Diagnosis and Management of Aortic Disease
Overview
Overview
Top 10 Take-Home Messages for the Diagnosis and Management of Aortic Disease
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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
Introducti...
Note: The numbering of the following...
...al Anatomy, Abnormal Anatomy, and...
...atomy of the Aorta and Its Main Branches...
...2. A Simplified Diagram Depicting the Key Histolog...
...fication of Aortic Anatomic Segments by 11 Landi...
...igure 4. Freedom From Ascending Aortic...
...Relative Risk of Aortic Dissection by Si...
...ble 3. Classification of Aortic Dissec...
...cute Aortic SyndromesIn aortic dissection, a...
...ification of Acute Aortic Dissection...
...igure 8. Anatomic Reporting of Aortic Disse...
...Mechanisms of Dynamic and Static Obstruction in ...
...assification of Thoracoabdominal Ao...
...lassification of Endoleak TypesEndoleaks a...
Assessment
...essment...
...aging and Measurements...
...aging Techniques to Determine Presence and Pro...
...with known or suspected aortic disease, aor...
...with known or suspected aortic disease,...
...nts with known or suspected aortic dise...
...with known or suspected aortic disease, t...
In patients with known or suspected aorti...
it is reasonable to measure the aorta from l...
...e to inner-edge measurements may also be...
...able 4. Essential Elements of CT and MRI Aort...
Table 5. Diagnostic Performance of Aor...
...rtic Imaging Techniques to Determine theÂ...
...rmatted CT Image Orthogonal to the Aor...
...ure 14. Observed Relationship Between Annual I...
...re 15. Predicted Risk of Mortality Derived...
Treatment
...atment...
...ciplinary Aortic Teams...
...0. Recommendations for Multidiscipli...
...For patients with acute aortic disease...
...For patients who are asymptomatic with extens...
...red Decision-Making...
....0. Recommendations for Shared Decision...
...s with aortic disease, shared decision-makin...
...ients with aortic disease who are contemplati...
6. Aneury...
...gure 16. Recommendations for Management...
...se of TAAHTAD (see Table 7): syndr...
...ble 7. TAA Syndromes and Conditions Attr...
...Risk Factors for Familial TAD TAD...
...enetic Testing and Screening of Family Members f...
...ts with aortic root/ascending aortic aneurysms...
...In patients with aortic root/ascending aortic an...
...ents with an established pathogeni...
...ients with TAD who have a pathogenic/likely pat...
...ly with aortic root/ascending aortic...
...nts with aortic root/ascending aorti...
...s with acute type A aortic dissection, the di...
...17. Evaluation and Genetic Testing Protoco...
...ical Considerations for Non-syndromi...
...asymptomatic patients with aneurys...
...tomatic patients with aneurysms of the...
...nts with aneurysms of the aortic ro...
...able 9. Features Associated With an Increased Ri...
...Thoracic Aortic Aneurysms and No Identified Gen...
...ry of aortic dissection at an aortic diameter...
....1.2.2.1. Diagnostic and Surveillance Aortic I...
...agnosis and Surveillance Imagin...
if the aortic diameters are stable,...
...oot, ascending aorta, or both are not a...
In adults with Marfan syndrome, after...
...aging After Aortic Root Replace...
...patients with Marfan syndrome who h...
...ith Marfan syndrome who have undergone aortic root...
...2.2.2. Medical Therapy in Marfan Syndrom...
...with Marfan syndrome, treatment with either a be...
In patients with Marfan syndrome, the use...
6.1.2.2.3. Marfan Syndrome Interventions: Repla...
...n patients with Marfan syndrome and an aorti...
...In patients with Marfan syndrome, an aortic roo...
...In patients with Marfan syndrome an...
...patients with Marfan syndrome and an aortic...
...e 10. Features Associated With Inc...
...Marfan Syndrome Interventions: Repla...
...s with Marfan syndrome and a nondissected aneu...
...1. Imaging in Loeys-Dietz Syn...
...In patients with Loeys-Dietz syndrome, a...
...In patients with Loeys-Dietz syndrome and a dilate...
...ients with Loeys-Dietz syndrome, a baseline...
...patients with Loeys-Dietz syndrome without dila...
...patients with Loeys-Dietz syndrome withou...
....1.2.3.2. Medical Therapy in Loeys-D...
