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

...ntroducti...

...ing of the following tables may differ fro...


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

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

...Simplified Diagram Depicting the Key Hist...

...Classification of Aortic Anatomic Segments b...

...4. Freedom From Ascending Aortic...

...Relative Risk of Aortic Dissection by Size RangeT...

...ssification of Aortic Dissection Ch...

...6. Acute Aortic SyndromesIn aortic dissection,...

Figure 7. Classification of Acute Aor...

...Anatomic Reporting of Aortic Dissection...

...9. Mechanisms of Dynamic and Static...

...sification of Thoracoabdominal Aortic Aneurysm...

...ification of Endoleak TypesEndoleaks a...


Assessment

...ssessme...

...ging and Measurements...

...tic Imaging Techniques to Determine...

...ents with known or suspected aortic...

...nts with known or suspected aortic...

...ts with known or suspected aortic dise...

...with known or suspected aortic disease, the aorti...

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

...t is reasonable to measure the aorta from...

Using inner-edge to inner-edge measu...

...al Elements of CT and MRI Aortic Ima...

...agnostic Performance of Aortic Imagi...

...Aortic Imaging Techniques to Dete...

...matted CT Image Orthogonal to the Aorti...

.... Observed Relationship Between Annual Institu...

...ure 15. Predicted Risk of Mortality Derived Fr...


Treatment

...reatme...

...ciplinary Aortic Teams...

...Recommendations for Multidisciplinary Aort...

...tients with acute aortic disease that req...

...ents who are asymptomatic with extensive...


5. Shared Decision-Ma...

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

...In patients with aortic disease, shared dec...

...patients with aortic disease who are conte...


...Aneurysms...

...e 16. Recommendations for Management of A...

...6. Cause of TAAHTAD (see Table 7): syn...

...ndromes and Conditions Attributable to...

...e 8. Risk Factors for Familial TAD TA...

....2.1. HTAD: Genetic Testing and Screening of...

...In patients with aortic root/ascending a...

...In patients with aortic root/ascending aort...

...nts with an established pathogenic or likely path...

...nts with TAD who have a pathogenic/likely pathog...

...In a family with aortic root/ascen...

In patients with aortic root/ascending a...

...In patients with acute type A aortic dissec...

...Evaluation and Genetic Testing Protocol fo...

...rgical Considerations for Non-syndromic Heritab...

...mptomatic patients with aneurysms of t...

In asymptomatic patients with aneurys...

...ts with aneurysms of the aortic ro...

...e 9. Features Associated With an Increased Risk o...

...ic Aortic Aneurysms and No Identified Geneti...

...amily history of aortic dissection...

....1.2.2.1. Diagnostic and Surveillanc...

...l Diagnosis and Surveillance Imag...

if the aortic diameters are stable, an annual su...

If the aortic root, ascending aorta...

...h Marfan syndrome, after the initial TTE, a...

Imaging After Aortic Root...

...tients with Marfan syndrome who have under...

...nts with Marfan syndrome who have undergone ao...

...1.2.2.2. Medical Therapy in Marfan Syndrom...

...with Marfan syndrome, treatment with...

...nts with Marfan syndrome, the use of both a be...

...an Syndrome Interventions: Replacement of...

...ents with Marfan syndrome and an aortic root...

...atients with Marfan syndrome, an aortic root di...

...tients with Marfan syndrome and a maxim...

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

...ble 10. Features Associated With Increased Ri...

....2.4. Marfan Syndrome Interventions: Repl...

...with Marfan syndrome and a nondissected aneurysm...

...Imaging in Loeys-Dietz Syndro...

...with Loeys-Dietz syndrome, a baseline TT...

...ith Loeys-Dietz syndrome and a dilated or dissec...

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

...In patients with Loeys-Dietz syndrome without...

...with Loeys-Dietz syndrome without...

6.1.2.3.2. Medical Therapy in Loeys-Dietz Syndro...

...tients with Loeys-Dietz syndrome, treatmen...

