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

...Introduction...

...The numbering of the following tables...


...2...

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

.... A Simplified Diagram Depicting the Key Histologi...

...Classification of Aortic Anatomic Segments...

...om From Ascending Aortic Complications for ...

...ure 5. Relative Risk of Aortic Dissection by Si...

...fication of Aortic Dissection Chronici...

...ure 6. Acute Aortic SyndromesIn aor...

Figure 7. Classification of Acute Aortic...

...ure 8. Anatomic Reporting of Aortic Disse...

...re 9. Mechanisms of Dynamic and Static Ob...

...Classification of Thoracoabdominal Aortic ...

...gure 11. Classification of Endoleak T...


Assessment

...Assessment

...3....

...3.1 Aortic Imag...

...patients with known or suspected aorti...

...In patients with known or suspected aortic dise...

...atients with known or suspected aortic d...

...with known or suspected aortic dis...

...patients with known or suspected aortic dis...

...e to measure the aorta from leading-edge...

...inner-edge to inner-edge measuremen...

...sential Elements of CT and MRI Aor...

...Diagnostic Performance of Aortic Im...

...ortic Imaging Techniques to Determin...

...eformatted CT Image Orthogonal to the Aortic Root...

...gure 14. Observed Relationship Between An...

...re 15. Predicted Risk of Mortality Derived...


Treatment

...Treatmen...

...4....

...4.0....

...nts with acute aortic disease that...

...patients who are asymptomatic with exte...


...5. Shared De...

...5.0. R...

...n patients with aortic disease, shared d...

...atients with aortic disease who are contemplating...


...6. Aneurysms...

...Recommendations for Management of Aneury...

...Cause of TAAHTAD (see Table 7): syndromi...

...Syndromes and Conditions Attributable to a Herita...

...ctors for Familial TAD TAD and syndromi...

...6.1.2.1. HTAD...

...s with aortic root/ascending aortic aneurysms o...

...ents with aortic root/ascending aortic aneury...

...atients with an established pathogenic or l...

...with TAD who have a pathogenic/likely p...

...with aortic root/ascending aortic aneury...

...ith aortic root/ascending aortic aneury...

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

...7. Evaluation and Genetic Testing Protocol f...

...6.1.2.1.1. Surgic...

...tomatic patients with aneurysms of the a...

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

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

...T...

...Heritab...

...history of aortic dissection at an...

...6.1.2.2.1. Diagn...

...Initial Diagno...

...ameters are stable, an annual surveil...

...oot, ascending aorta, or both are not ade...

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

...Imaging After Aortic...

...patients with Marfan syndrome who have under...

...In patients with Marfan syndrome who have un...

...6.1.2.2.2. Medical T...

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

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

...6.1.2.2.3. Marfan Syn...

...ith Marfan syndrome and an aortic root diamet...

...In patients with Marfan syndrome, an a...

...s with Marfan syndrome and a maximal cross-...

...with Marfan syndrome and an aortic diameter appro...

...0. Features Associated With Increased Risk of Ao...

...6.1.2.2.4. Marfa...

...ients with Marfan syndrome and a nondissected...

...6.1.2.3.1. Imaging i...

...with Loeys-Dietz syndrome, a baseline TTE is...

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

...ents with Loeys-Dietz syndrome, a baseline M...

...ents with Loeys-Dietz syndrome without dilation o...

...ts with Loeys-Dietz syndrome without dilation...

...6.1....

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

...6.1.2.3.3. Loe...

...tients with Loeys-Dietz syndrome and aortic dilat...

...nts with Loeys-Dietz syndrome attributable to...

...rgical Thresholds for Prophylactic...

...6.1.2.5. T...

...n patients with Turner syndrome, T...

...tients with Turner syndrome who are ...

...ith Turner syndrome without risk factors fo...

...In patients with Turner syndrome and an ASI >2.3...

...ents with Turner syndrome and risk factors for...

...us risk factors for aortic dissection (Table 12),...

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

...sk Factors for Aortic Dissection in Patie...

...e 18. Suggested Aortic Monitoring Protocol...

...gical Thresholds for Prophylactic Aortic R...

...6.1.3. BAV A...

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

...s with a BAV, CT or MRI of the thoracic aort...

