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
...troduction...
...ing of the following tables may diffe...
...tomy, Abnormal Anatomy, and Definitions...
...The Anatomy of the Aorta and Its Main Branches...
...ure 2. A Simplified Diagram Depicting the Key Hi...
...3. Classification of Aortic Anatomic Segment...
...re 4. Freedom From Ascending Aortic Com...
...5. Relative Risk of Aortic Dissecti...
...sification of Aortic Dissection Chronicity Bas...
...6. Acute Aortic SyndromesIn aortic dissection, a...
.... Classification of Acute Aortic DissectionThe DeB...
.... Anatomic Reporting of Aortic Dissection Based o...
...chanisms of Dynamic and Static Obstruction in Aor...
...ssification of Thoracoabdominal Aortic Aneurysm...
...ssification of Endoleak TypesEndoleaks are class...
Assessment
...sessment
...Imaging and Measurements...
...ortic Imaging Techniques to Determine Presenc...
...n patients with known or suspected aortic d...
...tients with known or suspected aor...
...ith known or suspected aortic disease...
...n patients with known or suspected aortic dise...
...ents with known or suspected aortic d...
...le to measure the aorta from leading-edge to le...
...er-edge to inner-edge measurements may...
...able 4. Essential Elements of CT an...
...e 5. Diagnostic Performance of Aortic Imag...
...c Imaging Techniques to Determine the...
...igure 13. Reformatted CT Image Orthogonal to the...
...igure 14. Observed Relationship Between A...
...5. Predicted Risk of Mortality Derived From theÂ...
Treatment
Treatmen...
...disciplinary Aortic Teams...
...commendations for Multidisciplinar...
...s with acute aortic disease that requires ur...
...ients who are asymptomatic with extensive aortic...
...d Decision-Making...
...Recommendations for Shared Decision-...
...patients with aortic disease, shared decision...
...patients with aortic disease who are contemplat...
...Aneurysms...
...Recommendations for Management of Aneurys...
...Cause of TAAHTAD (see Table 7): syndromic...
...dromes and Conditions Attributable to a Her...
...isk Factors for Familial TAD TAD and syn...
...1.2.1. HTAD: Genetic Testing and Screeni...
...s with aortic root/ascending aortic...
...In patients with aortic root/ascending a...
...ts with an established pathogenic o...
...with TAD who have a pathogenic/likel...
...n a family with aortic root/ascending aortic...
...In patients with aortic root/ascending aortic an...
...with acute type A aortic dissection,...
...re 17. Evaluation and Genetic Testing Pr...
...rgical Considerations for Non-syndromic H...
...omatic patients with aneurysms of the aortic...
...matic patients with aneurysms of the a...
...In patients with aneurysms of the aortic r...
...Features Associated With an Increased Risk of A...
...horacic Aortic Aneurysms and No Identified...
...ory of aortic dissection at an aortic diamete...
...Diagnostic and Surveillance Aortic Im...
Initial Diagnosis and Surveillance Imagi...
...aortic diameters are stable, an ann...
...oot, ascending aorta, or both are not adeq...
...lts with Marfan syndrome, after the initial TTE...
...r Aortic Root Replacement...
...nts with Marfan syndrome who have un...
...ents with Marfan syndrome who have underg...
.... Medical Therapy in Marfan Sy...
...In patients with Marfan syndrome, treat...
...atients with Marfan syndrome, the use...
....1.2.2.3. Marfan Syndrome Interventions: Replacem...
...n patients with Marfan syndrome and...
...In patients with Marfan syndrome, an aortic...
...patients with Marfan syndrome and a maximal cr...
...tients with Marfan syndrome and an ao...
...le 10. Features Associated With Increased Ris...
....2.4. Marfan Syndrome Interventions: Replaceme...
...nts with Marfan syndrome and a nondissected...
...Imaging in Loeys-Dietz Syndrome
...In patients with Loeys-Dietz syndrome, a...
...In patients with Loeys-Dietz syndrome and...
In patients with Loeys-Dietz syndrome, a b...
In patients with Loeys-Dietz syndrome...
...patients with Loeys-Dietz syndrome wi...
....2.3.2. Medical Therapy in Loeys-Dietz Syndrome...
...with Loeys-Dietz syndrome, treatm...