....1.2.3.3. Loeys-Dietz Syndrome Surgi...

...patients with Loeys-Dietz syndrome and ao...

...patients with Loeys-Dietz syndrome attr...

...urgical Thresholds for Prophylactic...

...urner Syndrome: Diagnostic Testing, Surveilla...

...tients with Turner syndrome, TTE and cardiac...

...ients with Turner syndrome who are â‰...

...with Turner syndrome without risk factors for aort...

...In patients with Turner syndrome and an A...

...In patients with Turner syndrome...

...s for aortic dissection (Table 12)...

...without risk factors for aortic dissection, surgi...

...ble 12. Risk Factors for Aortic Dissecti...

...18. Suggested Aortic Monitoring Protoco...

...able 13. Surgical Thresholds for Prophylactic A...

...3. BAV Aortopathy...

...In patients with a BAV, TTE is indicated to eva...

...ts with a BAV, CT or MRI of the thoracic aor...

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

...ts with a BAV and a dilated aortic root or ascend...

...ients with a BAV, screening of all...

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

...ith a BAV who have undergone previous a...

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

....1.3.2. BAV Aortopathy Interventions: Replacement...

...ith a BAV and a diameter of the aortic root,...

...patients with a BAV and a cross-sectional...

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

...n patients with a BAV who are undergoing surgical...

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

...k Factors for Aortic Dissection Family hist...

...2. AAA: Cause, Risk Factors, and Screening

...o are ≥65 years of age who have ever...

...r women who are ≥65 years of age and who ar...

In women who are ≥65 years of age...

...men or women...

...In asymptomatic men or women >75 years who...

...Algorithm for Identifying Patients...

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

...BP Management in Sporadic T...

In patients with TAA and an average syst...

...ts with TAA, regardless of cause and i...

...ts with TAA, regardless of etiology a...

...atment of TAA With Statins...

...patients with TAA and imaging or clinica...

...atients with TAA who have no evidence of atheroscl...

.... Smoking Cessation in TA...

...patients with TAA who smoke cigarettes,...

.... Antiplatelet Therapy in TAA...

...with atherosclerotic TAA and concomitant aor...

....4.2.1. BP Management...

...patients with AAA and an average SB...

...Treatment of AAA With Stati...

...ith AAA and evidence of aortic atheroscler...

...ts with AAA but no evidence of atheroscler...

....2.3. Smoking Cessation in AA...

...nts with AAA who smoke cigarettes, smoking ces...

...Antithrombotic Therapy in A...

...atients with AAA with concomitant ather...

6.4.3.1 Surveillance of Thoracic Aortic Dilation a...

...In patients with a dilated thoracic aor...

...nts with a dilated thoracic aorta, a CT or M...

...s with a dilated thoracic aorta, foll...

6.4.3.2. Surveillance of Abdominal Aortic Dilatio...

...ients with an AAA of 3.0 cm to 3.9 cm...

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

...n men with an AAA of ≥5.0 cm and women with a...

...ance CT is recommended. ( C-EO , I )...

...patients, when there is a contrain...

...n patients with an AAA that meets criteria for rep...

...Frequency of Surveillance Imaging of Abdom...

...5.1. Surgery for Sporadic Aneurysms o...

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

...mptomatic patients with aneurysms of t...

...ts with an aneurysm of the aortic root or as...

...matic patients with aneurysms of the a...

...s undergoing repair or replacement of a...

In patients undergoing repair or replacement of a...

...dergoing cardiac surgery for indica...

In patients with a height >1 standard...

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

...1. Surgical Approach for Patients Wi...

...tients with an aneurysm isolated to the as...

In patients undergoing aortic valve repair or...

...patients undergoing aortic root replacement with a...

...atients undergoing aortic root rep...

...Aortic Arch Aneurysms...

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

...ts with an isolated aortic arch aneurysm who ar...