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

...In patients with a BAV and a dilated aortic ro...

...patients with a BAV, screening of all first...

...6.1.3.1. Ro...

In patients with a BAV who have...

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

...6.1.3.2. BAV Aortopathy...

...tients with a BAV and a diameter of the aort...

...nts with a BAV and a cross-sectional aorti...

...with a BAV, a diameter of the aortic root or...

...patients with a BAV who are undergoing surgica...

...In patients with a BAV, a diameter of the aort...

...Risk Factors for Aortic Dissection F...

...en who are ≥65 years of age who have eve...

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

...who are ≥65 years of age who have eve...

...In men or wome...

...atic men or women >75 years who have ha...

Figure 19. Algorithm for Identifying Patients to...

.... Risk Factors for Abdominal Aortic AneurysmHavi...

...6.4.1.1. BP Manageme...

...ents with TAA and an average systolic BP (SBP) o...

...In patients with TAA, regardless of cause...

In patients with TAA, regardless of etiology a...

...ts with TAA and imaging or clinical...

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

...6.4.1.3....

...In patients with TAA who smoke cigarettes...

...6.4.1.4. A...

...ith atherosclerotic TAA and concomit...

...6.4.2.1. BP M...

...atients with AAA and an average SBP of ...

...6.4.2.2. Treatmen...

...In patients with AAA and evidence o...

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

...6.4.2.3. Smo...

...atients with AAA who smoke cigarettes,...

...6.4.2...

...ents with AAA with concomitant ath...

...6.4.3...

...patients with a dilated thoracic aorta, a...

...In patients with a dilated thoracic aorta,...

...s with a dilated thoracic aorta, follow-up imagin...

...6.4...

...tients with an AAA of 3.0 cm to 3.9 cm, surve...

...In men with an AAA of 4.0 cm to 4.9 cm and in wom...

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

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

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

In patients with an AAA that meets...

...he Frequency of Surveillance Imaging of Abdomina...

...6.5.1. Surgery for Sporadi...

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

In asymptomatic patients with aneurysm...

...patients with an aneurysm of the aortic ro...

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

...ents undergoing repair or replacem...

...n patients undergoing repair or replacement of...

...ndergoing cardiac surgery for indications...

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

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

...6.5...

...ients with an aneurysm isolated to...

In patients undergoing aortic valve...

...In patients undergoing aortic root replacement wi...

...patients undergoing aortic root replacement,...

...6.5.2. Aortic Arc...

...n patients with an aortic arch aneurysm who...

...n patients with an isolated aortic arch aneurysm...

...ts undergoing open surgical repair o...

...ents undergoing open surgical repair of an aortic...

...ith an aortic arch aneurysm who are asymptom...

...6.5.3.1. Si...

...nts with intact descending TAA, repair is r...

...ith intact descending TAA and risk factors fo...

...ts at increased risk for perioperative morbidity...

...rse Aortic Events at 1 Year, Based o...

Tabl...

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

...ble 18. Patient Characteristics Assoc...

6.5.3.2. E...

...In patients without Marfan syndrome,...

...ith a descending TAA that meets criter...

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

...6.5.3.3. Left Su...

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

...n patients with descending TAA who have under...

...6.5.3.4. Ce...

...n patients with descending TAA underg...

...6.5.3.5. Ru...

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

...atients with ruptured descending TAA under...

...6.5.3.6. Access...

...n patients with descending TAA undergoing TEVAR,...

...with descending TAA undergoing TEVAR, if...

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

...6.5.4.1....

...ts with intact degenerative TAAA,...

...tients with intact degenerative TAAA, repair...

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

...tures Associated With an Increased Risk of TAAA Ru...

...6.5.4.2. Open Versu...

...R...

...atients with ruptured TAAA requiring interventio...

...ients with ruptured TAAA requiring inte...

...Intac...

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

...nts with intact degenerative TAAA and suitable...

...nts undergoing open TAAA repair who...

...atients who experience delayed spinal cord dysfun...

Table 20. Measures to Optimize Spina...

...6.5.4...

...tients undergoing open repair of TAAA involving th...

...ients undergoing open or endovascula...

...6.5.5...

...In patients undergoing endovascular r...

...6.5.5.2. R...

...ients presenting with ruptured AAA...

