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

...roduction

...ing of the following tables may differ from th...


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

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

Figure 2. A Simplified Diagram Depictin...

...gure 3. Classification of Aortic Anatom...

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

...5. Relative Risk of Aortic Dissecti...

...able 3. Classification of Aortic Disse...

...cute Aortic SyndromesIn aortic dissection, a tear...

...e 7. Classification of Acute Aortic Dissection...

...natomic Reporting of Aortic Dissection Based on ...

Figure 9. Mechanisms of Dynamic and...

...sification of Thoracoabdominal Aortic ...

...ification of Endoleak TypesEndolea...


Assessment

...sessmen...

.... Imaging and Measureme...

...1 Aortic Imaging Techniques to Determine P...

In patients with known or suspected a...

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

...patients with known or suspected aor...

In patients with known or suspec...

...patients with known or suspected aortic disease...

...reasonable to measure the aorta from leading-...

...-edge to inner-edge measurements may a...

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

...ostic Performance of Aortic Imaging ModalitiesHavi...

.... Aortic Imaging Techniques to Dete...

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

Figure 14. Observed Relationship Between...

...redicted Risk of Mortality Derived From the Log...


Treatment

...eatment

...ultidisciplinary Aortic T...

...commendations for Multidisciplinary...

...nts with acute aortic disease that requires urgen...

...or patients who are asymptomatic w...


...red Decision-Maki...

...ecommendations for Shared Decision-Maki...

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

...In patients with aortic disease who...


...neurysms...

...16. Recommendations for Management...

...use of TAAHTAD (see Table 7): syndromic Marfan...

...e 7. TAA Syndromes and Conditions Attribut...

...able 8. Risk Factors for Familial TA...

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

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

...atients with aortic root/ascending...

...ents with an established pathogenic...

...ith TAD who have a pathogenic/likely pathoge...

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

...tients with aortic root/ascending aortic ane...

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

...uation and Genetic Testing Protocol for...

...1.2.1.1. Surgical Considerations f...

...mptomatic patients with aneurysms of the aort...

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

...ents with aneurysms of the aortic root or as...

...es Associated With an Increased Risk of Aortic Dis...

...Thoracic Aortic Aneurysms and No Identified...

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

6.1.2.2.1. Diagnostic and Surveillance Aortic...

...itial Diagnosis and Surveillan...

...f the aortic diameters are stable, an annual...

...aortic root, ascending aorta, or both are...

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

...ging After Aortic Root Replacemen...

...with Marfan syndrome who have undergone a...

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

....2.2. Medical Therapy in Marfan...

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

...s with Marfan syndrome, the use of b...

...3. Marfan Syndrome Interventions: Replacement...

...In patients with Marfan syndrome and an aortic...

...ients with Marfan syndrome, an aort...

...patients with Marfan syndrome and a maxi...

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

...able 10. Features Associated With Increased R...

.... Marfan Syndrome Interventions: Re...

...ts with Marfan syndrome and a nondissected ane...

....3.1. Imaging in Loeys-Dietz Syndr...

...In patients with Loeys-Dietz synd...

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

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

...ith Loeys-Dietz syndrome without dilation of th...

...ith Loeys-Dietz syndrome without dilation...

...2.3.2. Medical Therapy in Loeys-Dietz Syndrome

...n patients with Loeys-Dietz syndrome...

.... Loeys-Dietz Syndrome Surgical Intervention...

...ients with Loeys-Dietz syndrome and...

...with Loeys-Dietz syndrome attributable...

...urgical Thresholds for Prophylactic...

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

...atients with Turner syndrome, TTE...

...atients with Turner syndrome who are â...

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

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

...In patients with Turner syndrome and r...

...factors for aortic dissection (Table 12), surg...

...n those without risk factors for aortic...

...Factors for Aortic Dissection in Patients W...

...re 18. Suggested Aortic Monitoring Pro...

...rgical Thresholds for Prophylactic Aortic...

....1.3. BAV Aortopath...

...In patients with a BAV, TTE is in...

...atients with a BAV, CT or MRI of the thorac...

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

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

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

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

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

...In patients with a BAV and a diameter of...

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

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

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

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

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

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

...Factors for Aortic Dissection Famil...

...Cause, Risk Factors, and Screening...

...n men who are ≥65 years of age who have ever s...