...patients with Loeys-Dietz syndrome, treatme...
...Loeys-Dietz Syndrome Surgical Int...
In patients with Loeys-Dietz syndrome and aor...
...In patients with Loeys-Dietz syndrom...
...urgical Thresholds for Prophylacti...
...rner Syndrome: Diagnostic Testing, Surv...
...ents with Turner syndrome, TTE and cardiac MRI...
...In patients with Turner syndrome...
...In patients with Turner syndrome without r...
...n patients with Turner syndrome and an ASI >2....
...tients with Turner syndrome and risk...
...factors for aortic dissection (Table 12), surgic...
...hout risk factors for aortic dissec...
...k Factors for Aortic Dissection in Patie...
...e 18. Suggested Aortic Monitoring Protoc...
...le 13. Surgical Thresholds for Prophylacti...
....3. BAV Aortopathy
...n patients with a BAV, TTE is indicated to eval...
...In patients with a BAV, CT or MRI of the th...
...patients with a BAV and either HTAD or...
...ients with a BAV and a dilated aortic root or asc...
...ents with a BAV, screening of all fir...
....1.3.1. Routine Follow-Up of BAV Disease A...
...In patients with a BAV who have undergone...
...nts with a BAV and a diameter of the aortic root,...
....2. BAV Aortopathy Interventions: Replaceme...
...atients with a BAV and a diameter of th...
...with a BAV and a cross-sectional aorti...
...patients with a BAV, a diameter of...
...patients with a BAV who are undergo...
...ith a BAV, a diameter of the aortic r...
...le 14. Risk Factors for Aortic Dissec...
...A: Cause, Risk Factors, and Screening...
...In men who are ≥65 years of age who have ever...
In men or women who are ≥65 years of age a...
...In women who are ≥65 years of age who have eve...
...In men or women...
...n asymptomatic men or women >75 years who have h...
...e 19. Algorithm for Identifying Patients to Scree...
...k Factors for Abdominal Aortic AneurysmHav...
.... BP Management in Sporadic TAA
...s with TAA and an average systolic BP (SBP) of ≥...
...ents with TAA, regardless of cause and in...
...patients with TAA, regardless of etiology and in...
...atment of TAA With Statins...
In patients with TAA and imaging or cli...
...patients with TAA who have no evidence of a...
...Smoking Cessation in TAA...
...with TAA who smoke cigarettes, smoking cessa...
...Antiplatelet Therapy in TAA...
...nts with atherosclerotic TAA and co...
....2.1. BP Management in AAA...
...In patients with AAA and an average...
6.4.2.2. Treatment of AAA Wit...
...s with AAA and evidence of aortic athero...
...with AAA but no evidence of atherosclerosi...
....2.3. Smoking Cessation in AA...
...tients with AAA who smoke cigarettes, smoki...
...ithrombotic Therapy in AAA
...ts with AAA with concomitant ather...
...veillance of Thoracic Aortic Dilation and Aneurys...
...In patients with a dilated thoracic a...
...In patients with a dilated thoracic aorta, a CT o...
...In patients with a dilated thoracic aorta, fol...
...eillance of Abdominal Aortic Dilatio...
...with an AAA of 3.0 cm to 3.9 cm, surveillance ul...
...n with an AAA of 4.0 cm to 4.9 cm and in women...
...an AAA of ≥5.0 cm and women with...
...eillance CT is recommended. ( C-EO ,...
...ients, when there is a contraindication...
...In patients with an AAA that meets criter...
...igure 20. The Frequency of Surveillance Ima...
6.5.1. Surgery for Sporadic Aneurysms of the...
...ents with aneurysms of the aortic root and...
...In asymptomatic patients with aneurysms of...
...s with an aneurysm of the aortic root...
...atic patients with aneurysms of the aort...
...atients undergoing repair or replacemen...
...undergoing repair or replacement o...
...ergoing cardiac surgery for indications ot...
...tients with a height >1 standard deviation abo...
...ic patients with aneurysms of the aortic root o...
....5.1.1. Surgical Approach for Patie...
...ts with an aneurysm isolated to the ascending...
...undergoing aortic valve repair or replaceme...
...In patients undergoing aortic root r...
...s undergoing aortic root replacement, val...
