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
...oduction...
...umbering of the following tables m...
...atomy, Abnormal Anatomy, and Definitions...
...igure 1. The Anatomy of the Aorta and...
...lified Diagram Depicting the Key H...
...re 3. Classification of Aortic Anatomi...
...eedom From Ascending Aortic Complic...
Figure 5. Relative Risk of Aortic Diss...
...lassification of Aortic Dissection Chron...
...igure 6. Acute Aortic SyndromesIn aortic d...
...assification of Acute Aortic DissectionTh...
Figure 8. Anatomic Reporting of Ao...
...ure 9. Mechanisms of Dynamic and Static Obstructi...
...ification of Thoracoabdominal Aortic An...
.... Classification of Endoleak TypesEndoleak...
Assessment
Assessment
.... Imaging and Measureme...
...ortic Imaging Techniques to Determine Pre...
...s with known or suspected aortic dis...
In patients with known or suspecte...
...In patients with known or suspected aortic d...
...In patients with known or suspected ao...
...In patients with known or suspect...
...able to measure the aorta from lead...
...ner-edge to inner-edge measurements may also b...
...e 4. Essential Elements of CT and MRI Ao...
Table 5. Diagnostic Performance of Aortic Imaging...
...rtic Imaging Techniques to Determine the Pr...
...3. Reformatted CT Image Orthogonal to the A...
...e 14. Observed Relationship Between Annual...
...15. Predicted Risk of Mortality Derived From...
Treatment
Treatment
...Multidisciplinary Aort...
...tions for Multidisciplinary Aortic Teams...
...or patients with acute aortic disease th...
...tients who are asymptomatic with extensive ao...
...red Decision-Making...
...0. Recommendations for Shared Decision...
...ents with aortic disease, shared decision-...
...atients with aortic disease who ar...
.... Aneurysm...
...commendations for Management of Aneurysm...
...use of TAAHTAD (see Table 7): syndromic...
...dromes and Conditions Attributable t...
.... Risk Factors for Familial TAD TA...
....1.2.1. HTAD: Genetic Testing and Screening o...
...ts with aortic root/ascending aortic aneurysms or...
...ts with aortic root/ascending aortic aneu...
...with an established pathogenic or likely pathoge...
...s with TAD who have a pathogenic/likely pathogeni...
...family with aortic root/ascending aortic an...
...tients with aortic root/ascending aortic aneurys...
In patients with acute type A aortic...
...e 17. Evaluation and Genetic Testing Protocol for...
...ical Considerations for Non-syndro...
...ptomatic patients with aneurysms of the...
...ymptomatic patients with aneurysms of t...
...ients with aneurysms of the aortic...
...9. Features Associated With an Increased Risk...
...Thoracic Aortic Aneurysms and No Id...
...ory of aortic dissection at an aortic diamet...
...ostic and Surveillance Aortic Imaging in Marfan...
...nosis and Surveillance Imaging...
...aortic diameters are stable, an annual surveill...
...ortic root, ascending aorta, or both are not adeq...
...In adults with Marfan syndrome, aft...
...maging After Aortic Root Replac...
...with Marfan syndrome who have undergone a...
...with Marfan syndrome who have undergone a...
...2.2. Medical Therapy in Marfan Syndrome...
...patients with Marfan syndrome, treatment...
...In patients with Marfan syndrome, the...
...2.3. Marfan Syndrome Interventions: Repla...
...ts with Marfan syndrome and an aortic root diam...
...ents with Marfan syndrome, an aort...
...patients with Marfan syndrome and...
...tients with Marfan syndrome and an aortic diamet...
...10. Features Associated With Increased Ri...
....2.4. Marfan Syndrome Intervention...
...with Marfan syndrome and a nondissected a...
....1.2.3.1. Imaging in Loeys-Dietz Sy...
...n patients with Loeys-Dietz syndrom...
...patients with Loeys-Dietz syndrome a...
...patients with Loeys-Dietz syndrome,...
...s with Loeys-Dietz syndrome without di...
