Diagnosis and Management of Aortic Disease

Publication Date: November 2, 2022

Overview

Overview

Top 10 Take-Home Messages for the Diagnosis and Management of Aortic Disease

  1. Because outcomes for patients with aortic disease are enhanced at programs with higher volumes, experienced practitioners, and extensive management capabilities, Multidisciplinary Aortic Team care is considered in determining the appropriate timing of intervention.
  2. Shared decision-making involving the patient and a multidisciplinary team is highly encouraged to determine the optimal medical, endovascular, and open surgical therapies. In patients with aortic disease who are contemplating pregnancy or who are pregnant, shared decision-making is especially important when considering the cardiovascular risks of pregnancy, the diameter thresholds for prophylactic aortic surgery, and the mode of delivery.
  3. Computed tomography, magnetic resonance imaging, and echocardiographic imaging of patients with aortic disease should follow recommended approaches for image acquisition, measurement and reporting of relevant aortic dimensions, and the frequency of surveillance before and after intervention.
  4. At centers with Multidisciplinary Aortic Teams and experienced surgeons, the threshold for surgical intervention for sporadic aortic root and ascending aortic aneurysms has been lowered from 5.5 cm to 5.0 cm in selected patients, and even lower in specific scenarios among patients with heritable thoracic aortic aneurysms.
  5. In patients who are significantly smaller or taller than average, surgical thresholds may incorporate indexing of the aortic root or ascending aortic diameter to either patient body surface area or height, or aortic cross-sectional area to patient height.
  6. Rapid aortic root growth or ascending aortic aneurysm growth, an indication for intervention, is defined as ≥0.5 cm in 1 year or ≥0.3 cm per year in 2 consecutive years for those with sporadic aneurysms and ≥0.3 cm in 1 year for those with heritable thoracic aortic disease or bicuspid aortic valve.
  7. In patients undergoing aortic root replacement surgery, valve-sparing aortic root replacement is reasonable if the valve is suitable for repair and when performed by experienced surgeons in a Multidisciplinary Aortic Team.
  8. Patients with acute type A aortic dissection, if clinically stable, should be considered for transfer to a high-volume aortic center to improve survival. The operative repair of type A aortic dissection should entail at least an open distal anastomosis rather than just a simple supracoronary interposition graft.
  9. There is an increasing role for thoracic endovascular aortic repair in the management of uncomplicated type B aortic dissection. Clinical trials of repair of thoracoabdominal aortic aneurysms with endografts are reporting results that suggest endovascular repair is an option for patients with suitable anatomy.
  10. In patients with aneurysms of the aortic root or ascending aorta, or those with aortic dissection, screening of first-degree relatives with aortic imaging is recommended.

Introduction

...ntroduction...

Note: The numbering of the following tables may d...


...Anatomy, Abnormal Anatomy, and Definitions...

...1. The Anatomy of the Aorta and Its Main...

...gure 2. A Simplified Diagram Depic...

...sification of Aortic Anatomic Segm...

...e 4. Freedom From Ascending Aortic Complic...

...Relative Risk of Aortic Dissection b...

...ssification of Aortic Dissection Chronicit...

...gure 6. Acute Aortic SyndromesIn aortic dissec...

...ssification of Acute Aortic DissectionThe D...

...e 8. Anatomic Reporting of Aortic Dissecti...

...Mechanisms of Dynamic and Static Obstruction in...

...ure 10. Classification of Thoracoabd...

...Classification of Endoleak TypesEndoleaks a...


Assessment

Assessme...

...maging and Measurem...

...tic Imaging Techniques to Determine Presence...

...n patients with known or suspected aortic...

...n patients with known or suspected aortic diseas...

...nts with known or suspected aortic disease,...

...tients with known or suspected aortic disease...

In patients with known or suspe...

...reasonable to measure the aorta from...

...inner-edge to inner-edge measurem...

...ial Elements of CT and MRI Aortic Imagin...

...iagnostic Performance of Aortic Imaging...

Figure 12. Aortic Imaging Techniques to Det...

