, because
studies.
of continuous technical innovation in endovascular repair,
clinical practice tends to follow technical developments 5.2.2. It would be useful to define early unfavourable
without robust evidence from randomized trials. clinical and imaging signs and biomarkers as
Computed Tomograpic Angiography
AŌer 6 months
Gra infec on No signs of

Occlusion complica ons
Anastomotic
pseudoaneurysm
Treatment as required Gra infec on Annual CTA/MRI

Endovascular or open repair Occlusion AŌer 3 years follow up, the
Figure 4. Anastomo OR.
c interval can be extended to
pseudoaneurysm 2–3 years
Para-anastomic
aneurysm
Figure 4. Suggested surveillance algorithm after open repair.
Computed Tomograpic Angiography
Within 1 month and 12 months aŌer

procedure
Endoleak* No endoleak
(Other
Complica ons**) No signs of
complica ons
Type I and III Type II or

Stent gra Type IV Annual CTA/ MRI
migra on
Stent gra Type IB in AŌer 3 years follow-up, interval
collapse Dissec ons can be extended to 2–3 years
No endoleak
CTA/MRI at 3 and or
6 months complica ons
Aneurysm sac Aneurysm sac

growth growth <10mm
>10mm
Endovascular or open
repair as required
Figure 5. Suggested surveillance algorithm after TEVAR. *Endoleak types: type I, direct flow between endograft and aortic wall in the
proximal (type Ia) or distal sealing zone (type Ib); type II, blood flow in the aneurysm sac, originating from side branches; type III, direct
flow coming from overlapped segments or through a defect of the stent graft fabric; type IV, blood flow into the aneurysm sac because of
the porosity of the graft fabric. ** Other complications should be managed accordingly.
predictive factors to identify patients who can

benefit the most from thoracic endografting. 5.5.3. The natural history of CTBAD may be related to
morphological features that could be defined with
5.3. Thoracic aortic rupture newer imaging techniques such as 4D MRA.
Research is awaited.
5.3.1. There is no level A or B evidence for the best method of
repair of ruptured thoracic aortic aneurysms. A few 5.5.4. There is a lack of distinct evidence regarding early
non-consecutive studies report endovascular outcomes following repair of CTBAD e either open
techniques and compare these with earlier reports or endovascular. At present most data relate to a
using OR (level C). Intuitively, sealing an anatomically mixture of thoracic pathologies and more
suitable ruptured thoracic aneurysm via a femoral homogenous data series are required. There are only
approach with a stent graft without opening the a few series detailing the ability of OR to prevent
chest must put the patient at lower risk, and there aortic related death in the mid- and long-term. There
will therefore never be a randomized trial to are very few series detailing the ability of TEVAR to
compare the two modalities. In addition, the number prevent aortic related death in the mid- and long-term.
of affected patients is too small to support a
5.6. Descending thoracic aortic aneurysm
randomized trial and patients are very difficult to
recruit.
5.6.1. Intervention in DTAA <55 mm may not afford a
survival benefit. Outcomes of intervention need to
5.4. Blunt traumatic thoracic aortic injury be exceptional to justify repair in the small
aneurysm group. The advent of TEVAR justifies a
5.4.1. A randomized clinical trial is not justified to elucidate randomized prospective trial for small DTAA to
the benefit of TEVAR over OR in the acute setting. compare early TEVAR and surveillance only.
5.4.2. No evidence supports the choice between open and 5.6.2. Long-term durability of thoracic endografting,
endovascular repair for chronic post-traumatic especially for DTAA repair, is not well
pseudoaneurysms. determined. More data are needed from case
series and case control studies to elucidate the
5.4.3. More information about the natural history of late complications and re-intervention rate
chronic post-traumatic pseudoaneurysms is beyond 5 years follow up.
needed. No clear criteria for repair have been
described. The protective role of severe 5.7. Thoraco-abdominal aneurysm
calcification remains uncertain.
5.7.1. There is no level A or B evidence on the best method
5.4.4. A follow up programme for patients who have of repair for elective TAAA.
been treated by TEVAR for TAI needs to be
established. 5.8. Inflammatory diseases
5.5. Chronic type B dissection 5.8.1. A very low level of evidence is available for
determining indications for treating inflammatory
disease of the thoracic aorta. The same indications
5.5.1. The advent of endovascular repair suggests that
are recommended as for elective repair of any
TBAD may require an additional classification with
thoracic aortic disease, based on the risk of rupture
respect to chronicity. A sub-category of sub-acute
related to aneurysm dimension and the presence of
dissection may reflect the ability of the aorta to
secondary end organ ischaemia. This is level C
remodel between 2 and 12 weeks after the initial
evidence.
dissection. Further evidence of aortic remodelling
after TEVAR and the natural history of sub-acute
5.8.2. There is very poor evidence to support any specific
dissection is required.
follow up regimen after surgical or endovascular
repair of cardiovascular disorders in patients with
5.5.2. There is a lack of natural history data relating to
systemic inflammatory diseases receiving steroid
CTBAD in the era of modern medical therapy. Data
therapy.
are required to define the incidence of aortic
related events, aortic growth rates, and FL
5.9. Coarctation
thrombosis rates. These data may inform the
threshold and indications for interventional
therapy. At present there are no conclusive data to 5.9.1. There is no high quality evidence regarding the ideal
establish diameter thresholds for repair of TBAD. treatment strategy for native or recurrent coarctation
in adults. No randomized trials have compared open ACKNOWLEDGEMENTS