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

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

...In men or women...

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

...rithm for Identifying Patients to Screen for Ab...

...e 15. Risk Factors for Abdominal Aorti...

...Management in Sporadic TAA

...In patients with TAA and an average systolic...

...n patients with TAA, regardless of caus...

...patients with TAA, regardless of etiology and...

....4.1.2. Treatment of TAA With Statins

...nts with TAA and imaging or clinical evidence o...

...In patients with TAA who have no evidence of a...

....1.3. Smoking Cessatio...

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

...iplatelet Therapy in TAA...

...atients with atherosclerotic TAA and...

...BP Management in AAA...

...ents with AAA and an average SBP of ≥13...

...4.2.2. Treatment of AAA With Statins...

...with AAA and evidence of aortic ath...

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

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

...s with AAA who smoke cigarettes, smoking ce...

6.4.2.4. Antithrombotic Therapy in AA...

...tients with AAA with concomitant ather...

...llance of Thoracic Aortic Dilation and...

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

...n patients with a dilated thoracic aorta, a CT or...

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

...lance of Abdominal Aortic Dilation and Ane...

...ents with an AAA of 3.0 cm to 3.9 cm, surveillanc...

...men with an AAA of 4.0 cm to 4.9 cm a...

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

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

...atients, when there is a contraindicatio...

...ts with an AAA that meets criteria for repair, CT...

...gure 20. The Frequency of Surveillance...

...1. Surgery for Sporadic Aneurysms of the Aort...

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

...In asymptomatic patients with aneurysms of...

...ients with an aneurysm of the aortic...

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

In patients undergoing repair or replaceme...

...rgoing repair or replacement of a tricuspid...

...atients undergoing cardiac surgery for ind...

...with a height >1 standard deviation above or below...

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

....1. Surgical Approach for Patients With Sp...

...patients with an aneurysm isolated to the asc...

...ndergoing aortic valve repair or replacement wi...

...In patients undergoing aortic root re...

...ts undergoing aortic root replacement, valve-s...

6.5.2. Aortic Arch Aneurys...

...ith an aortic arch aneurysm who have symptoms...

...In patients with an isolated aortic arch a...

In patients undergoing open surgical repa...

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

...nts with an aortic arch aneurysm who...

...resholds for Repair of Descending TAA...

...ents with intact descending TAA, repair is recomme...

...ients with intact descending TAA and risk factors...

...s at increased risk for perioperat...

...Adverse Aortic Events at 1 Year, Based on...

...Risk Factors for Aortic Rupture Among Pat...

...k Features for Rupture Aneurysm...

.... Patient Characteristics Associated With In...

...cular Versus Open Repair of Descending TAA...

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

...In patients with a descending TAA tha...

In patients with a descending TAA that meets...

...t Subclavian Artery Managemen...

...with descending TAA who undergo TEVAR wi...

...In patients with descending TAA who have undergone...

...5.3.4. Celiac Artery Management...

...ts with descending TAA undergoing TEVAR in...

....3.5. Ruptured Descending TAA...

...In patients with ruptured descending...

...s with ruptured descending TAA und...

.... Access Issues for TEVAR in Descending TAA...

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

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

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

...Size Thresholds for Open Surgical...

...patients with intact degenerative...

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

...In patients with intact degenerative TAAA w...

...eatures Associated With an Increased R...

....2. Open Versus Endovascular Repair of TA...

...ptured TAAA...

...nts with ruptured TAAA requiring i...

In patients with ruptured TAAA requiring interv...

...act TAAA...

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

...n patients with intact degenerative TAAA and su...

...5.4.3. TAAA Spinal Cord...

...undergoing open TAAA repair who are at high risk...

...atients who experience delayed spinal cord dysf...

.... Measures to Optimize Spinal Cord and...

...enal and Visceral Organ Protection

...nts undergoing open repair of TAAA involving the...

...In patients undergoing open or endovas...

6.5.5.1. Access During Endovascular Re...

In patients undergoing endovascu...

...Repair of Ruptured A...

...tients presenting with ruptured AAA who are hemody...

...ients presenting with ruptured AAA who have...

...In patients undergoing endovascula...

...atients with ruptured AAA, permissive hypotensi...

...3. Threshold for AAA Re...