...3. Reformatted CT Image Orthogonal to...

...erved Relationship Between Annual Institution...

.... Predicted Risk of Mortality Derived From...


Treatment

Treatmen...

...disciplinary Aortic Te...

...ecommendations for Multidisciplinary Aortic Team...

...For patients with acute aortic disease t...

...tients who are asymptomatic with exte...


...Shared Decision-Ma...

...0. Recommendations for Shared De...

...patients with aortic disease, shared deci...

...ients with aortic disease who are con...


...Aneurysms...

...ure 16. Recommendations for Management of Aneury...

...e 6. Cause of TAAHTAD (see Table 7): syndromic...

...Syndromes and Conditions Attributable to a Her...

...8. Risk Factors for Familial TAD...

...Genetic Testing and Screening of Family Member...

...n patients with aortic root/ascending aortic aneur...

...ents with aortic root/ascending aortic aneurys...

...ents with an established pathogenic or like...

...In patients with TAD who have a pathogenic/likely...

...ily with aortic root/ascending aortic...

...with aortic root/ascending aortic a...

...atients with acute type A aortic dissection...

...ure 17. Evaluation and Genetic Testing Protocol...

...Surgical Considerations for Non-syndromic Herit...

In asymptomatic patients with aneurysms of...

...atic patients with aneurysms of the...

...ts with aneurysms of the aortic root or...

...Features Associated With an Increased Risk of...

...able Thoracic Aortic Aneurysms and No Iden...

Family history of aortic dissec...

.... Diagnostic and Surveillance Aortic Im...

...tial Diagnosis and Surveillance I...

...diameters are stable, an annual surveillance TT...

...ot, ascending aorta, or both are not adequate...

...lts with Marfan syndrome, after the initi...

...After Aortic Root Replacemen...

...patients with Marfan syndrome who...

...n patients with Marfan syndrome who...

.... Medical Therapy in Marfan Syndrome...

...nts with Marfan syndrome, treatment wit...

...In patients with Marfan syndrome, the...

....2.2.3. Marfan Syndrome Interventions: Replaceme...

...s with Marfan syndrome and an aortic root diameter...

...n patients with Marfan syndrome, an aortic root d...

...patients with Marfan syndrome and a maximal cros...

...atients with Marfan syndrome and an a...

...ble 10. Features Associated With Incr...

...Marfan Syndrome Interventions: Replacement of...

...nts with Marfan syndrome and a nondisse...

...Imaging in Loeys-Dietz Syndrome...

...nts with Loeys-Dietz syndrome, a basel...

...with Loeys-Dietz syndrome and a dilat...

...In patients with Loeys-Dietz syndrom...

In patients with Loeys-Dietz syndro...

In patients with Loeys-Dietz syndrome with...

...cal Therapy in Loeys-Dietz Syndrome...

...atients with Loeys-Dietz syndrome, tr...

...-Dietz Syndrome Surgical Interventions: Replacem...

...In patients with Loeys-Dietz syndrome...

...In patients with Loeys-Dietz syndrome a...

...11. Surgical Thresholds for Prophyl...

...5. Turner Syndrome: Diagnostic Testing, Surveilla...

...nts with Turner syndrome, TTE and ca...

...ith Turner syndrome who are ≥15 years ol...

...tients with Turner syndrome without risk...

...patients with Turner syndrome and an ASI >2...

...with Turner syndrome and risk factors fo...

...actors for aortic dissection (Table 12), sur...

...out risk factors for aortic dissection, sur...

...Factors for Aortic Dissection in Patients Wi...

...uggested Aortic Monitoring Protocol for Girls...

...Surgical Thresholds for Prophylactic Aortic Root...

...BAV Aortopathy

...ith a BAV, TTE is indicated to evalu...

...ents with a BAV, CT or MRI of the thoracic...

...patients with a BAV and either HTAD or p...

...tients with a BAV and a dilated aortic root or...

...ents with a BAV, screening of all first-...

...ine Follow-Up of BAV Disease Aortopathy...