6.5.2. Aortic Arch Aneurysms
...with an aortic arch aneurysm who have symptoms at...
...ients with an isolated aortic arch aneur...
...nts undergoing open surgical repair of an a...
...undergoing open surgical repair of an aortic...
...ients with an aortic arch aneurysm who are asym...
...Thresholds for Repair of Descending TA...
...In patients with intact descendin...
...ts with intact descending TAA and...
...In patients at increased risk for periope...
...e 16. Adverse Aortic Events at 1 Year, Based on B...
...le 17. Risk Factors for Aortic Rupt...
...Risk Features for Rupture Aneurysm growth...
...e 18. Patient Characteristics Associated Wit...
...dovascular Versus Open Repair of Descendin...
In patients without Marfan syndrome, Lo...
...In patients with a descending TAA that meets c...
...ients with a descending TAA that meets c...
...Left Subclavian Artery Management...
...ts with descending TAA who undergo TEVAR...
...In patients with descending TAA wh...
....5.3.4. Celiac Artery M...
...ith descending TAA undergoing TEVAR in...
.... Ruptured Descending...
...patients with ruptured descending TAA who a...
...with ruptured descending TAA undergo...
...Access Issues for TEVAR in Descending TA...
...s with descending TAA undergoing TEVAR,...
...with descending TAA undergoing TEVAR, if iliac...
...s with descending TAA undergoing TEVAR who have...
....4.1. Size Thresholds for Open Surgica...
...ients with intact degenerative TAAA,...
...ts with intact degenerative TAAA, repair is...
...In patients with intact degenerat...
...es Associated With an Increased Risk of T...
...rsus Endovascular Repair of TAAA...
...ured TAAA
...ts with ruptured TAAA requiring inter...
...ith ruptured TAAA requiring intervention, p...
...tact TAAA...
...s with Marfan syndrome, Loeys-Dietz syndrome, or...
...patients with intact degenerative TAA...
.... TAAA Spinal Cord Protection...
...In patients undergoing open TAAA repair w...
...In patients who experience delayed spinal cord...
...ures to Optimize Spinal Cord and End-Organ Perfusi...
....4. TAAA Renal and Visceral Organ Protection...
...In patients undergoing open repair of TAAA i...
...undergoing open or endovascular TAAA re...
....5.5.1. Access During Endovascular Repai...
...nts undergoing endovascular repair of AAA who hav...
...Repair of Ruptured AAA...
...presenting with ruptured AAA who are hemodynam...
...In patients presenting with ruptured AAA who...
...nts undergoing endovascular repair for ruptu...
...ts with ruptured AAA, permissive h...
...Threshold for AAA Repair...
...ts with unruptured AAA, repair is recommended...
...ients with unruptured AAA who have...
...In patients with unruptured saccula...
...In patients with unruptured AAA and aneur...
...4. Open Versus Endovascular Repair o...
...In patients with nonruptured AAA with low to mode...
...In patients undergoing elective endova...
...tients with nonruptured AAA and a h...
...For patients with nonruptured AAA,...
...Treatment of Concomitant Common Iliac...
...or patients with asymptomatic smal...
...ng common iliac artery aneurysms or ectasia...
....1. Surveillance After TAA Repair...
...treated with TEVAR, surveillance imaging with CT...
...ients treated with TEVAR, longitudinal surveill...
...ts treated with open repair of the thoracic aorta...
...treated with open repair of the thoracic...
6.5.6.2. Surveillance After...
In patients with AAA treated with EVAR,...
...atients with AAA treated with EVAR...
...In patients with AAA treated with EVAR and abno...
...s with AAA treated with complex EVA...
...atients with AAA who have undergone open repai...
.... Abnormal Findings on Duplex Imagin...
...Aortic Syndromes...
...and Symptoms of AASHaving trouble view...
...AS: Diagnostic Evaluation (Imaging,...
...tients with a suspected AAS, CT is recommend...
...ients with a suspected AAS, TEE and MRI ar...
...e 23. Plain Chest X-Ray Findings Suggestive...
...f Aortic Dissection on Chest X-Ray...
...Aortic Dissection Detection Risk Score (ADD-RS...
...Aorta Simplified Score (AORTAs) Pret...
...te Medical Management of AAS...
...tients presenting to the hospital with...
...atients with AAS should be treated to...
...management should include intravenous...