...ients presenting with ruptured AAA who h...

...In patients undergoing endovascular repair for...

...In patients with ruptured AAA, permissiv...

...6...

...n patients with unruptured AAA, repair is r...

...ith unruptured AAA who have symptoms...

...atients with unruptured saccular AAA, intervent...

...with unruptured AAA and aneurysm growth of ≥0.5...

...6.5.5.4. Open...

...s with nonruptured AAA with low to moderate operat...

In patients undergoing elective endo...

...In patients with nonruptured AAA and a hig...

...For patients with nonruptured AAA, a mod...

...6.5.5.5. Treatme...

...For patients with asymptomatic sma...

...hen treating common iliac artery aneurysms or...

...6...

...patients treated with TEVAR, surveillance imagin...

...In patients treated with TEVAR, longitu...

...ents treated with open repair of the thor...

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

...6.5.6.2. Sur...

...n patients with AAA treated with EVAR, b...

...tients with AAA treated with EVAR who are underg...

...nts with AAA treated with EVAR and ab...

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

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

...bnormal Findings on Duplex Imaging After E...


...7. Acute A...

...s and Symptoms of AASHaving troubl...

...7.2. AAS: Diagnosti...

...with a suspected AAS, CT is recommended for init...

...In patients with a suspected AAS, TEE and MRI...

...Table 23. Plai...

...tic Dissection on Chest X-Ray Mediastinal wid...

...Aortic Dissection Detection Risk Score (A...

...le 25. Aorta Simplified Score (AORTAs) Pr...

...7.3.1. Acute Medical M...

...ients presenting to the hospital with AAS,...

...atients with AAS should be treated to an S...

...ement should include intravenous beta block...

...se with contraindications or intolerance to bet...

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

...Patients with AAS should be treated with p...

...21. Acute Aortic Dissection: Malperfusion Treatm...

...7.3.2. Subsequent Me...

...tients with AAS, it is recommended to treat wi...

...7.4.1.1....

...atients presenting with suspected or confir...

...patients presenting with acute type...

In patients presenting with non-...

...7.4.1.2. Management...

...tients with acute type A aortic dissection...

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

...able 26. Clinical Evidence of Malperfusion...

...7.4.1.3. Surgical Rep...

...Aortic Repair Str...

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

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

...lected patients who are stable, valve-sp...

In patients with acute type A a...

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

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

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

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

...atients with acute type A aortic dissectio...

...7.4.2. Manage...

...In all patients with uncomplicated acute ty...

intervention is recommended. ( C-LD , I )...

...tients with rupture, in the presence of...

...tients with other complications, i...

...patients with uncomplicated acute type B ao...

.... Consensus Features of Complicated Acu...

...Table 28....

Hig...

...Maximal aortic diameter >40 mm Fa...

...High-Risk Clinical...

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

...7.5. Man...

In patients with complicated (Table 29) ac...

...en surgical repair is recommended. ( B-NR ,...

...lected patients with uncomplicated acute type...

In patients with uncomplicated acute type...

...patients with type B IMH who requir...

...n patients with type B IMH who require re...

...In patients with uncomplicated type B...

...Table 29. Features...

...Fea...

...Malperfusion Periaortic hematoma Pericardial...

...le 30. High-Risk Imaging Features of IMH...

...7.6.1. PAU...

...In patients with PAU of the aorta...

...In patients with PAU of the ascending aorta...

...s with PAU of the aortic arch or descending thora...

...n patients with PAU of the abdominal aorta with a...

...7.6.2. Isolated...

...with isolated PAU who are symptomatic...

...ts with isolated PAU who are asympto...

...Table 31. High-...

...iameter ≥13–20 mm Maximum PAU depth ≥...

...re 22. Dimensions of Penetrating Atherosclerotic U...

...7.6.3. PAU O...

...who require repair of a PAU in the ascending a...

...who require repair of a PAU in the distal aortic...

...7.7.1....

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

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

...7.7.1.2. Ap...

...ts with grade 1 BTTAI (Figure 23), nonoperative...

In patients with grade 3 to 4 BTTAI (Figure...

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

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

...gure 23. Classification System for BTTAIsAort...

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

...7.7.1.3. Endova...

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

...7.7.2. I...