with endovascular strategies and such a comparison The Writing Committee would like to thank F. Bastos
is impractical because of the rareness of the disease Gonçalves (Netherlands), C. Brinster (Spain), E. Civilini
and constant developments in endovascular (Italy), G. Coppi (Italy), G. de Donato (Italy), G. Maritati
technology. Further comparative evidence from (Italy), G. Mestres (Spain), K. Paraskevas (Greece), F.
case control studies and case series is required. Pecoraro (Switzerland), and JL. Tolenaar (Netherlands) for
Long-term data regarding durability and late their contribution on preparing and writing this manuscript.
complications following endovascular repair are
essential.
APPENDIX. RECOMMENDED ADDITIONAL REFERENCES
5.9.2. Concerning endovascular repair, no high quality The recommended additional references related to this
evidence supports the routine use of bare or article can be found online at http://dx.doi.org/10.1016/j.
covered stents. A prospective randomized trial ejvs.2016.06.005.
could clarify the optimal strategy in patients with
native or recurrent coarctation, with attention to
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Eur J Vasc Endovasc Surg (2017) 53, 4e52

Editor’s Choice e Management of Descending Thoracic Aorta Diseases

3.1.3. Ruptured aneurysm of the descending thoracic aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . 18

ABBREVIATIONS AND ACRONYMS LDS Loeys-Dietz Syndrome

1. INTRODUCTION document to avoid potential inter-specialty conﬂict. The

Table 1. Levels of evidence.1 For each recommendation, two members of the WC

RecommendaƟon 1 Class Level of evidence References

RecommendaƟon 2 Class Level of evidence References

Mul detector computed tomographic angiography from thoracic IIa C 20,21

For pa ents at increased risk of contrast induced nephropathy, IIa C 22

Table 3. Comparison of different imaging modalities for DTA diagnosis.17

RecommendaƟon 5 Class Level of evidence References

During open thoracic or thoraco-abdominal aor c repair, IIb C 30,33

RecommendaƟon 6 Class Level of evidence References

Cerebrospinal fluid drainage has a role in the preven on of IIa B 29,37

Systemic cooling to less than 32 °C in combination with cerebrospinal III B 39,42

RecommendaƟon 10 Class Level of evidence References

RecommendaƟon 11 Class Level of evidence References

In elec ve thoracic endogra ing cases when it is planned to IIa C 44

3. SPECIFIC THORACIC AORTIC DISORDERS

Recommendaon 12 Class Level of evidence References

Paents with acute type B aorc dissecon who develop new or I C 78

Recommendaon 13 Class Level of evidence References

Medical therapy should always be part of the treatment of paents I C 55,56,60,65,70,77–79

RecommendaƟon 16 Class Level of evidence References

In pa ents with complicated acute type B aor c dissec on, I C 6,85–89,92–94,96–99,

In complicated acute type B aor c dissec on, endovascular IIa C 73,100–102

Recommendaon 19 Class Level of evidence References

Recommendaon 20 Class Level of evidence References

Uncomplicateda type B intramural haematoma and penetrang I C 121,122

Endovascular repair should be considered for complicateda type B IIa C 123–125

Endovascular repair should be considered for complicatedb type B IIa C 119,121,123,124

RecommendaƟon 23 Class Level of evidence References

In pa ents with ruptured descending thoracic aor c aneurysm, I B 127

In emergency ruptured descending thoracic aor c aneurysm in I C 49

RecommendaƟon 25 Class Level of evidence References

Computed tomographic angiography should be performed for the I C 142

In trauma c thoracic aor c injury, transoesophageal IIa C 142,143

RecommendaƟon 27 Class Level of evidence References

RecommendaƟon 29 Class Level of evidence References

RecommendaƟon 30 Class Level of evidence References

Long-term medical treatment with β-blockers should be given to I C 80

RecommendaƟon 33 Class Level of evidence References

In pa ents with chronic aor c dissec on and thoraco-abdominal IIa C 167,176

RecommendaƟon 36 Class Level of evidence References

Open repair of aneurysmal or symptoma c chronic type B aor c IIa C 41,180

RecommendaƟon 38 Class Level of evidence References

Figure 2. Classiﬁcation of DTAA according to the extension.202

RecommendaƟon 40 Class Level of evidence References

Computed tomographic angiography or magne c resonance I C 198,199

To reduce the incidence of postopera ve paraplegia, le heart bypass is III C 114

RecommendaƟon 46a Class Level of evidence References

Figure 3. Crawford TAAA classification modified by Safi.222

RecommendaƟon 47 Class Level References

RecommendaƟon 48 Class Level of evidence References

RecommendaƟon 53 Class Level of evidence References

Centralisa on of endovascular repair of thoraco-abdominal aor c IIb C 242

RecommendaƟon 56 Class Level of evidence References

RecommendaƟon 60 Class Level of evidence References