...patients with Loeys-Dietz syndrome without dil...
.... Medical Therapy in Loeys-Dietz Syndrome...
...patients with Loeys-Dietz syndrome, treatmen...
.... Loeys-Dietz Syndrome Surgical Interventions: Rep...
...n patients with Loeys-Dietz syndrome and...
...tients with Loeys-Dietz syndrome attributable to...
...ical Thresholds for Prophylactic Aortic...
...r Syndrome: Diagnostic Testing, Surveillan...
...with Turner syndrome, TTE and cardiac MRI are...
...atients with Turner syndrome who are ≥15 years...
...ts with Turner syndrome without ri...
...In patients with Turner syndrome and an A...
...ents with Turner syndrome and risk fact...
...s risk factors for aortic dissection (Tabl...
...ithout risk factors for aortic diss...
...k Factors for Aortic Dissection in Pa...
...ested Aortic Monitoring Protocol for Gi...
...cal Thresholds for Prophylactic Aorti...
6.1.3. BAV Aorto...
...ents with a BAV, TTE is indicated to...
...ents with a BAV, CT or MRI of the thoraci...
...tients with a BAV and either HTAD or phenotypi...
...ts with a BAV and a dilated aortic root...
...In patients with a BAV, screening of all first-de...
...1. Routine Follow-Up of BAV Disease Aortop...
In patients with a BAV who have undergone pr...
...patients with a BAV and a diameter of the aortic...
...BAV Aortopathy Interventions: Replacement...
...atients with a BAV and a diameter of the aorti...
In patients with a BAV and a cross-...
...In patients with a BAV, a diameter of the aort...
...In patients with a BAV who are undergoing...
...patients with a BAV, a diameter of the aortic r...
Table 14. Risk Factors for Aortic Dis...
...AAA: Cause, Risk Factors, and Screening...
...who are ≥65 years of age who hav...
In men or women who are ≥65 y...
...who are ≥65 years of age who have ever smoked,...
...In men or wo...
...omatic men or women >75 years who have had a...
...ithm for Identifying Patients to Screen...
...able 15. Risk Factors for Abdominal Aortic An...
...Management in Sporadic TAA
...ts with TAA and an average systolic...
...ents with TAA, regardless of cause and i...
...tients with TAA, regardless of etio...
....1.2. Treatment of TAA With Stati...
...n patients with TAA and imaging or clinical evide...
...with TAA who have no evidence of athe...
...Smoking Cessation in TAA...
...n patients with TAA who smoke ciga...
...Antiplatelet Therapy in TAA...
...In patients with atherosclerotic TAA and conco...
....2.1. BP Management in AAA
...n patients with AAA and an average SBP of ≥13...
...4.2.2. Treatment of AAA With Statin...
...with AAA and evidence of aortic atherosclerosis...
...In patients with AAA but no evidenc...
...3. Smoking Cessation in AA...
...In patients with AAA who smoke cigarettes, s...
6.4.2.4. Antithrombotic Therapy in...
...n patients with AAA with concomitan...
...rveillance of Thoracic Aortic Dila...
...tients with a dilated thoracic aorta, a T...
...with a dilated thoracic aorta, a CT or...
...atients with a dilated thoracic aorta, f...
....2. Surveillance of Abdominal Aortic Di...
...atients with an AAA of 3.0 cm to 3.9...
In men with an AAA of 4.0 cm to...
...In men with an AAA of ≥5.0 cm and...
...rveillance CT is recommended. (...
In such patients, when there is a contraindicat...
...In patients with an AAA that meets criteria for re...
...20. The Frequency of Surveillance Ima...
...ery for Sporadic Aneurysms of the Aortic...
...In patients with aneurysms of the aortic root and...
...mptomatic patients with aneurysms of...
...ents with an aneurysm of the aorti...
...In asymptomatic patients with aneur...
...undergoing repair or replacement of a tricus...
...ndergoing repair or replacement of a tricuspid ao...