...those with contraindications or into...
...s with AAS, initial management should include i...
...Patients with AAS should be treated with p...
...te Aortic Dissection: Malperfusion Treatment...
.... Subsequent Medical Management of...
...tients with AAS, it is recommended to tre...
...itial Surgical Considerations in A...
...patients presenting with suspected or confir...
...nts presenting with acute type A aortic dissect...
...In patients presenting with non-hemorrhagi...
....2. Management of Malperfusion...
...ients with acute type A aortic dissecti...
...tients with acute type A aortic dissection...
Table 26. Clinical Evidence of Malp...
....3. Surgical Repair Strategies in Acute Type A Ao...
...c Repair Strategies...
...tients with acute type A aortic diss...
...lacement is recommended with a mechanical or...
...lected patients who are stable, valve-s...
...In patients with acute type A aortic dissectio...
...ents with acute type A aortic dissection...
...In patients with acute type A aor...
...usion and Cannulation Strat...
In patients with acute type A aorti...
...ents with acute type A aortic dissection u...
...atients with acute type A aortic d...
...gement of Acute Type B Aortic Dissection...
...n all patients with uncomplicated acut...
...n is recommended. ( C-LD , I )706...
...with rupture, in the presence of suitable anatom...
...atients with other complications, in the...
...In patients with uncomplicated acute type B a...
...Consensus Features of Complicated Acute Type B A...
...-Risk Features in Uncomplicated Acute T...
...k Imaging Findings...
...aximal aortic diameter >40 mm False-lumen d...
...-Risk Clinical Findi...
...ctory hypertension despite >3 different classes o...
...Management of IMH...
...In patients with complicated (Table 29)...
...surgical repair is recommended. ( B-NR , I )706...
...ed patients with uncomplicated acute type A IMH w...
...In patients with uncomplicated acute typ...
...nts with type B IMH who require repair of the di...
...nts with type B IMH who require repair of the dis...
...ients with uncomplicated type B IM...
...29. Features of Complicated IMH...
Feature
...Malperfusion Periaortic hematom...
...isk Imaging Features of IMHHaving troubl...
...PAU With IMH, Rupture, or Both...
In patients with PAU of the aorta with rupture,...
...ts with PAU of the ascending aorta with...
...In patients with PAU of the aortic...
...In patients with PAU of the abdominal aor...
....6.2. Isolated PAU...
...patients with isolated PAU who are symptomatic...
...n patients with isolated PAU who are asympto...
...31. High-Risk Imaging Features of PAUs
...ature...
...ximum PAU diameter ≥13–20 mm Maximum PAU...
...22. Dimensions of Penetrating Atherosclerot...
...PAU Open Surgical Repair Versus Endovascul...
...ients who require repair of a PAU in t...
...In patients who require repair of a...
...ial Management of BTTAI in the Emergency De...
...ts with BTTAI, management and treatment...
...ts with BTTAI, anti-impulse therapy to r...
...7.1.2. Approach to the Initial Management of...
...In patients with grade 1 BTTAI (Figur...
...In patients with grade 3 to 4 BTTAI (Figure 23)...
...ith grade 2 BTTAI (Figure 23) and...
...with grade 2 BTTAI (Figure 23) and withou...
...23. Classification System for BTTAIsAortic...
...able 32. High-Risk Imaging Features of BT...
....1.3. Endovascular Versus Open Surgical Repair...
...atients with BTTAI who meet indications for repai...
...anagement of Blunt Traumatic Abdominal Aortic In...
...s with grade 1 to 2 BAAI (Table 33) without ma...
...patients with grade 4 BAAI (Table 33), repa...
...with grade 2 BAAI (Table 33) and associated...
...In patients with BAAI, treatment with...
...atients with grade 3 BAAI (Table 33),...
...n patients with BAAI, the usefulness...
.... Descriptions of Blunt Aortic Injury Gra...
...g-Term Management and Surveillance A...
...In patients with BTAI who have unde...
...with BTAI who have not undergone repair, surveill...
Figure 24. Abdominal Aortic Zones of...
...ng-Term Surveillance Imaging After Aortic Dissecti...
...atients who have had an acute aortic d...
...who have had an acute aortic dissectio...
....2. Long-Term Management After Acu...
...with a previous acute aortic dissection and I...
