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
Introductio...
...ering of the following tables may diff...
...ormal Anatomy, Abnormal Anatomy, and Defin...
...atomy of the Aorta and Its Main Branches...
...A Simplified Diagram Depicting the Key Histologic...
...lassification of Aortic Anatomic Segments b...
...4. Freedom From Ascending Aortic...
...igure 5. Relative Risk of Aortic Dissection by Si...
...le 3. Classification of Aortic Dissection...
...6. Acute Aortic SyndromesIn aortic dissection,...
...e 7. Classification of Acute Aortic DissectionTh...
...8. Anatomic Reporting of Aortic Dissect...
...Mechanisms of Dynamic and Static...
Figure 10. Classification of Thoracoa...
Figure 11. Classification of Endol...
Assessment
...ssessment
...Imaging and Measureme...
...Aortic Imaging Techniques to Determine P...
...ith known or suspected aortic disease, aor...
...patients with known or suspected aortic diseas...
...patients with known or suspected aort...
...n patients with known or suspected...
...tients with known or suspected aort...
...ble to measure the aorta from leading-edge...
...sing inner-edge to inner-edge measurement...
...ial Elements of CT and MRI Aortic Imaging Reports...
...agnostic Performance of Aortic Imaging ModalitiesH...
...ure 12. Aortic Imaging Techniques to D...
Figure 13. Reformatted CT Image Orthogonal to th...
Figure 14. Observed Relationship Between A...
...Predicted Risk of Mortality Derived Fr...
Treatment
...atment...
...tidisciplinary Aortic T...
.... Recommendations for Multidisciplinary...
...ents with acute aortic disease that require...
...For patients who are asymptomatic with exten...
...hared Decision-Ma...
...Recommendations for Shared Decision-Maki...
...n patients with aortic disease, shared deci...
...atients with aortic disease who are con...
6. Aneury...
.... Recommendations for Management of...
...e of TAAHTAD (see Table 7): syndromic...
...ble 7. TAA Syndromes and Conditions Attributa...
...Risk Factors for Familial TAD TAD and syndrom...
...HTAD: Genetic Testing and Screening of Fami...
...s with aortic root/ascending aortic aneurysms or a...
...patients with aortic root/ascending aortic a...
...with an established pathogenic or likely pat...
...tients with TAD who have a pathogeni...
...amily with aortic root/ascending aortic an...
...In patients with aortic root/ascending aort...
...ients with acute type A aortic dissection, the...
...valuation and Genetic Testing Protocol for...
...rgical Considerations for Non-syndromic Herita...
...In asymptomatic patients with aneu...
...asymptomatic patients with aneurysms of t...
...tients with aneurysms of the aortic root or ascend...
...es Associated With an Increased Ri...
...cic Aortic Aneurysms and No Identified Genet...
...tory of aortic dissection at an aorti...
...1. Diagnostic and Surveillance Aortic Imagi...
...nosis and Surveillance Imagi...
...diameters are stable, an annual sur...
...tic root, ascending aorta, or both are not adequa...
...adults with Marfan syndrome, after the i...
...r Aortic Root Replacement...
...nts with Marfan syndrome who have undergon...
...patients with Marfan syndrome who have unde...
...2. Medical Therapy in Marfan S...
...n patients with Marfan syndrome, treatment...
...ents with Marfan syndrome, the use...
...2.2.3. Marfan Syndrome Interventions: Replac...
...atients with Marfan syndrome and an aortic ro...
...ts with Marfan syndrome, an aortic...
...n patients with Marfan syndrome and a maxim...
...patients with Marfan syndrome and an ao...
...es Associated With Increased Risk of Aor...
...arfan Syndrome Interventions: Replacement of Prim...
...tients with Marfan syndrome and a n...
....1. Imaging in Loeys-Dietz Syndrome
...In patients with Loeys-Dietz synd...
...nts with Loeys-Dietz syndrome and a dilat...
...ith Loeys-Dietz syndrome, a baseline...
...with Loeys-Dietz syndrome without dilation of the...
...ts with Loeys-Dietz syndrome without dilat...
...2.3.2. Medical Therapy in Loeys-Dietz Syndrome...
...In patients with Loeys-Dietz syndrome, treatmen...
