Infection (mycotic): Localized infection weakens/compromises integrity of aortic wall
Typically saccular configuration
Pseudoaneurysm: Disruption of ≥ 1 arterial wall layers; forms contained rupture
May be due to aortic injury following penetrating or blunt trauma (e.g., deceleration injury)
Also associated with infectious, inflammatory, & neoplastic disease
Increased rupture risk if TAA > 5-cm diameter or growth of > 1 cm per year
Rupture risk doubles per 1-cm aneurysm increment
Great vessel aneurysms: Account for only 1-3% of all arterial aneurysms
Brachiocephalic artery most commonly involved
Same etiologies as TAAs
Stenoses/occlusions of thoracic aorta & great vessels
Atherosclerosis: Most common cause
Severe stenoses may progress to chronic occlusion
Often occur at/near origins from thoracic aorta
Coarctation: Congenital condition in which thoracic aorta narrows in region where ductus arteriosus inserts; 3 subtypes
Preductal: Occurs proximal to ductus arteriosus
Due to decreased left heart blood flow from fetal cardiac anomaly; leads to hypoplastic aorta
Aortic perfusion distal to narrowing relies on ductus arteriosus; can be life threatening if severe
Occurs in ~ 5% of infants with Turner syndrome
Ductal: Narrowing at ductus arteriosus insertion
Usually appears when ductus arteriosus closes
Postductal: Narrowing distal to ductus arteriosus
Likely due to contraction & fibrosis of ductus arteriosus at birth, subsequently narrowing aorta
Most common type in adults
Pseudocoarctation: Rare congenital anomaly in which kinking/buckling of thoracic aorta occurs; resembles true thoracic coarctation on imaging
Involves descending thoracic aorta immediately distal to left subclavian artery origin
Differentiated from true coarctation by absence of significant hemodynamic obstruction
Subclavian steal syndrome: Retrograde flow in vertebral &/or internal mammary artery due to proximal subclavian artery stenosis or occlusion
Can cause posterior fossa ischemia resulting in ataxia, dizziness
May also present with arm claudication
Vasculitis
Giant cell arteritis (GCA): Vasculitis affecting medium- to large-caliber arteries; similar to Takayasu arteritis & may represent single disease spectrum
Left common carotid artery: 2nd branch of thoracic aorta; divides into external & internal carotid arteries
Left subclavian artery: 3rd branch of thoracic aorta; yields same branches as right subclavian artery
Significant Variant Vascular Anatomy
Aberrant right subclavian artery: Arises as last branch of aortic arch; most common clinically significant aortic arch malformation (0.4-2.3% incidence)
Usually passes posterior to trachea & esophagus
Patients rarely symptomatic; can cause stridor &/or swallowing difficulties (dysphagia lusoria)
Diverticulum of Kommerell: Conical dilatation of aberrant right subclavian artery origin
Represents distal remnant of right aortic arch
Large diverticulum can be symptomatic
19% present with rupture
Can also present with distal embolization
Right aortic arch
With aberrant left subclavian artery: Most common type of right aortic arch (0.1% incidence)
Arch courses over right mainstem bronchus but may descend on either side of spine; left subclavian artery is last aortic branch
Associated with diverticulum of Kommerell
Associated congenital heart disease in 5-10%
Tetralogy of Fallot (71%)
Atrial or ventricular septal defect (21%)
Aortic coarctation, rarely (7%)
With mirror image branching: Descending aorta almost always courses to right of spine
Left innominate artery followed by right carotid & right subclavian arteries; no retroesophageal vessel
Associated congenital heart disease in 90-95%
Tetralogy of Fallot (90%)
Truncus arteriosis (6%)
Tricuspid atresia (5%)
Double aortic arch
Most common vascular ring
Aortic arches pass on both sides of trachea, join posterior to esophagus
Right arch larger & higher, supplies right subclavian & common carotid arteries
Smaller & lower left aortic arch supplies left subclavian & common carotid arteries
Rarely associated with congenital heart disease
Clinical symptoms of dysphagia &/or stridor
Cervical aortic arch
Rare anomaly; usually asymptomatic
May present as pulsating supraclavicular mass
May rarely produce vascular ring, compress airway
Right-sided location in > 80%
Branching may be normal or mirror image
PREPROCEDURE
Indications
Contraindications
Preprocedure Imaging
Getting Started
PROCEDURE
Patient Position/Location
Procedure Steps
Alternative Procedures/Therapies
OUTCOMES
Complications
Expected Outcomes
Selected References