6.1.2.3.3. Loeys-Dietz Syndrome Surg...
...with Loeys-Dietz syndrome and aortic dil...
...ients with Loeys-Dietz syndrome attrib...
...Surgical Thresholds for Prophylactic Aortic Root...
...er Syndrome: Diagnostic Testing, Surveillanc...
...with Turner syndrome, TTE and cardiac MR...
...patients with Turner syndrome who are ≥...
...ts with Turner syndrome without risk...
...In patients with Turner syndrome and an ASI...
...with Turner syndrome and risk factors fo...
...risk factors for aortic dissection (Table 12), sur...
...e without risk factors for aortic disse...
...sk Factors for Aortic Dissection in Patie...
...gure 18. Suggested Aortic Monitoring Protocol for...
...urgical Thresholds for Prophylactic Aortic...
....1.3. BAV Aortop...
...n patients with a BAV, TTE is indicated to...
...ients with a BAV, CT or MRI of the tho...
...In patients with a BAV and either HTAD...
...ents with a BAV and a dilated aortic root or...
...ts with a BAV, screening of all first...
...outine Follow-Up of BAV Disease Aortop...
...n patients with a BAV who have undergone previous...
...n patients with a BAV and a diameter...
....1.3.2. BAV Aortopathy Interventions...
...patients with a BAV and a diameter of the...
...nts with a BAV and a cross-sectional ao...
...n patients with a BAV, a diameter of the aort...
...with a BAV who are undergoing surg...
...tients with a BAV, a diameter of t...
...sk Factors for Aortic Dissection F...
...se, Risk Factors, and Screening...
...e ≥65 years of age who have ever smoked, ultra...
...en or women who are ≥65 years of age and who...
...In women who are ≥65 years of age who hav...
...men or women...
...ptomatic men or women >75 years who have h...
...gure 19. Algorithm for Identifying Patients t...
...5. Risk Factors for Abdominal Aortic AneurysmH...
...Management in Sporadic TAA...
...ients with TAA and an average systolic BP (SBP...
...In patients with TAA, regardless of cause and in t...
...with TAA, regardless of etiology and in the ab...
.... Treatment of TAA With Statins...
...patients with TAA and imaging or clinical evid...
In patients with TAA who have no evidence of...
...3. Smoking Cessation in...
...ith TAA who smoke cigarettes, smoking cessation...
...1.4. Antiplatelet Therapy in TAA...
...ents with atherosclerotic TAA and concomitant aort...
...BP Management in AAA...
...In patients with AAA and an avera...
6.4.2.2. Treatment of AAA With...
...with AAA and evidence of aortic atheroscler...
...In patients with AAA but no evidence of atheroscl...
....4.2.3. Smoking Cessation in AAA
...tients with AAA who smoke cigarettes,...
....2.4. Antithrombotic Therapy in AAA...
...nts with AAA with concomitant atheroma and/or PAU...
...llance of Thoracic Aortic Dilation and Aneurysm...
...In patients with a dilated thoracic...
...ts with a dilated thoracic aorta, a CT or MRI at...
...nts with a dilated thoracic aorta, fol...
...eillance of Abdominal Aortic Dilation a...
...s with an AAA of 3.0 cm to 3.9 cm, surveillance ul...
...In men with an AAA of 4.0 cm to 4.9...
...an AAA of ≥5.0 cm and women with an AAA...
...is recommended. ( C-EO , I )706...
...uch patients, when there is a contra...
...atients with an AAA that meets criteria for repa...
...The Frequency of Surveillance Imaging of Abdo...
....1. Surgery for Sporadic Aneurysms of the Aort...
...ith aneurysms of the aortic root and...
...omatic patients with aneurysms of the aorti...
...n patients with an aneurysm of the aortic root...
...In asymptomatic patients with aneurysms of the ao...
...dergoing repair or replacement of a tric...
In patients undergoing repair or replacement of...
...s undergoing cardiac surgery for indications other...
...ients with a height >1 standard dev...
...ptomatic patients with aneurysms of the aortic ro...
....5.1.1. Surgical Approach for Patients With Sp...
...with an aneurysm isolated to the a...
...patients undergoing aortic valve re...
...ndergoing aortic root replacement with an aor...
...patients undergoing aortic root replacement...