...tients with a BAV who have undergone previ...

...n patients with a BAV and a diameter of the...

....2. BAV Aortopathy Interventions: Replaceme...

...s with a BAV and a diameter of the aorti...

...with a BAV and a cross-sectional aortic ro...

...tients with a BAV, a diameter of the aortic root o...

...ents with a BAV who are undergoing surgical aorti...

...with a BAV, a diameter of the aortic r...

...Risk Factors for Aortic Dissection Family...

...: Cause, Risk Factors, and Screenin...

...are ≥65 years of age who have ever smoked, u...

...In men or women who are ≥65 years of...

...In women who are ≥65 years of age who...

...In men or women...

...In asymptomatic men or women >75 year...

...re 19. Algorithm for Identifying Patients...

...ble 15. Risk Factors for Abdominal Aortic Aneurysm...

...Management in Sporadic TAA...

...nts with TAA and an average systol...

...ients with TAA, regardless of cause and in the...

...ients with TAA, regardless of etiology and i...

6.4.1.2. Treatment of TAA W...

...n patients with TAA and imaging or clinic...

...ts with TAA who have no evidence of a...

...1.3. Smoking Cessation in TAA...

...nts with TAA who smoke cigarettes, smoking cessati...

...ntiplatelet Therapy in TAA...

...tients with atherosclerotic TAA and concomitan...

6.4.2.1. BP Management i...

...patients with AAA and an average SBP...

...2.2. Treatment of AAA With Stat...

...with AAA and evidence of aortic atherosclerosis,...

In patients with AAA but no evidence of ath...

...Smoking Cessation in AAA...

In patients with AAA who smoke cigarettes...

...tithrombotic Therapy in AAA...

...patients with AAA with concomitant atheroma...

6.4.3.1 Surveillance of Thoracic Aortic Dila...

...tients with a dilated thoracic aorta,...

...nts with a dilated thoracic aorta, a C...

...ts with a dilated thoracic aorta, f...

....3.2. Surveillance of Abdominal Aortic Dilation...

...patients with an AAA of 3.0 cm to...

...an AAA of 4.0 cm to 4.9 cm and in women with...

...th an AAA of ≥5.0 cm and women with an AAA...

...e CT is recommended. ( C-EO , I )706...

..., when there is a contraindication to CT or t...

...atients with an AAA that meets criter...

...e Frequency of Surveillance Imaging...

6.5.1. Surgery for Sporadic Aneurysms of the Ao...

...patients with aneurysms of the aortic...

...tomatic patients with aneurysms of...

...patients with an aneurysm of the aort...

...symptomatic patients with aneurysms...

...ents undergoing repair or replacement of...

In patients undergoing repair or replacement of a...

...s undergoing cardiac surgery for indications othe...

...patients with a height >1 standard deviation abo...

...matic patients with aneurysms of the aortic root...

.... Surgical Approach for Patients With Sporadic...

...nts with an aneurysm isolated to the ascendi...

...undergoing aortic valve repair or replacemen...

...nts undergoing aortic root replacement...

...n patients undergoing aortic root repl...

...2. Aortic Arch Aneurysms

...atients with an aortic arch aneurysm who have...

...tients with an isolated aortic arch aneurysm wh...

...ts undergoing open surgical repair of an a...

...patients undergoing open surgical repair of an aor...

...In patients with an aortic arch ane...

....5.3.1. Size Thresholds for Repair of Descend...

...with intact descending TAA, repair is recommen...

...nts with intact descending TAA and risk factors fo...

...t increased risk for perioperative morbidi...

...dverse Aortic Events at 1 Year, Bas...

...Factors for Aortic Rupture Among Patien...

...Risk Features for Rupture Aneurysm grow...

...ble 18. Patient Characteristics Associate...

...ascular Versus Open Repair of Descending...

...ithout Marfan syndrome, Loeys-Dietz syndrome, or v...

...In patients with a descending TAA that meets...

...tients with a descending TAA that meet...

...3.3. Left Subclavian Artery Manage...