...Loeys-Dietz Syndrome Surgical Int...
...atients with Loeys-Dietz syndrome a...
...s with Loeys-Dietz syndrome attributable t...
.... Surgical Thresholds for Prophylactic Aortic R...
...1.2.5. Turner Syndrome: Diagnostic Testing, Su...
...ents with Turner syndrome, TTE and cardiac...
...with Turner syndrome who are ≥15...
In patients with Turner syndrome wi...
...s with Turner syndrome and an ASI >2.3 cm/m2,...
...patients with Turner syndrome and risk fac...
...factors for aortic dissection (Table 12), s...
...thout risk factors for aortic dissection, surgical...
...Risk Factors for Aortic Dissection in Patients...
...8. Suggested Aortic Monitoring Proto...
...gical Thresholds for Prophylactic...
...3. BAV Aortopathy
...with a BAV, TTE is indicated to evaluate valve...
...nts with a BAV, CT or MRI of the thora...
...nts with a BAV and either HTAD or phenotypic f...
...patients with a BAV and a dilated aorti...
...ients with a BAV, screening of all first-...
...ne Follow-Up of BAV Disease Aortopathy...
...s with a BAV who have undergone previous...
In patients with a BAV and a diameter of the...
...BAV Aortopathy Interventions: Replacement of...
...ents with a BAV and a diameter of the aor...
...patients with a BAV and a cross-sectional a...
...atients with a BAV, a diameter of the a...
...patients with a BAV who are undergoing su...
...patients with a BAV, a diameter of the aorti...
...4. Risk Factors for Aortic Dissection Family...
...AA: Cause, Risk Factors, and Screenin...
...n men who are ≥65 years of age who have ever...
...omen who are ≥65 years of age and who are firs...
...who are ≥65 years of age who have ever smoke...
In men or wom...
...n asymptomatic men or women >75 years who have...
...9. Algorithm for Identifying Patients to Sc...
...actors for Abdominal Aortic AneurysmH...
6.4.1.1. BP Management in Sporad...
...In patients with TAA and an average systoli...
...patients with TAA, regardless of cause and in the...
...patients with TAA, regardless of etiol...
....2. Treatment of TAA With St...
...with TAA and imaging or clinical ev...
...In patients with TAA who have no...
....3. Smoking Cessation in TAA...
...In patients with TAA who smoke cigarette...
...Antiplatelet Therapy in TAA...
...with atherosclerotic TAA and concomitant a...
...4.2.1. BP Management in...
...ients with AAA and an average SBP...
...2. Treatment of AAA With St...
...ents with AAA and evidence of aort...
...atients with AAA but no evidence of ather...
...4.2.3. Smoking Cessation...
In patients with AAA who smoke cigarettes, smok...
...4.2.4. Antithrombotic Therapy in...
...patients with AAA with concomitant...
...llance of Thoracic Aortic Dilation and Ane...
...atients with a dilated thoracic aorta, a TT...
...with a dilated thoracic aorta, a CT or MRI...
...s with a dilated thoracic aorta, fol...
...illance of Abdominal Aortic Dilation and Aneurys...
...In patients with an AAA of 3.0 cm to 3.9 cm...
...ith an AAA of 4.0 cm to 4.9 cm and in...
...an AAA of ≥5.0 cm and women with an AAA of ≥...
...urveillance CT is recommended. ( C-EO , I...
In such patients, when there is a contraindi...
...with an AAA that meets criteria for repair, CT...
Figure 20. The Frequency of Survei...
...urgery for Sporadic Aneurysms of the Aortic Root...
...n patients with aneurysms of the aortic ro...
...In asymptomatic patients with aneurysms of the...
...ents with an aneurysm of the aortic root or asce...
...omatic patients with aneurysms of the aortic r...
...tients undergoing repair or replacement of a t...
...ents undergoing repair or replacement of a...
...patients undergoing cardiac surgery for...
...s with a height >1 standard deviation above or...
...ic patients with aneurysms of the aor...
...cal Approach for Patients With Sporadi...
...tients with an aneurysm isolated to the a...
...In patients undergoing aortic valve repa...
...atients undergoing aortic root replacemen...
...nts undergoing aortic root replacement,...
...Aortic Arch Aneurysms...
