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8.3.2.1. Clinical presentation and diagnosis ................................. 3598 8.3.2.1.1. Epidemiology ................................................................... 3598 8.3.2.1.2. Clinical presentation ..................................................... 3598 8.3.2.1.3. Diagnosis of renal artery disease ............................ 3598 8.3.2.1.4. Prognosis ........................................................................... 3598 8.3.2.2. Treatment strategy (medical and interventional) ..... 3598 8.3.2.2.1. Medical therapy .............................................................. 3598 8.3.2.2.2. Revascularization ............................................................ 3598 8.3.2.3. Follow-up ................................................................................... 3600 8.3.3. Visceral artery disease .................................................................. 3600 8.3.3.1. Acute mesenteric ischaemia .............................................. 3600 8.3.3.1.1. Clinical presentation and diagnosis ........................ 3600 8.3.3.1.2. Treatment strategy ....................................................... 3600 8.3.3.1.3. Follow-up .......................................................................... 3601 8.3.3.2. Chronic mesenteric artery disease ................................. 3601 8.3.3.2.1. Clinical presentation and diagnosis ........................ 3601 8.3.3.2.2. Treatment strategy ....................................................... 3602 8.3.3.2.3. Follow-up .......................................................................... 3602 9. Aorta ..................................................................................................................... 3602 9.1. Atheromatous disease of the aorta ............................................... 3602 9.1.1. General concepts ............................................................................ 3602 9.1.2. Treatment .......................................................................................... 3602 9.1.2.1. Primary prevention ................................................................ 3602 9.1.2.2. Secondary prevention .......................................................... 3602 9.2. Aortic aneurysms ................................................................................... 3603 9.2.1. General concepts ............................................................................ 3603 9.2.1.1. Definitions ................................................................................. 3603 9.2.2. Thoracic aortic aneurysms ......................................................... 3603 9.2.2.1. Aetiology, risk factors, and natural history ................. 3603 9.2.2.2. Ascending thoracic aorta and arch aneurysms ......... 3603 9.2.2.3. Descending thoracic aorta and thoracoabdominal aorta aneurysms ..................................................................................... 3604 9.2.2.4. Surveillance ............................................................................... 3607 9.2.3. Abdominal aortic aneurysms ..................................................... 3607 9.2.3.1. General concepts ................................................................... 3607 9.2.3.2. Aetiology, risk factors, and natural history ................. 3607 9.2.3.3. Surveillance ............................................................................... 3607 9.2.4. Optimal medical treatment of aortic aneurysms .............. 3609 9.2.5. Surgical management of aortic aneurysms .......................... 3609 9.2.5.1. Surgical treatment of aortic root and ascending aorta ............................................................................................................ 3609 9.2.5.2. Surgical treatment of aortic arch aneurysms ............. 3611 9.2.5.3. Surgical treatment of the thoracic descending aorta 3612 9.2.5.3.1. General considerations ............................................... 3612 9.2.5.3.2. Open repair ..................................................................... 3612 9.2.5.3.3. Endovascular repair ...................................................... 3612 9.2.5.4. Surgical treatment of thoracoabdominal aorta aneurysms ................................................................................................. 3612 9.2.5.4.1. General considerations ............................................... 3612 9.2.5.4.2. Open repair ..................................................................... 3612 9.2.5.4.3. Endovascular repair ...................................................... 3612 9.2.5.5. Surgical treatment of abdominal aorta aneurysms .. 3613 9.2.5.5.1. General considerations ............................................... 3613 9.2.5.5.2. Pre-operative cardiovascular evaluation and choice of treatment ......................................................................... 3613 9.2.5.5.3. Open abdominal aorta aneurysm repair ............. 3615 9.2.5.5.4. Endovascular abdominal aorta aneurysm repair 3615 9.2.6. Endoleaks ........................................................................................... 3615 9.2.7. Long-term follow-up after aortic repair ............................... 