...tients with unruptured AAA, repair is recommended...

...In patients with unruptured AAA wh...

...ents with unruptured saccular AAA, intervention...

In patients with unruptured AAA and aneur...

...Versus Endovascular Repair of AAA...

...patients with nonruptured AAA with low t...

...ents undergoing elective endovascular repair for...

...ts with nonruptured AAA and a high perioper...

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

...5.5.5. Treatment of Concomitant Common Il...

...with asymptomatic small AAA and concomitan...

...treating common iliac artery aneurysms or e...

...1. Surveillance After TAA R...

...tients treated with TEVAR, surveillance imaging...

...In patients treated with TEVAR, longitudinal surve...

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

...treated with open repair of the thoracic ao...

...5.6.2. Surveillance After AAA Rep...

...In patients with AAA treated with EVAR, bas...

...with AAA treated with EVAR who are...

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

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

...nts with AAA who have undergone op...

...Abnormal Findings on Duplex Imaging Afte...


...Acute Aortic Syndromes...

...22. Signs and Symptoms of AASHaving trouble vi...

...Diagnostic Evaluation (Imaging, L...

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

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

...lain Chest X-Ray Findings Suggestive of A...

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

...rtic Dissection Detection Risk Scor...

...able 25. Aorta Simplified Score (AORTAs) Pretest...

....1. Acute Medical Management of AAS

...s presenting to the hospital with AAS, prompt tr...

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

...ent should include intravenous beta blockers,...

...e with contraindications or intoleran...

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

...with AAS should be treated with pain contr...

...re 21. Acute Aortic Dissection: Malpe...

....2. Subsequent Medical Management of AAS...

...In patients with AAS, it is recommended to treat...

...tial Surgical Considerations in Acute Type...

...In patients presenting with suspected or confirme...

...In patients presenting with acute t...

...nts presenting with non-hemorrhagic stroke...

....2. Management of Malperfusion...

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

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

.... Clinical Evidence of Malperfusion (â...

...4.1.3. Surgical Repair Strategies in Ac...

...epair Strategies...

...ith acute type A aortic dissection and a partiall...

...lacement is recommended with a mechanical or bi...

...selected patients who are stable, valve...

...nts with acute type A aortic disse...

In patients with acute type A a...

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

...ion and Cannulation Strategies...

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

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

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

.... Management of Acute Type B Aortic Dissec...

...nts with uncomplicated acute type B...

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

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

...atients with other complications, in the pres...

...atients with uncomplicated acute type...

.... Consensus Features of Complicated Acut...

...28. High-Risk Features in Uncomplicated...

High-Risk Imaging Fi...

...ic diameter >40 mm False-lumen diam...

...sk Clinical Findings...

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

....5. Management o...

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

...urgical repair is recommended. ( B-NR , I )706...

In selected patients with uncomplicated acute t...

...ents with uncomplicated acute type B IMH...

...with type B IMH who require repair o...

...s with type B IMH who require repair o...

...ents with uncomplicated type B IMH and high...

...able 29. Features of Complica...

...ature

...fusion Periaortic hematoma Perica...

...ble 30. High-Risk Imaging Features...

....1. PAU With IMH, Rupture, or Both

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

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

...atients with PAU of the aortic arch or des...

...ents with PAU of the abdominal aort...

...6.2. Isolated PAU

...nts with isolated PAU who are symptomatic and have...

...ith isolated PAU who are asymptoma...

...ble 31. High-Risk Imaging Featur...

...ature...

...Maximum PAU diameter ≥13–20 mm...

...nsions of Penetrating Atherosclerotic Ulcer...

...PAU Open Surgical Repair Versus Endovascular Re...

...ients who require repair of a PAU in the ascendin...

...ts who require repair of a PAU in the d...

...1. Initial Management of BTTAI in the Emergency De...

...In patients with BTTAI, management and...

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

...ach to the Initial Management of BTTA...

In patients with grade 1 BTTAI (Figur...

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

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

...In patients with grade 2 BTTAI (Fig...

.... Classification System for BTTAIsAo...

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

....7.1.3. Endovascular Versus Open Surgical R...

...ts with BTTAI who meet indications for repa...

...tial Management of Blunt Traumatic Abdom...

...atients with grade 1 to 2 BAAI (Table...

...ients with grade 4 BAAI (Table 33), re...