...ng-Term Management and Surveillance for PAUs...
...patients with a PAU who have undergone ao...
...atients with a PAU that is being m...
...Pregnancy in Patients With Aortopath...
...ing and Management of Aortic Diseas...
...pregnancy...
...with genetic aortopathies attribu...
...In patients with syndromic and nsHTAD, Turner synd...
...In patients with syndromic and nsHTAD...
...ring Pregna...
...ts with aortic aneurysms, or at in...
...tients with aortopathies who are pregnant...
...with syndromic and nsHTAD, beta-blocker therapy...
...egnant patients with an aortopathic...
...nant patients with aortic disease who req...
...Delivery in Pregnant Patients With...
...In pregnant patients with a history of...
...egnant patients with an aortopathy and an a...
...pregnant patients with a diameter of th...
...patients with a diameter of the aortic root...
...pregnant patients with syndromic and nsHTAD, and...
.... Surgery Before Pregnancy in Women With Aortic D...
...ortic root diameter of >4.5 cm, aortic surgery...
...e aortic root diameter is 4.0 cm to 4.5 c...
...and an aortic diameter of ≥4.5 cm, surgery befo...
...TGFBR2, or SMAD3, and the aortic diameter...
...ameter of ≥4.5 cm, surgery before pregnancy...
...c diameter is 4.0 cm to 4.4 cm, surger...
...with Turner syndrome and ASI of ≥2.5 cm/m2 ,...
...atients with a BAV (in the absence of Turner s...
...atients with sporadic aortic root a...
...Prophylactic Aortic Surgery Before Pregnancy...
...ancy in Patients With Aortopathy: Aorti...
...atients experiencing an acute type A aortic...
...atients experiencing an acute type A aortic dissec...
...patients experiencing an acute type B aor...
...ents with progressive aortic dilatio...
...Aortic Conditions ...
...flammatory Aortitis: Diagnosis and Treatment of...
...gnosis...
...tients with large vessel vasculitis (LVV), promp...
...reatmen...
...with active GCA or Takayasu arteritis, init...
...atients with GCA who have evidence of active a...
...ents with Takayasu arteritis, nonbiological di...
...ith active GCA or Takayasu arterit...
...ith GCA or Takayasu arteritis who are in r...
...patients with GCA or Takayasu arteritis and...
...stic Criteria for Inflammatory AortitisHavi...
...iagnosis and Management of Infectio...
...repair is recommended. ( C-EO , I...
...tients, treatment with endovascular repair...
...ith infectious aortitis complicated by rupt...
...patients with infectious aortitis, intravenous...
...5. The 2018 European Alliance of A...
...igure 26. The 2018 European Alliance of Associat...
...e 36. Management of Aortic Mycotic...
...Diagnosis and Management of Prosthetic Aor...
...iagnosis
...tients with a prosthetic aortic graft, wh...
...reatment...
...with an infected prosthetic aortic graft who ar...
...patients with an infected prosthetic aort...
...tients with an infected prosthetic aortic graft, e...
...e Management...
In patients who have undergone...
...ents with an infected prosthetic aorti...
...rosclerotic Disease...
In patients with aortic atheroscle...
...In patients with aortic atherosclerotic disease...
...tients with aortic atheromas of a thickne...
9.4.1. Coarctation of...
...ts with CoA, including those who have...
In patients with CoA, BPs should be measured i...
...ts with significant native or recurr...
...ents with CoA, guideline-directed m...
...dult patients with CoA, screening for intracran...
...ble 37. Criteria for Significan...
...ence of significant CoA is based on...
...sive blood pressure difference of >20 mm Hg be...
...1. Aberrant Subclavian Artery, Kommerellâ...
...In patients discovered to have an...
...ients with Kommerell’s diverticu...
...Aberrant Left Vertebral Artery Or...
...atients with an aberrant left vert...
...gure 27. Measurements of Kommerell’s Diver...
.... Bovine Arch (Common Innominate and Lef...
...nts with bovine arch (common innominate and left c...
...igure 28. Normal and Bovine Aortic Arch Con...
...cal Activity and Quality of Life...
...mendations for Physical Activity and Quality o...
...For patients with significant aor...
...For patients who have undergone sur...
...patients with thoracic or abdominal aortic aneur...
...nts with clinically significant aortic dise...