...ents with an aortic arch aneurysm who have sy...
...with an isolated aortic arch aneurysm who...
...In patients undergoing open surgical repa...
...patients undergoing open surgical repair of...
...s with an aortic arch aneurysm who are as...
...1. Size Thresholds for Repair of Descending TAA...
...n patients with intact descending TAA,...
...ts with intact descending TAA and risk fact...
In patients at increased risk for perioper...
...le 16. Adverse Aortic Events at 1 Year,...
...7. Risk Factors for Aortic Rupture Am...
...-Risk Features for Rupture Aneurysm...
...atient Characteristics Associated With...
...Endovascular Versus Open Repair o...
...In patients without Marfan syndrome, Loeys-...
...atients with a descending TAA that meets criteria...
...tients with a descending TAA that...
.... Left Subclavian Artery Management
...with descending TAA who undergo TEV...
In patients with descending TAA...
....3.4. Celiac Artery Manag...
...patients with descending TAA underg...
...5.3.5. Ruptured Descendin...
...ts with ruptured descending TAA who are a...
...In patients with ruptured descending...
...s Issues for TEVAR in Descending TAA...
...ith descending TAA undergoing TEVAR, review of...
In patients with descending TAA und...
...ients with descending TAA undergoing TEVAR wh...
....4.1. Size Thresholds for Open Surgical R...
...In patients with intact degenerative...
...In patients with intact degenerati...
...ts with intact degenerative TAAA who ha...
...able 19. Features Associated With an Increased Ri...
...ersus Endovascular Repair of TAAA...
...ptured TAA...
...ith ruptured TAAA requiring intervention...
...tients with ruptured TAAA requiring int...
Intact TAA...
...In patients with Marfan syndrome, Loe...
...ents with intact degenerative TAAA and sui...
...AAA Spinal Cord Protectio...
...tients undergoing open TAAA repair who ar...
...ts who experience delayed spinal cord dysfunctio...
...easures to Optimize Spinal Cord and...
...Renal and Visceral Organ Protection...
...atients undergoing open repair of...
...n patients undergoing open or endovascula...
...5.1. Access During Endovascular Repair of AA...
...In patients undergoing endovascula...
....5.2. Repair of Ruptu...
...tients presenting with ruptured AAA who are hem...
...n patients presenting with ruptured AA...
...nts undergoing endovascular repair...
...atients with ruptured AAA, permissive hy...
...reshold for AAA Repair...
...In patients with unruptured AAA, re...
...atients with unruptured AAA who hav...
...nts with unruptured saccular AAA, intervent...
...ts with unruptured AAA and aneurys...
....5.4. Open Versus Endovascular Repai...
...ients with nonruptured AAA with low to moder...
...In patients undergoing elective endovascular...
...ith nonruptured AAA and a high perioperativ...
...s with nonruptured AAA, a moderate...
...reatment of Concomitant Common Iliac...
...ts with asymptomatic small AAA and concomitan...
...g common iliac artery aneurysms or ectasia as pa...
...veillance After TAA Repair...
...tients treated with TEVAR, surveill...
...n patients treated with TEVAR, longitudin...
...tients treated with open repair of the thoracic...
...treated with open repair of the thoracic a...
...urveillance After AAA Repair...
...In patients with AAA treated with EVAR, baselin...
...ts with AAA treated with EVAR who are unde...
...with AAA treated with EVAR and abnor...
...s with AAA treated with complex EVAR,...
In patients with AAA who have under...
Table 21. Abnormal Findings on Duplex Im...
...ute Aortic Syndromes
...s and Symptoms of AASHaving trouble vi...
...AAS: Diagnostic Evaluation (Imaging, Laborator...
...In patients with a suspected AAS, CT is re...
...In patients with a suspected AAS, TEE...
...le 23. Plain Chest X-Ray Findings Suggestive of Ao...
...gns of Aortic Dissection on Chest X-Ray Media...
...ble 24. Aortic Dissection Detection Risk S...
...25. Aorta Simplified Score (AORTAs) Pretest...
...e Medical Management of AAS...
...tients presenting to the hospital with AAS, promp...
...h AAS should be treated to an SBP...
...l management should include intravenous beta bl...
...e with contraindications or intolerance to bet...