...rtic Arch Aneurysms...
...with an aortic arch aneurysm who have sy...
...ts with an isolated aortic arch aneurysm who are...
...tients undergoing open surgical repair of an asce...
...ts undergoing open surgical repair of...
...In patients with an aortic arch aneurysm who a...
...hresholds for Repair of Descending TAA...
...patients with intact descending TA...
In patients with intact descending TAA and ris...
...patients at increased risk for perioperative m...
...Adverse Aortic Events at 1 Year, Based on Bas...
Table 17. Risk Factors for Aortic Rupture...
...eatures for Rupture Aneurysm growth of ≥0...
...atient Characteristics Associated With Incr...
.... Endovascular Versus Open Repair of Descending TA...
...nts without Marfan syndrome, Loeys-Diet...
...In patients with a descending TAA that...
...In patients with a descending TAA that meets...
.... Left Subclavian Artery Manage...
...with descending TAA who undergo TEVAR with...
...In patients with descending TAA who...
....5.3.4. Celiac Artery Man...
...ts with descending TAA undergoing...
....5. Ruptured Descending T...
...nts with ruptured descending TAA who are...
...ents with ruptured descending TAA undergoi...
...ess Issues for TEVAR in Descending T...
...In patients with descending TAA underg...
...nts with descending TAA undergoing TEVAR...
...patients with descending TAA unde...
...e Thresholds for Open Surgical Rep...
...atients with intact degenerative TAA...
...ents with intact degenerative TAAA, repair is r...
...In patients with intact degenerative TAAA...
...eatures Associated With an Increased R...
...pen Versus Endovascular Repair of TAAA...
...ured TAAA
...ients with ruptured TAAA requiring int...
...In patients with ruptured TAAA requir...
...tact TAAA...
...s with Marfan syndrome, Loeys-Dietz syndrom...
...In patients with intact degenerativ...
6.5.4.3. TAAA Spinal Cord...
...patients undergoing open TAAA repair who a...
...who experience delayed spinal cord dys...
...ble 20. Measures to Optimize Spinal Cord...
...A Renal and Visceral Organ Protection...
...patients undergoing open repair o...
...In patients undergoing open or endovas...
.... Access During Endovascular Repair...
...In patients undergoing endovascular repair of AAA...
...5.5.2. Repair of Ruptured...
...In patients presenting with ruptured AAA...
...In patients presenting with rupture...
...patients undergoing endovascular repair for ruptu...
...atients with ruptured AAA, permissive...
...3. Threshold for AAA Repa...
...In patients with unruptured AAA, repai...
...In patients with unruptured AAA who have sym...
...s with unruptured saccular AAA, interv...
...In patients with unruptured AAA and aneurysm gro...
...4. Open Versus Endovascular Repair of AAA...
...atients with nonruptured AAA with low t...
...ndergoing elective endovascular repair for...
...ients with nonruptured AAA and a high perioperativ...
...ients with nonruptured AAA, a moderate to high per...
...Treatment of Concomitant Common Iliac Aneu...
...ients with asymptomatic small AAA and concomit...
...When treating common iliac artery aneurysms or e...
6.5.6.1. Surveillance After TAA Rep...
In patients treated with TEVAR, surve...
...In patients treated with TEVAR, longitudin...
...nts treated with open repair of the thoracic a...
...atients treated with open repair of the tho...
....2. Surveillance After AAA Re...
...nts with AAA treated with EVAR, base...
...ents with AAA treated with EVAR who are undergoin...
...ts with AAA treated with EVAR and abnormal...
...tients with AAA treated with comple...
...tients with AAA who have undergone open repair, s...
...rmal Findings on Duplex Imaging After EVAR That...
...e Aortic Syndromes...
...22. Signs and Symptoms of AASHaving tr...
7.2. AAS: Diagnostic Evaluation (Ima...
...with a suspected AAS, CT is recommended...
...In patients with a suspected AAS, TEE and...
.... Plain Chest X-Ray Findings Suggestive of Aortic...
...gns of Aortic Dissection on Chest X-Ray Media...
.... Aortic Dissection Detection Risk Score (...
...able 25. Aorta Simplified Score (AORTAs) Pret...
...te Medical Management of AAS...
...resenting to the hospital with AAS, prom...
...Patients with AAS should be treated to an SBP...