...undergoing cardiac surgery for indications othe...
...n patients with a height >1 standard deviati...
...ptomatic patients with aneurysms of...
...l Approach for Patients With Sporad...
...s with an aneurysm isolated to the ascendi...
...patients undergoing aortic valve repair or repl...
...undergoing aortic root replacement with...
...undergoing aortic root replacement,...
...rtic Arch Aneurysms...
...In patients with an aortic arch an...
...In patients with an isolated aortic arch aneurys...
...undergoing open surgical repair of a...
...ients undergoing open surgical repair of an aorti...
...ith an aortic arch aneurysm who ar...
...ze Thresholds for Repair of Descending TAA...
...with intact descending TAA, repair is recommended...
...patients with intact descending TAA a...
...ents at increased risk for perioperative m...
...Adverse Aortic Events at 1 Year, Based on Baseli...
.... Risk Factors for Aortic Rupture Among Patient...
...atures for Rupture Aneurysm growth o...
...Patient Characteristics Associated With Increas...
...ndovascular Versus Open Repair of Descendin...
...s without Marfan syndrome, Loeys-Diet...
...In patients with a descending TAA that...
...In patients with a descending TAA th...
.... Left Subclavian Artery Man...
...n patients with descending TAA who undergo...
...s with descending TAA who have underg...
....4. Celiac Artery Managemen...
...ts with descending TAA undergoing TEVAR i...
.... Ruptured Descending TAA...
...ts with ruptured descending TAA who are anatomi...
...tients with ruptured descending TAA un...
6.5.3.6. Access Issues for TEVAR in Descending TAA
...with descending TAA undergoing TE...
...n patients with descending TAA undergoing TEVA...
...patients with descending TAA under...
...5.4.1. Size Thresholds for Open Surgical Repai...
...with intact degenerative TAAA, repair is...
...ts with intact degenerative TAAA, repair is reas...
...atients with intact degenerative TA...
...res Associated With an Increased Risk...
....2. Open Versus Endovascular Repair o...
...ptured TAAA...
...In patients with ruptured TAAA requiring int...
...ents with ruptured TAAA requiring intervention, pr...
Intact TA...
...nts with Marfan syndrome, Loeys-Dietz syndr...
...In patients with intact degenerative...
...4.3. TAAA Spinal Cord Prote...
...ts undergoing open TAAA repair who are at high ri...
...patients who experience delayed spinal cord...
...asures to Optimize Spinal Cord and End-Organ Perf...
...AA Renal and Visceral Organ Protectio...
...undergoing open repair of TAAA involving the re...
...In patients undergoing open or endovascul...
...Access During Endovascular Repair of...
...ndergoing endovascular repair of AAA w...
...5.2. Repair of Ruptured...
In patients presenting with ruptured AAA who ar...
...In patients presenting with rupt...
...patients undergoing endovascular repa...
...tients with ruptured AAA, permissi...
....5.3. Threshold for AAA Repair...
...atients with unruptured AAA, repair is...
...n patients with unruptured AAA who have symptom...
...with unruptured saccular AAA, interventi...
...ts with unruptured AAA and aneurysm growth of ≥...
...ersus Endovascular Repair of AAA...
...with nonruptured AAA with low to moderate o...
...ients undergoing elective endovasc...
...s with nonruptured AAA and a high perioperative ri...
...s with nonruptured AAA, a moderate to high perio...
....5.5. Treatment of Concomitant Common...
...with asymptomatic small AAA and concomitant c...
...en treating common iliac artery aneurysm...
....5.6.1. Surveillance After TAA Repai...
...atients treated with TEVAR, surveill...
...patients treated with TEVAR, longitudinal su...
...s treated with open repair of the thora...
...In patients treated with open repair o...
.... Surveillance After AAA Repa...
In patients with AAA treated with EVA...
...nts with AAA treated with EVAR who...
...with AAA treated with EVAR and abnormal finding...
...atients with AAA treated with complex EVAR, a mo...
...with AAA who have undergone open repair, surveil...