...ts with grade 1 to 2 BAAI (Table 33) without malpe...

...tients with grade 4 BAAI (Table 33), r...

...In patients with grade 2 BAAI (Table 33) a...

...nts with BAAI, treatment with either endovas...

...n patients with grade 3 BAAI (Table 33),...

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

...scriptions of Blunt Aortic Injury GradesHaving t...

...7.7.3. Long-Ter...

...with BTAI who have undergone aortic repa...

...with BTAI who have not undergone...

...inal Aortic Zones of Injury for Surgic...

...7.8.1...

...n patients who have had an acute aor...

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

...7.8.2....

In patients with a previous acute aortic...

...7.8.3...

...In patients with a PAU who have undergone aortic...

...s with a PAU that is being managed medically,...


...8. Pregnancy in...

...8.1. Counseling...

...Prepregnancy

...patients with genetic aortopathies attrib...

...ients with syndromic and nsHTAD, Turner syndrom...

...patients with syndromic and nsHTAD, Turner s...

...Duri...

...In patients with aortic aneurysms, or at...

...In patients with aortopathies who are pregnant,...

...n patients with syndromic and nsHTAD, beta-b...

...t patients with an aortopathic condition o...

...In pregnant patients with aortic diseas...

...8.2. De...

...regnant patients with a history of chronic aort...

...patients with an aortopathy and an aortic dia...

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

...nant patients with a diameter of the ao...

...nant patients with syndromic and nsHTAD, and a dia...

...8.3. Surgery B...

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

...ot diameter is 4.0 cm to 4.5 cm, a...

...nd an aortic diameter of ≥4.5 cm...

...TGFBR2, or SMAD3, and the aortic diamet...

...n aortic diameter of ≥4.5 cm, surgery before...

...aortic diameter is 4.0 cm to 4.4 cm, surgery b...

...patients with Turner syndrome and ASI of ≥2...

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

...nts with sporadic aortic root aneurysms,...

...34. Prophylactic Aortic Surgery B...

...8.4. P...

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

...In patients experiencing an acute...

...n patients experiencing an acute type B ao...

...atients with progressive aortic dilation during pr...


...9. Other Aor...

...Diagnosis...

In patients with large vessel vasculitis...

...Treatment...

...with active GCA or Takayasu arteritis, initial m...

...s with GCA who have evidence of ac...

...all patients with Takayasu arteritis, non...

...patients with active GCA or Takayasu art...

In patients with GCA or Takayasu arteri...

...ients with GCA or Takayasu arteritis and...

...iagnostic Criteria for Inflammatory Aortit...

...9.2.1....

...rgical repair is recommended. ( C-EO...

...patients, treatment with endovascu...

...atients with infectious aortitis complicated...

...with infectious aortitis, intravenous antimi...

...re 25. The 2018 European Alliance o...

...2018 European Alliance of Associations for Rheumat...

...6. Management of Aortic Mycotic Aneurys...

...9.2.2. Diagnosis a...

...Diagnosis...

...In patients with a prosthetic aortic graft, wh...

...Treatment...

...with an infected prosthetic aortic graft who are...

...tients with an infected prosthetic aortic gr...

...with an infected prosthetic aortic...

...Late Management...

...ients who have undergone treatment of an acute...

In patients with an infected prosth...

...9.3. Atherosc...

...s with aortic atherosclerotic disease and con...

...In patients with aortic atherosclerotic...

...tients with aortic atheromas of a thickness ≥...

...ith CoA, including those who have underg...

...In patients with CoA, BPs should be measur...

...In patients with significant native or r...

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

...In adult patients with CoA, screening...

...Table 37. C...

...presence of significant CoA is based o...

...nvasive blood pressure difference of >20 mm...

...9.4.2.1...

...atients discovered to have an ASCA in t...

...with Kommerell’s diverticulum, depending on p...

...9.4.2.2. Aberrant Left Ver...

...with an aberrant left vertebral arter...

.... Measurements of Kommerell’s Di...

...9.4.2.3....

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

...ure 28. Normal and Bovine Aortic Arch Conf...


...10. Physic...

Recomme...

...patients with significant aortic...

...patients who have undergone surgery for aort...

...with thoracic or abdominal aortic aneurysms who...

...patients with clinically significant aortic di...