...ment should include intravenous beta blockers...
...ith contraindications or intolerance to beta bl...
...ts with AAS, initial management should includ...
...Patients with AAS should be treated with p...
...Acute Aortic Dissection: Malperfusion Treatm...
...3.2. Subsequent Medical Managemen...
...with AAS, it is recommended to treat with long-...
....1.1. Initial Surgical Considerations in Acute Ty...
...patients presenting with suspecte...
...In patients presenting with acute type...
...ents presenting with non-hemorrhagic strok...
...nagement of Malperfusion
...In patients with acute type A aortic dissec...
...tients with acute type A aortic dissec...
...nical Evidence of Malperfusion (“Malpe...
7.4.1.3. Surgical Repair Strategies...
...Repair Strategies...
...In patients with acute type A aortic...
...acement is recommended with a mecha...
...ected patients who are stable, valve-sparing root...
...atients with acute type A aortic dissection...
...tients with acute type A aortic dissection withou...
...atients with acute type A aortic dissection a...
...usion and Cannulation Strategies...
...patients with acute type A aortic dissect...
...tients with acute type A aortic dissectio...
...nts with acute type A aortic dissection unde...
....2. Management of Acute Type B Aorti...
...In all patients with uncomplicated acute type B a...
...vention is recommended. ( C-LD ,...
...h rupture, in the presence of suitable ana...
...with other complications, in the presence of su...
...patients with uncomplicated acute type...
...sus Features of Complicated Acute T...
...28. High-Risk Features in Uncomplic...
...sk Imaging Finding...
...ortic diameter >40 mm False-lumen di...
...sk Clinical Findings...
...ry hypertension despite >3 different c...
...5. Management of IM...
...In patients with complicated (Table...
...open surgical repair is recommended. (...
...patients with uncomplicated acute type A IMH w...
...In patients with uncomplicated acute type...
...patients with type B IMH who require r...
...atients with type B IMH who require repa...
...In patients with uncomplicated type B...
...eatures of Complicated IMH...
...eature
...erfusion Periaortic hematoma Pe...
...h-Risk Imaging Features of IMHHaving trou...
....1. PAU With IMH, Rupture, or Bot...
...s with PAU of the aorta with rupture, urg...
...ts with PAU of the ascending aorta with...
...ents with PAU of the aortic arch or desc...
...s with PAU of the abdominal aorta with associa...
....2. Isolated PAU
...tients with isolated PAU who are sympto...
...In patients with isolated PAU wh...
...gh-Risk Imaging Features of PAUs...
...eatur...
...Maximum PAU diameter ≥13–20 mm Maximum PAU...
...Dimensions of Penetrating Atherosclerotic Ulcer...
...PAU Open Surgical Repair Versus Endovascular Repa...
...who require repair of a PAU in the ascendin...
...ho require repair of a PAU in the distal...
....7.1.1. Initial Management of BTTAI in the Eme...
...with BTTAI, management and treatment at a...
...In patients with BTTAI, anti-impulse...
...h to the Initial Management of BTTAI...
...s with grade 1 BTTAI (Figure 23), nonop...
...ents with grade 3 to 4 BTTAI (Figur...
...In patients with grade 2 BTTAI (Figure 23) a...
In patients with grade 2 BTTAI (Figure 23) and...
...lassification System for BTTAIsAortic inju...
...e 32. High-Risk Imaging Features of BTTAI...
...cular Versus Open Surgical Repair...
In patients with BTTAI who meet indicat...
...al Management of Blunt Traumatic Abdomi...
...s with grade 1 to 2 BAAI (Table 33) wi...
...atients with grade 4 BAAI (Table 33), repai...
...s with grade 2 BAAI (Table 33) and as...
...n patients with BAAI, treatment with...
...atients with grade 3 BAAI (Table 33), it may...
...In patients with BAAI, the usefulness...
...le 33. Descriptions of Blunt Aortic...
...Term Management and Surveillance After B...
...atients with BTAI who have undergone aortic repa...
...with BTAI who have not undergone repair, surv...
...Abdominal Aortic Zones of Injury fo...
...Long-Term Surveillance Imaging Af...
...patients who have had an acute aort...
...patients who have had an acute aortic dissecti...
...2. Long-Term Management After Acute Aortic D...