In patients with descending TAA wh...

...nts with descending TAA who have undergone TEVAR...

.... Celiac Artery Management...

...In patients with descending TAA un...

....3.5. Ruptured Descending T...

...ents with ruptured descending TAA wh...

...ents with ruptured descending TAA un...

....6. Access Issues for TEVAR in Descend...

...with descending TAA undergoing TEVAR, review o...

...patients with descending TAA undergo...

...s with descending TAA undergoing TEVAR who have...

....5.4.1. Size Thresholds for Open Surgical...

...ents with intact degenerative TAAA, repair is rec...

...n patients with intact degenerative TAAA, repai...

...In patients with intact degenerative...

.... Features Associated With an Increased...

...Versus Endovascular Repair of TAAA...

Ruptured TAAA

...ith ruptured TAAA requiring intervention, o...

...ients with ruptured TAAA requiring interve...

...tact TAAA

...patients with Marfan syndrome, Lo...

...s with intact degenerative TAAA and suitabl...

.... TAAA Spinal Cord Protection...

...In patients undergoing open TAAA repair wh...

...patients who experience delayed spinal cord dysf...

...asures to Optimize Spinal Cord and End-Orga...

...A Renal and Visceral Organ Protection

...undergoing open repair of TAAA involving...

...ts undergoing open or endovascular TAAA repair...

....1. Access During Endovascular Repair of...

...s undergoing endovascular repair of AAA w...

...5.2. Repair of Ruptured AAA

...In patients presenting with ruptured AAA who are...

...nts presenting with ruptured AAA who...

...ts undergoing endovascular repair for rupt...

...In patients with ruptured AAA, permissive...

....5.3. Threshold for AAA Repai...

...s with unruptured AAA, repair is recommended in...

...patients with unruptured AAA who have sy...

...nts with unruptured saccular AAA, interv...

In patients with unruptured AAA and ane...

6.5.5.4. Open Versus Endovascular Re...

...ith nonruptured AAA with low to mod...

...undergoing elective endovascular repai...

...ith nonruptured AAA and a high perioperative...

...For patients with nonruptured AAA,...

...5.5. Treatment of Concomitant Common Iliac...

...patients with asymptomatic small AA...

...ting common iliac artery aneurysms or ectasia...

...Surveillance After TAA Repair...

...patients treated with TEVAR, surveillan...

...ts treated with TEVAR, longitudinal surveill...

...patients treated with open repair of the t...

...treated with open repair of the thoracic aorta w...

....5.6.2. Surveillance After AAA Re...

...tients with AAA treated with EVAR, baseline s...

...s with AAA treated with EVAR who are unde...

...patients with AAA treated with EVAR and abnor...

...s with AAA treated with complex EVAR,...

...n patients with AAA who have undergone open re...

.... Abnormal Findings on Duplex Imaging After EVAR...


...Acute Aortic Syndromes

...gns and Symptoms of AASHaving troubl...

...AS: Diagnostic Evaluation (Imaging, Laborato...

...patients with a suspected AAS, CT is recommended...

...nts with a suspected AAS, TEE and MRI are...

Table 23. Plain Chest X-Ray Findings Suggesti...

...Aortic Dissection on Chest X-Ray Medi...

...tic Dissection Detection Risk Score (AD...

...ta Simplified Score (AORTAs) Pretest Probabi...

...cute Medical Management of AAS...

In patients presenting to the hospital wit...

...with AAS should be treated to an SBP...

...l management should include intravenous be...

...ith contraindications or intolerance to beta blo...

...In patients with AAS, initial management should...

...th AAS should be treated with pain...

...Acute Aortic Dissection: Malperfusion...

...3.2. Subsequent Medical Manageme...

...with AAS, it is recommended to trea...

...Surgical Considerations in Acute Type A Aortic...

...atients presenting with suspected or confirmed...

...atients presenting with acute type A...

...presenting with non-hemorrhagic str...

.... Management of Malperfusion...

...ients with acute type A aortic dissection presenti...