3617 9.2.7.1. Follow-up after thoracic aortic aneurysm treatment 3617 9.2.7.2. Follow-up after abdominal aortic aneurysm treatment ................................................................................................... 3617 9.3. Acute thoracic aortic syndromes .................................................... 3618 9.3.1. General concepts ............................................................................ 3618 9.3.1.1. Epidemiology and risk factors ........................................... 3618 9.3.1.1.1. Sex differences ................................................................ 3620 9.3.1.1.2. Chronobiology ................................................................ 3621 9.3.1.1.3. Outcomes ......................................................................... 3621 9.3.1.2. Clinical presentation ............................................................. 3622 9.3.1.3. Diagnostic work-up ............................................................... 3622 9.3.1.4. Therapeutic intervention in acute aortic dissection 3622 9.3.1.4.1. Initial treatment .............................................................. 3622 9.3.1.4.2. Type A aortic dissection interventional treatment .............................................................................................. 3623 9.3.1.4.3. Acute type B aortic dissection interventional treatment .............................................................................................. 3628 9.3.1.4.4. Chronic type B aortic dissection interventional treatment .............................................................................................. 3629 9.3.1.4.5. Management during pregnancy ................................ 3629 9.3.2. Intramural haematoma ................................................................. 3630 9.3.2.1. Diagnostic work-up ............................................................... 3630 9.3.2.2. Clinical outcomes ................................................................... 3630 9.3.2.3. Geographical variations ....................................................... 3630 9.3.2.4. Management ............................................................................. 3630 9.3.2.4.1. Type A intramural haematoma ............................... 3630 9.3.2.4.2. Type B intramural haematoma ................................ 3630 9.3.3. Penetrating atherosclerotic ulcer ............................................ 3631 9.3.3.1. Diagnosis .................................................................................... 3631 9.3.3.2. Treatment ................................................................................. 3631 9.3.4. Aortic pseudo-aneurysm ............................................................. 3632 9.3.5. Traumatic aortic injury ................................................................. 3633 9.3.5.1. Diagnosis and therapeutic interventions ...................... 3633 9.3.5.2. Long-term surveillance in traumatic aortic injury .... 3633 9.3.6. Iatrogenic aortic injuries .............................................................. 3633 9.3.7. Long-term follow-up of acute aortic syndrome ............... 3633 9.3.7.1. Follow-up after invasive treatment ................................ 3634 9.3.7.2. Follow-up under medical treatment (chronic type B aortic dissection, intramural haematoma, penetrating atherosclerotic ulcer) ........................................................................... 3636 10. Genetic and congenital diseases of the aorta ................................... 3637 10.1. Genetic and chromosomal diseases ............................................ 3637 10.1.1. Turner syndrome ......................................................................... 3638 10.1.1.1. Diagnosis, clinical presentation, and natural history 3638 10.1.1.2. Medical treatment ............................................................... 3640 10.1.1.3. Surgery of aortic aneurysms ........................................... 3640 10.1.1.4. Pregnancy and physical exercise ................................... 3640 10.1.2. Vascular Ehlers–Danlos syndrome ....................................... 3641 10.1.2.1. Diagnosis, clinical presentation, and natural history 3641 10.1.2.2. Surveillance and imaging ................................................... 3641 10.1.2.3. Medical treatment ............................................................... 3641 10.1.2.4. Surgical treatment ............................................................... 3641 10.1.2.5. Pregnancy ................................................................................ 3641 10.1.3. Marfan syndrome ......................................................................... 3641 10.1.3.1. Diagnosis, clinical presentation, and natural history 3641 10.1.3.2. Imaging surveillance ............................................................ 3641 10.1.3.3. Medical treatment ............................................................... 3642 10.1.3.4. Aortic surgery ....................................................................... 3642 10.1.3.5. Pregnancy and physical exercise ................................... 3643

ESC Guidelines                                                                                                                                                                                          3541

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