...In patients with AAS, initial manageme...
...h AAS should be treated with pain c...
...Acute Aortic Dissection: Malperfusion Tr...
...quent Medical Management of AAS...
...ith AAS, it is recommended to treat with lo...
...Initial Surgical Considerations in Acute Type A A...
...atients presenting with suspected or co...
...presenting with acute type A aortic...
...In patients presenting with non-hemorrhagic st...
7.4.1.2. Management of Malperfusio...
...patients with acute type A aortic dissection pr...
...In patients with acute type A aortic dissec...
...26. Clinical Evidence of Malperfusi...
...urgical Repair Strategies in Acute...
...ic Repair Strategie...
...with acute type A aortic dissection and a partial...
...rtic root replacement is recommended wit...
...ents who are stable, valve-sparing root repair may...
In patients with acute type A aortic d...
...ents with acute type A aortic diss...
...with acute type A aortic dissection...
Perfusion and Cannulation Strat...
...nts with acute type A aortic dissection undergoing...
...tients with acute type A aortic dissection under...
...n patients with acute type A aortic dissection...
...2. Management of Acute Type B Aortic Dis...
...ts with uncomplicated acute type B aort...
intervention is recommended. ( C-LD , I )70...
...ts with rupture, in the presence of suitable an...
...nts with other complications, in the presence...
...n patients with uncomplicated acute typ...
Table 27. Consensus Features of Complicated A...
...e 28. High-Risk Features in Uncomplic...
...k Imaging Findings...
...imal aortic diameter >40 mm False-lumen diamet...
...isk Clinical Finding...
...pertension despite >3 different classes of anti...
....5. Management of...
...In patients with complicated (Table 29...
...n surgical repair is recommended....
...lected patients with uncomplicated acute type A...
...tients with uncomplicated acute type B IMH,...
...n patients with type B IMH who require re...
...In patients with type B IMH who re...
...nts with uncomplicated type B IMH an...
...Features of Complicated I...
Featur...
...Malperfusion Periaortic hematoma Per...
Table 30. High-Risk Imaging Featur...
...ith IMH, Rupture, or Both...
...patients with PAU of the aorta with r...
...s with PAU of the ascending aorta wi...
...ients with PAU of the aortic arch o...
...patients with PAU of the abdominal aorta w...
.... Isolated PAU...
...patients with isolated PAU who are sympto...
...ients with isolated PAU who are asympto...
...1. High-Risk Imaging Features of PAUs
Feature
...U diameter ≥13–20 mm Maximum PAU...
...22. Dimensions of Penetrating Atheroscleroti...
...n Surgical Repair Versus Endovascula...
...atients who require repair of a PAU in the asce...
...patients who require repair of a PAU in the d...
....1. Initial Management of BTTAI in th...
...patients with BTTAI, management and treatment at...
...n patients with BTTAI, anti-impulse therapy to re...
...Approach to the Initial Management of BTTAI...
...with grade 1 BTTAI (Figure 23), no...
...atients with grade 3 to 4 BTTAI (Figur...
...ents with grade 2 BTTAI (Figure 23)...
...s with grade 2 BTTAI (Figure 23) an...
...23. Classification System for BTTAIsAortic in...
...2. High-Risk Imaging Features of B...
...dovascular Versus Open Surgical R...
...n patients with BTTAI who meet indicatio...
...ial Management of Blunt Traumatic Abdo...
...with grade 1 to 2 BAAI (Table 33) without malpe...
...atients with grade 4 BAAI (Table 3...
...tients with grade 2 BAAI (Table 33)...
...atients with BAAI, treatment with either endovasc...
...In patients with grade 3 BAAI (Table 33), it may...
...tients with BAAI, the usefulness of r...
...le 33. Descriptions of Blunt Aortic Injury Grade...
...3. Long-Term Management and Surveillance After...
...ts with BTAI who have undergone ao...
...n patients with BTAI who have not undergone...
...minal Aortic Zones of Injury for Su...
...Surveillance Imaging After Aortic Dis...
...nts who have had an acute aortic dissection...
...In patients who have had an acute aortic dissec...
7.8.2. Long-Term Management After Acute Aortic Dis...
...with a previous acute aortic dissec...
.... Long-Term Management and Surveillance...