...ts with a previous acute aortic dis...
...erm Management and Surveillance for PAUs...
...atients with a PAU who have undergone aortic rep...
...patients with a PAU that is being managed me...
...cy in Patients With Aortopathy...
....1. Counseling and Management of Aortic Disease in...
...pregnancy...
...tients with genetic aortopathies attributa...
...patients with syndromic and nsHTAD, Turner s...
...ith syndromic and nsHTAD, Turner s...
...ng Pregnancy...
...ients with aortic aneurysms, or at increa...
...patients with aortopathies who ar...
...with syndromic and nsHTAD, beta-blocker therapy...
...ant patients with an aortopathic cond...
...nt patients with aortic disease who re...
...y in Pregnant Patients With Aortopathy...
...In pregnant patients with a history of chronic aor...
...patients with an aortopathy and an aor...
In pregnant patients with a diameter o...
...patients with a diameter of the aortic...
...pregnant patients with syndromic and ns...
...gery Before Pregnancy in Women With Aorti...
...ortic root diameter of >4.5 cm, aortic surgery...
If the aortic root diameter is 4.0...
...or TGFB3 and an aortic diameter of...
...2, or SMAD3, and the aortic diameter i...
...tic diameter of ≥4.5 cm, surgery...
If the aortic diameter is 4.0 cm to 4.4...
...nts with Turner syndrome and ASI of ≥2.5 cm/m2...
In patients with a BAV (in the absence...
...s with sporadic aortic root aneurysms, a...
.... Prophylactic Aortic Surgery Before Pregnancy i...
8.4. Pregnancy in Patients With Aortopathy: Aortic...
...In patients experiencing an acute type A aor...
...In patients experiencing an acute ty...
...In patients experiencing an acute type B ao...
...tients with progressive aortic dilation during...
...r Aortic ConditionsÂ
9.1. Inflammatory Aortitis: Diagnosis and...
...agnosis...
...In patients with large vessel vascul...
...eatmen...
...n patients with active GCA or Takayasu a...
...n patients with GCA who have eviden...
...ll patients with Takayasu arteritis, nonbiol...
...patients with active GCA or Takayasu arteriti...
...nts with GCA or Takayasu arteritis who...
In patients with GCA or Takayasu arteritis a...
...e 35. Diagnostic Criteria for Inflamma...
9.2.1. Diagnosis and Management of Infection...
...en surgical repair is recommended....
...tients, treatment with endovascular repair may be...
...patients with infectious aortitis complicat...
...atients with infectious aortitis, int...
...gure 25. The 2018 European Alliance of...
...018 European Alliance of Associations for Rheumat...
...e 36. Management of Aortic Mycotic Aneurys...
...2. Diagnosis and Management of Prosthe...
...gnosis...
...tients with a prosthetic aortic graft, who...
Treatmen...
...ith an infected prosthetic aortic graft...
...In patients with an infected prosthetic...
...with an infected prosthetic aortic gr...
...Management...
...In patients who have undergone tre...
...In patients with an infected prosthe...
...Atherosclerotic Disease...
...ith aortic atherosclerotic disease and concomitan...
...with aortic atherosclerotic disease...
...ith aortic atheromas of a thickness â‰...
.... Coarctation of the Aorta...
...ith CoA, including those who have un...
...In patients with CoA, BPs should be measured in...
In patients with significant native or...
...s with CoA, guideline-directed medica...
...t patients with CoA, screening for intracrani...
...Criteria for Significant C...
...sence of significant CoA is based on ev...
...vasive blood pressure difference of >20 m...
...rant Subclavian Artery, Kommerell’s Diverti...
...In patients discovered to have an ASCA in th...
...tients with Kommerell’s diverticulum, depending...
...2.2. Aberrant Left Vertebral Artery Ori...
...n patients with an aberrant left vertebral arter...
...7. Measurements of Kommerell’s Diverticu...
...ine Arch (Common Innominate and Left Carotid...
...In patients with bovine arch (common...
...re 28. Normal and Bovine Aortic Arc...
...l Activity and Quality of Life
...mmendations for Physical Activity and Quality of...
...r patients with significant aortic disease, educa...
...nts who have undergone surgery for...
...In patients with thoracic or abdominal...
...For patients with clinically significa...