...ents with acute type A aortic dissection pr...

...nical Evidence of Malperfusion (“...

...cal Repair Strategies in Acute Type A Aortic Diss...

...ic Repair Strategies...

...with acute type A aortic dissection a...

...c root replacement is recommended with a mechani...

...lected patients who are stable, valve-sparing r...

...patients with acute type A aortic dissection unde...

...nts with acute type A aortic dissection with...

...s with acute type A aortic dissection and a dis...

...rfusion and Cannulation Strategi...

...ients with acute type A aortic dissectio...

...ts with acute type A aortic dissection underg...

...patients with acute type A aortic dissection...

....2. Management of Acute Type B Aortic Dis...

...all patients with uncomplicated acute type...

...tion is recommended. ( C-LD , I )706...

...patients with rupture, in the pres...

...with other complications, in the presence of su...

...ts with uncomplicated acute type B aortic d...

...7. Consensus Features of Complicated...

...28. High-Risk Features in Uncompli...

...-Risk Imaging Findings...

...c diameter >40 mm False-lumen diameter >20–22...

High-Risk Clinical Findings

...ory hypertension despite >3 different classes...

7.5. Management...

...nts with complicated (Table 29) acute type A o...

...n surgical repair is recommended. ( B-...

...ted patients with uncomplicated acute type A I...

...In patients with uncomplicated acute type B IMH,...

...In patients with type B IMH who require repai...

...In patients with type B IMH who require repair...

...In patients with uncomplicated type B IMH and hi...

...29. Features of Complicate...

Featu...

...Malperfusion Periaortic hematoma Pericardial...

...isk Imaging Features of IMHHaving trou...

7.6.1. PAU With IMH, Rupture,...

In patients with PAU of the aorta...

...nts with PAU of the ascending aorta with...

...tients with PAU of the aortic arch or d...

...ts with PAU of the abdominal aorta...

...2. Isolated PAU

...patients with isolated PAU who are symptom...

...In patients with isolated PAU who are asymp...

...31. High-Risk Imaging Features...

Featur...

...ximum PAU diameter ≥13–20 mm Maximu...

...sions of Penetrating Atherosclerotic UlcersA,...

....3. PAU Open Surgical Repair Versus Endovascular...

...patients who require repair of a PAU in th...

...patients who require repair of a PAU in...

...1.1. Initial Management of BTTAI in the E...

...patients with BTTAI, management and treatment at...

...with BTTAI, anti-impulse therapy to...

...1.2. Approach to the Initial Manageme...

...In patients with grade 1 BTTAI (Figure 2...

...nts with grade 3 to 4 BTTAI (Figur...

...patients with grade 2 BTTAI (Figure 23) and with...

...s with grade 2 BTTAI (Figure 23) a...

...ssification System for BTTAIsAortic inju...

...32. High-Risk Imaging Features of BTTA...

...dovascular Versus Open Surgical Repair...

...nts with BTTAI who meet indications for repair...

...al Management of Blunt Traumatic Abdomina...

...In patients with grade 1 to 2 BAAI (Table 33) w...

...with grade 4 BAAI (Table 33), repair shoul...

...with grade 2 BAAI (Table 33) and associated...

In patients with BAAI, treatmen...

...nts with grade 3 BAAI (Table 33), it may be...

...nts with BAAI, the usefulness of routine app...

...criptions of Blunt Aortic Injury G...

....3. Long-Term Management and Surveillan...

...In patients with BTAI who have undergone aortic...

...In patients with BTAI who have not undergone r...

...4. Abdominal Aortic Zones of Injury f...

...erm Surveillance Imaging After Aortic...

...who have had an acute aortic dissection and IMH tr...

...patients who have had an acute aortic...

....8.2. Long-Term Management After Acute Aorti...

In patients with a previous acute...

...Long-Term Management and Surveilla...

...ith a PAU who have undergone aortic repair...

...ts with a PAU that is being managed medically, su...


...in Patients With Aortopathy...

...and Management of Aortic Disease in Pregnancy...