In patients undergoing open sur...

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

...ts with an aortic arch aneurysm who are as...

...Size Thresholds for Repair of Descending TAA...

...ith intact descending TAA, repair i...

...tients with intact descending TAA and r...

...at increased risk for perioperative...

...se Aortic Events at 1 Year, Based on Baseline...

...7. Risk Factors for Aortic Rupture Among Pati...

...Features for Rupture Aneurysm growth of...

...18. Patient Characteristics Associated With I...

...dovascular Versus Open Repair of Descending TA...

...ithout Marfan syndrome, Loeys-Dietz syndrom...

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

...atients with a descending TAA that meets...

...eft Subclavian Artery Management...

...atients with descending TAA who undergo TEVAR w...

...atients with descending TAA who have und...

.... Celiac Artery Management...

...atients with descending TAA undergoing TEVA...

....5.3.5. Ruptured Descending TAA

...In patients with ruptured descending TAA who ar...

...atients with ruptured descending TAA undergo...

...s Issues for TEVAR in Descending TAA...

...patients with descending TAA undergoing...

...In patients with descending TAA undergoing...

...ents with descending TAA undergoing TEVAR who ha...

...resholds for Open Surgical Repair of TAAA...

...In patients with intact degenerative TAAA,...

...ith intact degenerative TAAA, repair is...

...tients with intact degenerative TAAA who h...

...19. Features Associated With an Increased Risk o...

....2. Open Versus Endovascular Repair of TAAA...

Ruptured TA...

...In patients with ruptured TAAA requiring interv...

...In patients with ruptured TAAA requir...

...act TAAA...

...patients with Marfan syndrome, Loeys-Dietz...

...ts with intact degenerative TAAA and s...

....5.4.3. TAAA Spinal Cord Protec...

...tients undergoing open TAAA repair who are at...

...ts who experience delayed spinal cord d...

...le 20. Measures to Optimize Spinal Cord an...

....4. TAAA Renal and Visceral Organ Protection...

...ts undergoing open repair of TAAA invol...

In patients undergoing open or e...

....5.5.1. Access During Endovascular Repair of AA...

...patients undergoing endovascular repair o...

....5.5.2. Repair of Ruptured A...

...presenting with ruptured AAA who are hemodynamic...

...ents presenting with ruptured AAA who hav...

...tients undergoing endovascular repair for ruptured...

...In patients with ruptured AAA, permi...

...Threshold for AAA Repair...

...patients with unruptured AAA, repair is recommen...

...tients with unruptured AAA who have sympto...

...In patients with unruptured saccular...

...ents with unruptured AAA and aneurysm growth of â‰...

....5.4. Open Versus Endovascular Repair of...

In patients with nonruptured AAA with low t...

...In patients undergoing elective endovascular re...

...In patients with nonruptured AAA and a high per...

For patients with nonruptured AAA, a moderate t...

...nt of Concomitant Common Iliac Aneurysms...

...ients with asymptomatic small AAA a...

When treating common iliac artery a...

...rveillance After TAA Repair...

...treated with TEVAR, surveillance im...

...tients treated with TEVAR, longitudinal surve...

...s treated with open repair of the thoraci...

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

....6.2. Surveillance After AAA Repa...

...ts with AAA treated with EVAR, baseline...

...ith AAA treated with EVAR who are un...

In patients with AAA treated with EVAR a...

...with AAA treated with complex EVAR, a modif...

...In patients with AAA who have undergone open r...

...mal Findings on Duplex Imaging After...


...Acute Aortic Syndromes...

...Signs and Symptoms of AASHaving t...

7.2. AAS: Diagnostic Evaluation (Imaging, L...

...In patients with a suspected AAS, CT is rec...

...n patients with a suspected AAS, TEE and MRI are r...

...ain Chest X-Ray Findings Suggestive of Aort...

...gns of Aortic Dissection on Chest X-Ray M...

...able 24. Aortic Dissection Detection Risk Sc...