...nts with a PAU who have undergone...
...with a PAU that is being managed medic...
...ancy in Patients With Aortopathy...
...and Management of Aortic Disease in...
Prepregn...
...In patients with genetic aortopathies at...
In patients with syndromic and nsHTAD, Tu...
...ts with syndromic and nsHTAD, Turner syn...
...ring Pregnancy...
...ts with aortic aneurysms, or at in...
...with aortopathies who are pregnant...
...In patients with syndromic and nsHTAD,...
...pregnant patients with an aortopathic condition...
...egnant patients with aortic disease who require s...
...ry in Pregnant Patients With Aortopathy
...egnant patients with a history of chronic aortic d...
...atients with an aortopathy and an a...
...In pregnant patients with a diameter o...
...atients with a diameter of the aortic root, as...
In pregnant patients with syndromic and ns...
...ore Pregnancy in Women With Aortic Di...
...n aortic root diameter of >4.5 cm, aortic sur...
...he aortic root diameter is 4.0 cm to 4.5...
...TGFB3 and an aortic diameter of ≥...
..., TGFBR2, or SMAD3, and the aortic diameter i...
...iameter of ≥4.5 cm, surgery before...
...tic diameter is 4.0 cm to 4.4 cm, su...
...patients with Turner syndrome and ASI of ≥2.5 cm...
...ients with a BAV (in the absence of Tur...
...ients with sporadic aortic root aneurysms, ascen...
...phylactic Aortic Surgery Before Pregnancy...
8.4. Pregnancy in Patients With Ao...
...In patients experiencing an acute type A aorti...
...ents experiencing an acute type A aortic dissec...
...patients experiencing an acute type B aortic...
...tients with progressive aortic dilat...
...Other Aortic Condit...
...ory Aortitis: Diagnosis and Treatment...
...gnosis...
...ith large vessel vasculitis (LVV),...
...reatment
...n patients with active GCA or Takayasu arteri...
In patients with GCA who have evidence...
...n all patients with Takayasu arteritis,...
...with active GCA or Takayasu arteritis, t...
...n patients with GCA or Takayasu arteri...
...In patients with GCA or Takayasu arteritis a...
...nostic Criteria for Inflammatory AortitisHaving tr...
...osis and Management of Infection of the...
...pair is recommended. ( C-EO , I )706...
...patients, treatment with endovascular repair may...
...ts with infectious aortitis complicated by r...
...atients with infectious aortitis, intraven...
...25. The 2018 European Alliance of Associatio...
...26. The 2018 European Alliance of...
...anagement of Aortic Mycotic Aneurysm: C...
...osis and Management of Prosthetic A...
...gnosis...
...nts with a prosthetic aortic graft, who have sig...
Treatment
In patients with an infected prosthetic aort...
...ts with an infected prosthetic aortic graft who...
...In patients with an infected prosthetic aorti...
Late Manag...
...patients who have undergone treatment of an acut...
...patients with an infected prosthetic aortic gra...
9.3. Atherosclerotic Disea...
...patients with aortic atherosclerotic diseas...
...tients with aortic atherosclerotic disease an...
...patients with aortic atheromas of a...
...4.1. Coarctation of the Aorta...
...n patients with CoA, including those who have...
...ts with CoA, BPs should be measure...
...In patients with significant native or recu...
...with CoA, guideline-directed medical therapy is re...
In adult patients with CoA, screening for...
...le 37. Criteria for Significant CoA...
...resence of significant CoA is based o...
...A noninvasive blood pressure difference of >20...
...berrant Subclavian Artery, Kommerell’s Divertic...
...ts discovered to have an ASCA in the absence...
...with Kommerell’s diverticulum, depending on...
....4.2.2. Aberrant Left Vertebral Arter...
...In patients with an aberrant left...
...igure 27. Measurements of Kommerell’s...
....2.3. Bovine Arch (Common Innominate and Left Car...
...In patients with bovine arch (common innom...
...igure 28. Normal and Bovine Aortic Arch Conf...
.... Physical Activity and Quality o...
...mmendations for Physical Activity and...
...with significant aortic disease, education and gu...
...who have undergone surgery for aortic aneury...
...nts with thoracic or abdominal aorti...
...atients with clinically significant aortic...