Prepregnancy

...atients with genetic aortopathies attributable...

...In patients with syndromic and nsHTAD...

...ts with syndromic and nsHTAD, Turner syn...

...g Pregnancy...

...nts with aortic aneurysms, or at inc...

...ients with aortopathies who are pregnant, gui...

...ents with syndromic and nsHTAD, bet...

...egnant patients with an aortopathic...

...n pregnant patients with aortic disease...

...very in Pregnant Patients With Aort...

...regnant patients with a history of chroni...

In pregnant patients with an aortopath...

...In pregnant patients with a diamet...

...In pregnant patients with a diameter of the aor...

...atients with syndromic and nsHTAD, and a...

...ore Pregnancy in Women With Aortic Disease...

...aortic root diameter of >4.5 cm, aortic surgery...

...e aortic root diameter is 4.0 cm to 4.5 cm, aort...

...or TGFB3 and an aortic diameter of ≥...

...FBR2, or SMAD3, and the aortic diamete...

...an aortic diameter of ≥4.5 cm, surgery bef...

...aortic diameter is 4.0 cm to 4.4 cm, sur...

...In patients with Turner syndrome and ASI of...

...patients with a BAV (in the absence...

In patients with sporadic aortic root ane...

...34. Prophylactic Aortic Surgery Before...

...ancy in Patients With Aortopathy:...

...experiencing an acute type A aorti...

...In patients experiencing an acute type A...

In patients experiencing an acute type...

...nts with progressive aortic dilation during pr...


...r Aortic Conditions ...

...matory Aortitis: Diagnosis and Treatment...

Diagno...

...s with large vessel vasculitis (LVV), prompt evalu...

...atment...

...In patients with active GCA or Takayas...

...ients with GCA who have evidence o...

...ients with Takayasu arteritis, nonbiol...

...with active GCA or Takayasu arteritis, t...

...ients with GCA or Takayasu arteritis who are in re...

...ith GCA or Takayasu arteritis and aortic...

...agnostic Criteria for Inflammatory A...

...iagnosis and Management of Infection of...

...repair is recommended. ( C-EO , I )...

...select patients, treatment with endovascu...

...ents with infectious aortitis complicated...

...ients with infectious aortitis, intraven...

...re 25. The 2018 European Alliance of Ass...

...re 26. The 2018 European Alliance of Associations...

.... Management of Aortic Mycotic Aneurysm: Compar...

...and Management of Prosthetic Aort...

...iagnosis...

...patients with a prosthetic aortic graft...

...reatment...

...n patients with an infected prosthetic aortic gra...

...nts with an infected prosthetic aorti...

...ients with an infected prosthetic aortic graft, en...

...te Management

...s who have undergone treatment of an a...

...ents with an infected prosthetic aortic...

...Atherosclerotic Dise...

...ients with aortic atherosclerotic dise...

...with aortic atherosclerotic disease and risk f...

...ith aortic atheromas of a thicknes...

...4.1. Coarctation of the Aort...

In patients with CoA, including those who...

...atients with CoA, BPs should be measu...

...In patients with significant nativ...

...patients with CoA, guideline-direc...

...In adult patients with CoA, screenin...

...e 37. Criteria for Significant...

...f significant CoA is based on evidence of upper...

...A noninvasive blood pressure differe...

...berrant Subclavian Artery, Kommerell’s...

...atients discovered to have an ASCA in the absen...

...s with Kommerell’s diverticulum, de...

9.4.2.2. Aberrant Left Vertebra...

...tients with an aberrant left verte...

.... Measurements of Kommerell’s DiverticulumTwo di...

...Arch (Common Innominate and Left Carotid Artery...

...s with bovine arch (common innominate and left...

...al and Bovine Aortic Arch ConfigurationsA, Type I...


...ical Activity and Quality of Life...

...for Physical Activity and Quality of Life...

...For patients with significant aortic...

...who have undergone surgery for ao...

...ts with thoracic or abdominal aortic aneurysm...

For patients with clinically significant aorti...