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

...Acute Medical Management of A...

...ients presenting to the hospital w...

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

...anagement should include intravenous be...

...those with contraindications or int...

...nts with AAS, initial management s...

...with AAS should be treated with pain control, a...

...1. Acute Aortic Dissection: Malperfusio...

....3.2. Subsequent Medical Management o...

...ts with AAS, it is recommended to t...

...nitial Surgical Considerations in Acute Ty...

...s presenting with suspected or confirmed acut...

...ts presenting with acute type A aortic di...

...ts presenting with non-hemorrhagic stroke comp...

...nagement of Malperfusion...

...s with acute type A aortic dissectio...

...patients with acute type A aortic dissect...

...26. Clinical Evidence of Malperfusion (“Malpe...

....4.1.3. Surgical Repair Strategies in Acute Typ...

...rtic Repair Strategies

...ts with acute type A aortic dissection and a parti...

...root replacement is recommended with a...

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

...with acute type A aortic dissection un...

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

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

...d Cannulation Strategies...

...n patients with acute type A aortic...

...s with acute type A aortic dissection under...

In patients with acute type A aortic dissect...

...ement of Acute Type B Aortic Dissection...

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

...vention is recommended. ( C-LD , I )70...

...ients with rupture, in the presence of suitable...

...tients with other complications, in the pre...

...ents with uncomplicated acute type B aortic di...

...ble 27. Consensus Features of Complicate...

...able 28. High-Risk Features in Uncomplicat...

...igh-Risk Imaging Fin...

...ic diameter >40 mm False-lumen diameter >20–...

...h-Risk Clinical Findings

...y hypertension despite >3 different classes of...

...anagement of IMH...

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

...surgical repair is recommended. ( B-NR , I )7...

...patients with uncomplicated acute type A...

...with uncomplicated acute type B IMH, med...

...with type B IMH who require repair of the distal...

...In patients with type B IMH who req...

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

...29. Features of Complicat...

Featur...

...n Periaortic hematoma Pericardial ef...

...igh-Risk Imaging Features of IMHHaving...

...ith IMH, Rupture, or Both...

...atients with PAU of the aorta with rupt...

...ients with PAU of the ascending aorta with a...

...ts with PAU of the aortic arch or descen...

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

...2. Isolated PA...

...tients with isolated PAU who are symp...

...patients with isolated PAU who are asymptomatic bu...

Table 31. High-Risk Imaging Featu...

...ature...

...um PAU diameter ≥13–20 mm Maximum PAU depth...

...gure 22. Dimensions of Penetrating Atheroscler...

...PAU Open Surgical Repair Versus Endovascular Rep...

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

...ho require repair of a PAU in the...

...Management of BTTAI in the Emergen...

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

...atients with BTTAI, anti-impulse therap...

...Approach to the Initial Management...

...atients with grade 1 BTTAI (Figure 23...

...patients with grade 3 to 4 BTTAI (Fig...

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

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

...3. Classification System for BTTAIsA...

...-Risk Imaging Features of BTTAI Posterior medi...

...Endovascular Versus Open Surgical Repair...

...ents with BTTAI who meet indications for repair an...

...ial Management of Blunt Traumatic Abdominal Aort...

...n patients with grade 1 to 2 BAAI (Table 33...

...ith grade 4 BAAI (Table 33), repai...

...patients with grade 2 BAAI (Table 33) and asso...

...patients with BAAI, treatment wit...

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

...tients with BAAI, the usefulness of routine appl...

.... Descriptions of Blunt Aortic Injur...

...ng-Term Management and Surveillance A...

...patients with BTAI who have undergone...

...atients with BTAI who have not undergone re...

...dominal Aortic Zones of Injury for Su...

...Long-Term Surveillance Imaging After Ao...

...who have had an acute aortic dissection a...

...In patients who have had an acute...

...-Term Management After Acute Aortic Diss...