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

...with BAAI, treatment with either endovascular o...

...atients with grade 3 BAAI (Table 33), it ma...

...ents with BAAI, the usefulness of routine...

...e 33. Descriptions of Blunt Aortic...

...erm Management and Surveillance After Blun...

In patients with BTAI who have underg...

...In patients with BTAI who have not u...

...24. Abdominal Aortic Zones of Injury for...

....1. Long-Term Surveillance Imaging...

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

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

.... Long-Term Management After Acute Aortic Di...

...atients with a previous acute aortic dissection an...

...3. Long-Term Management and Surveillance for...

...ients with a PAU who have undergone aortic repa...

...atients with a PAU that is being m...


...in Patients With Aortopathy...

...unseling and Management of Aortic Disease in Preg...

...regnancy...

...with genetic aortopathies attributable to...

...nts with syndromic and nsHTAD, Turner syndrome, BA...

...In patients with syndromic and nsHTAD, T...

...g Pregnancy...

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

...tients with aortopathies who are pregn...

...ients with syndromic and nsHTAD, beta...

...In pregnant patients with an aortopathic condition...

In pregnant patients with aortic disease w...

...ry in Pregnant Patients With Aortopathy...

...In pregnant patients with a history of chr...

In pregnant patients with an aortopathy and...

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

...nant patients with a diameter of the aortic root...

...pregnant patients with syndromic and...

...efore Pregnancy in Women With Aort...

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

...c root diameter is 4.0 cm to 4.5 cm, aortic...

...FB3 and an aortic diameter of ≥4.5 cm, sur...

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

...d an aortic diameter of ≥4.5 cm,...

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

In patients with Turner syndrom...

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

...with sporadic aortic root aneurysms, ascendi...

...hylactic Aortic Surgery Before Pregnancy in Wome...

...Pregnancy in Patients With Aortopathy: Aort...

...ents experiencing an acute type A aortic diss...

...ts experiencing an acute type A aortic dis...

...tients experiencing an acute type B aortic d...

...ts with progressive aortic dilation during...


...Other Aortic Conditions ...

.... Inflammatory Aortitis: Diagnosis and...

Diagnos...

...n patients with large vessel vasculitis (L...

...reatme...

...ith active GCA or Takayasu arteritis, in...

...In patients with GCA who have evidence of active a...

...all patients with Takayasu arteritis...

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

...n patients with GCA or Takayasu art...

...n patients with GCA or Takayasu arteritis...

...Diagnostic Criteria for Inflammatory...

...nosis and Management of Infection of the Native A...

...al repair is recommended. ( C-EO , I )706...

...ents, treatment with endovascular repair may b...

...ith infectious aortitis complicated by rupt...

In patients with infectious aortitis...

...25. The 2018 European Alliance of Associati...

...2018 European Alliance of Associations for Rheuma...

...nagement of Aortic Mycotic Aneurysm: Com...

.... Diagnosis and Management of Prosthetic...

...iagnosis...

...with a prosthetic aortic graft, who hav...

...eatment

...patients with an infected prosthetic aorti...

...with an infected prosthetic aortic...

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

...te Management...

...n patients who have undergone treatment o...

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

...Atherosclerotic Disease

...ts with aortic atherosclerotic disease and conco...

...atients with aortic atherosclerotic disease and r...

...ents with aortic atheromas of a thickness ≥4...

...rctation of the Aorta...

...n patients with CoA, including those...

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

...patients with significant native or...

...nts with CoA, guideline-directed med...

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

Table 37. Criteria for Signi...

...of significant CoA is based on evi...

...vasive blood pressure difference of >2...

....1. Aberrant Subclavian Artery, Komme...

...patients discovered to have an ASCA in th...

...tients with Kommerell’s diverticu...

9.4.2.2. Aberrant Left Vertebral Artery Ori...

...ents with an aberrant left vertebral...

...re 27. Measurements of Kommerell’s Diverticulum...

....2.3. Bovine Arch (Common Innominate and Left Ca...

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

...gure 28. Normal and Bovine Aortic...


10. Physical Activity and Quality...

...tions for Physical Activity and Qual...

...or patients with significant aortic disease, educ...

...or patients who have undergone surgery for aor...

...atients with thoracic or abdominal aortic a...

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