Jenkins P et al: Learning from mistakes when reporting urgent and emergency vascular studies. Clin Radiol. 77(3):159-66, 2022
Nugraha RA et al: Descending aorta diameters as predictor of late adverse outcomes in patients with uncomplicated type B aortic dissection: a systematic review and meta-analysis. Ann Vasc Surg. 80:333-44, 2022
Preventza O et al: Endovascular repair of the ascending aorta: the last frontier. Ann Cardiothorac Surg. 11(1):26-30, 2022
Liu D et al: Comparison of the efficacy and safety of thoracic endovascular aortic repair with open surgical repair and optimal medical therapy for acute type B aortic dissection: a systematic review and meta-analysis. Int J Surg. 83:53-61, 2020
Mussa FF et al: Acute aortic dissection and intramural hematoma: a systematic review. JAMA. 316(7):754-63, 2016
Harris KM et al: Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation. 126(11 Suppl 1):S91-6, 2012
Waterman AL et al: Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg. 55(5):1234-41, 2012
Aiello F et al: Open and endovascular management of subclavian and innominate arterial pathology. Semin Vasc Surg. 24(1):31-5, 2011
Yuan SM et al: Cystic medial necrosis: pathological findings and clinical implications. Rev Bras Cir Cardiovasc. 26(1):107-15, 2011
Chao CP et al: Natural history and CT appearances of aortic intramural hematoma. Radiographics. 29(3):791-804, 2009
Patel SN et al: Catheter-based treatment of the subclavian and innominate arteries. Catheter Cardiovasc Interv. 71(7):963-8, 2008
van Hattum ES et al: Angioplasty with or without stent placement in the brachiocephalic artery: feasible and durable? A retrospective cohort study. J Vasc Interv Radiol. 18(9):1088-93, 2007
Brountzos EN et al: Endovascular treatment of occlusive lesions of the subclavian and innominate arteries. Cardiovasc Intervent Radiol. 29(4):503-10, 2006
Chambers CM et al: Treatment of nonaortic aneurysms in the endograft era: aneurysms of the innominate and subclavian arteries. Semin Vasc Surg. 18(4):184-90, 2005
Sueyoshi E et al: New development of an ulcerlike projection in aortic intramural hematoma: CT evaluation. Radiology 224: 536-41, 2002
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Procedure
Outcomes
TERMINOLOGY
Definitions
Acute aortic syndromes: Spectrum of abnormalities that acutely compromise aortic wall integrity
Infection (mycotic): Localized infection weakens/compromises integrity of aortic wall
Typically saccular configuration
Pseudoaneurysm: Disruption of ≥ 1 arterial wall layers; forms contained rupture
May be due to aortic injury following penetrating or blunt trauma (e.g., deceleration injury)
Also associated with infectious, inflammatory, & neoplastic disease
Increased rupture risk if TAA > 5-cm diameter or growth of > 1 cm per year
Rupture risk doubles per 1-cm aneurysm increment
Great vessel aneurysms: Account for only 1-3% of all arterial aneurysms
Brachiocephalic artery most commonly involved
Same etiologies as TAAs
Stenoses/occlusions of thoracic aorta & great vessels
Atherosclerosis: Most common cause
Severe stenoses may progress to chronic occlusion
Often occur at/near origins from thoracic aorta
Coarctation: Congenital condition in which thoracic aorta narrows in region where ductus arteriosus inserts; 3 subtypes
Preductal: Occurs proximal to ductus arteriosus
Due to decreased left heart blood flow from fetal cardiac anomaly; leads to hypoplastic aorta
Aortic perfusion distal to narrowing relies on ductus arteriosus; can be life threatening if severe
Occurs in ~ 5% of infants with Turner syndrome
Ductal: Narrowing at ductus arteriosus insertion
Usually appears when ductus arteriosus closes
Postductal: Narrowing distal to ductus arteriosus
Likely due to contraction & fibrosis of ductus arteriosus at birth, subsequently narrowing aorta
Most common type in adults
Pseudocoarctation: Rare congenital anomaly in which kinking/buckling of thoracic aorta occurs; resembles true thoracic coarctation on imaging
Involves descending thoracic aorta immediately distal to left subclavian artery origin
Differentiated from true coarctation by absence of significant hemodynamic obstruction
Subclavian steal syndrome: Retrograde flow in vertebral &/or internal mammary artery due to proximal subclavian artery stenosis or occlusion
Can cause posterior fossa ischemia resulting in ataxia, dizziness
May also present with arm claudication
Vasculitis
Giant cell arteritis (GCA): Vasculitis affecting medium- to large-caliber arteries; similar to Takayasu arteritis & may represent single disease spectrum