...patients with a previous acute aortic diss...

...m Management and Surveillance for PAUs...

...ts with a PAU who have undergone aortic r...

...patients with a PAU that is being manag...


...regnancy in Patients With Aortopathy...

...eling and Management of Aortic Disease...

...epregnancy...

...s with genetic aortopathies attrib...

...patients with syndromic and nsHTAD,...

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

During Pregna...

...ents with aortic aneurysms, or at increased ris...

...patients with aortopathies who are pre...

...ith syndromic and nsHTAD, beta-blocker therap...

...In pregnant patients with an aortopat...

...nant patients with aortic disease wh...

...ivery in Pregnant Patients With Aor...

...regnant patients with a history of chron...

...patients with an aortopathy and an aortic di...

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

In pregnant patients with a diameter of...

...pregnant patients with syndromic and nsHTAD, a...

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

...ic root diameter of >4.5 cm, aortic su...

...aortic root diameter is 4.0 cm to 4...

...B3 and an aortic diameter of ≥4.5...

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

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

...ortic diameter is 4.0 cm to 4.4 cm,...

In patients with Turner syndrome an...

...with a BAV (in the absence of Turn...

...s with sporadic aortic root aneurys...

...ylactic Aortic Surgery Before Pregnancy in Women W...

...Pregnancy in Patients With Aortopathy: Aortic D...

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

...atients experiencing an acute type A aortic di...

...ients experiencing an acute type B aortic...

...tients with progressive aortic dilation duri...


...Other Aortic Conditions...

...ory Aortitis: Diagnosis and Treatment of T...

...iagnosis...

...ith large vessel vasculitis (LVV), promp...

...eatmen...

...tients with active GCA or Takayasu arte...

...ts with GCA who have evidence of active...

...ients with Takayasu arteritis, nonbiolo...

...s with active GCA or Takayasu arteritis, treat...

...In patients with GCA or Takayasu arterit...

...with GCA or Takayasu arteritis an...

...Diagnostic Criteria for Inflammator...

...osis and Management of Infection of the Native Ao...

...en surgical repair is recommended. ( C-EO ,...

...tients, treatment with endovascular...

...patients with infectious aortitis complic...

...s with infectious aortitis, intravenous...

Figure 25. The 2018 European Alliance...

...018 European Alliance of Associations for Rheumat...

...ble 36. Management of Aortic Mycotic Aneurysm...

...sis and Management of Prosthetic Aortic Graft...

...iagnos...

...s with a prosthetic aortic graft, who have sig...

...reatmen...

...In patients with an infected prosth...

...In patients with an infected prosth...

...s with an infected prosthetic aortic graft, endo...

Late Managem...

...ho have undergone treatment of an acute...

...nts with an infected prosthetic aor...

...herosclerotic Disease...

...patients with aortic atherosclerotic d...

...ents with aortic atherosclerotic disease...

...tients with aortic atheromas of a thic...

...oarctation of the Aorta...

...tients with CoA, including those who have u...

...s with CoA, BPs should be measured in both a...

...ts with significant native or recurrent C...

...s with CoA, guideline-directed medical therapy is...

...patients with CoA, screening for in...

...7. Criteria for Significant...

The presence of significant CoA is based on ev...

...sive blood pressure difference of >20 mm Hg...

...rant Subclavian Artery, Kommerell’s D...

...discovered to have an ASCA in the abs...

...nts with Kommerell’s diverticulum, d...

...Aberrant Left Vertebral Artery Origin...

...with an aberrant left vertebral artery...

...asurements of Kommerell’s DiverticulumTwo...

....4.2.3. Bovine Arch (Common Innominate and...

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

Figure 28. Normal and Bovine Aortic Arch C...


...Physical Activity and Quality...

...commendations for Physical Activity...

...patients with significant aortic disease,...

...For patients who have undergone su...

...nts with thoracic or abdominal aortic aneurysms wh...

...with clinically significant aortic disease,...