Left common carotid artery: 2nd branch of thoracic aorta; divides into external & internal carotid arteries
Left subclavian artery: 3rd branch of thoracic aorta; yields same branches as right subclavian artery
Significant Variant Vascular Anatomy
Aberrant right subclavian artery: Arises as last branch of aortic arch; most common clinically significant aortic arch malformation (0.4-2.3% incidence)
Usually passes posterior to trachea & esophagus
Patients rarely symptomatic; can cause stridor &/or swallowing difficulties (dysphagia lusoria)
Diverticulum of Kommerell: Conical dilatation of aberrant right subclavian artery origin
Represents distal remnant of right aortic arch
Large diverticulum can be symptomatic
19% present with rupture
Can also present with distal embolization
Right aortic arch
With aberrant left subclavian artery: Most common type of right aortic arch (0.1% incidence)
Arch courses over right mainstem bronchus but may descend on either side of spine; left subclavian artery is last aortic branch
Associated with diverticulum of Kommerell
Associated congenital heart disease in 5-10%
Tetralogy of Fallot (71%)
Atrial or ventricular septal defect (21%)
Aortic coarctation, rarely (7%)
With mirror image branching: Descending aorta almost always courses to right of spine
Left innominate artery followed by right carotid & right subclavian arteries; no retroesophageal vessel
Associated congenital heart disease in 90-95%
Tetralogy of Fallot (90%)
Truncus arteriosis (6%)
Tricuspid atresia (5%)
Double aortic arch
Most common vascular ring
Aortic arches pass on both sides of trachea, join posterior to esophagus
Right arch larger & higher, supplies right subclavian & common carotid arteries
Smaller & lower left aortic arch supplies left subclavian & common carotid arteries
Rarely associated with congenital heart disease
Clinical symptoms of dysphagia &/or stridor
Cervical aortic arch
Rare anomaly; usually asymptomatic
May present as pulsating supraclavicular mass
May rarely produce vascular ring, compress airway
Right-sided location in > 80%
Branching may be normal or mirror image
PREPROCEDURE
Indications
Contraindications
Preprocedure Imaging
Getting Started
PROCEDURE
Patient Position/Location
Procedure Steps
Alternative Procedures/Therapies
OUTCOMES
Complications
Expected Outcomes
Selected References
Jenkins P et al: Learning from mistakes when reporting urgent and emergency vascular studies. Clin Radiol. 77(3):159-66, 2022
Nugraha RA et al: Descending aorta diameters as predictor of late adverse outcomes in patients with uncomplicated type B aortic dissection: a systematic review and meta-analysis. Ann Vasc Surg. 80:333-44, 2022
Preventza O et al: Endovascular repair of the ascending aorta: the last frontier. Ann Cardiothorac Surg. 11(1):26-30, 2022
Liu D et al: Comparison of the efficacy and safety of thoracic endovascular aortic repair with open surgical repair and optimal medical therapy for acute type B aortic dissection: a systematic review and meta-analysis. Int J Surg. 83:53-61, 2020
Mussa FF et al: Acute aortic dissection and intramural hematoma: a systematic review. JAMA. 316(7):754-63, 2016
Harris KM et al: Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation. 126(11 Suppl 1):S91-6, 2012
Waterman AL et al: Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg. 55(5):1234-41, 2012
Aiello F et al: Open and endovascular management of subclavian and innominate arterial pathology. Semin Vasc Surg. 24(1):31-5, 2011
Yuan SM et al: Cystic medial necrosis: pathological findings and clinical implications. Rev Bras Cir Cardiovasc. 26(1):107-15, 2011
Chao CP et al: Natural history and CT appearances of aortic intramural hematoma. Radiographics. 29(3):791-804, 2009
Patel SN et al: Catheter-based treatment of the subclavian and innominate arteries. Catheter Cardiovasc Interv. 71(7):963-8, 2008
van Hattum ES et al: Angioplasty with or without stent placement in the brachiocephalic artery: feasible and durable? A retrospective cohort study. J Vasc Interv Radiol. 18(9):1088-93, 2007
Brountzos EN et al: Endovascular treatment of occlusive lesions of the subclavian and innominate arteries. Cardiovasc Intervent Radiol. 29(4):503-10, 2006
Chambers CM et al: Treatment of nonaortic aneurysms in the endograft era: aneurysms of the innominate and subclavian arteries. Semin Vasc Surg. 18(4):184-90, 2005
Sueyoshi E et al: New development of an ulcerlike projection in aortic intramural hematoma: CT evaluation. Radiology 224: 536-41, 2002
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