...Abnormal Findings on Duplex Imaging After EVAR Th...
...cute Aortic Syndromes...
...ble 22. Signs and Symptoms of AASH...
...gnostic Evaluation (Imaging, Laboratory Testing)...
...tients with a suspected AAS, CT is recomme...
...n patients with a suspected AAS, TEE and MRI...
...Chest X-Ray Findings Suggestive of Aortic Dis...
...ic Dissection on Chest X-Ray Medias...
Table 24. Aortic Dissection Detection Risk...
...able 25. Aorta Simplified Score (A...
...e Medical Management of AAS...
...ients presenting to the hospital with AAS, prom...
...ith AAS should be treated to an SBP...
...l management should include intrav...
...contraindications or intolerance...
...In patients with AAS, initial manage...
...AAS should be treated with pain con...
...igure 21. Acute Aortic Dissection: Malp...
...equent Medical Management of AAS...
...with AAS, it is recommended to treat with lo...
...1. Initial Surgical Considerations in Acute...
...patients presenting with suspected or co...
...tients presenting with acute type A aortic...
...atients presenting with non-hemorrhagic stroke co...
...Management of Malperfusio...
...with acute type A aortic dissection pr...
...patients with acute type A aortic dissectio...
Table 26. Clinical Evidence of Malperfusio...
....1.3. Surgical Repair Strategies in...
...ic Repair Strategies...
In patients with acute type A aor...
...oot replacement is recommended with a mech...
...patients who are stable, valve-sparing root...
...In patients with acute type A aortic dissect...
...s with acute type A aortic dissection w...
...ts with acute type A aortic dissection an...
...n and Cannulation Strategies...
...In patients with acute type A aortic dissecti...
...s with acute type A aortic dissection...
...n patients with acute type A aortic dissectio...
...anagement of Acute Type B Aortic Dissec...
...tients with uncomplicated acute type B aortic diss...
...on is recommended. ( C-LD , I...
...patients with rupture, in the presence of su...
...h other complications, in the presence...
...In patients with uncomplicated acu...
...27. Consensus Features of Complicated Acute Type...
...High-Risk Features in Uncomplicated Ac...
...sk Imaging Findings...
...tic diameter >40 mm False-lumen diamet...
...isk Clinical Findings...
...tory hypertension despite >3 differ...
...anagement of IM...
...tients with complicated (Table 29) acut...
...pt open surgical repair is recommended. (...
...d patients with uncomplicated acute type A IMH...
...ents with uncomplicated acute type B I...
...In patients with type B IMH who require repair o...
...In patients with type B IMH who require repair...
...tients with uncomplicated type B IMH...
Table 29. Features of Com...
...ature...
...Periaortic hematoma Pericardial...
...able 30. High-Risk Imaging Features of IMHHa...
...With IMH, Rupture, or Both...
...In patients with PAU of the aorta wi...
In patients with PAU of the ascending ao...
...nts with PAU of the aortic arch or descending tho...
...s with PAU of the abdominal aorta with associated...
7.6.2. Isolated...
...patients with isolated PAU who are...
...nts with isolated PAU who are asymptoma...
...1. High-Risk Imaging Features...
Featur...
...U diameter ≥13–20 mm Maximum PAU d...
...mensions of Penetrating Atheroscleroti...
...Surgical Repair Versus Endovascular Repair...
...s who require repair of a PAU in the...
...nts who require repair of a PAU in the distal a...
...itial Management of BTTAI in the Emergency Depar...
...ients with BTTAI, management and tr...
...ients with BTTAI, anti-impulse therapy to reduce...
...h to the Initial Management of BTTAI...
...s with grade 1 BTTAI (Figure 23), n...
...In patients with grade 3 to 4 BTTAI...
...ts with grade 2 BTTAI (Figure 23) and with high-r...
In patients with grade 2 BTTAI (Figu...
.... Classification System for BTTAIsAor...
...Risk Imaging Features of BTTAI Post...
....1.3. Endovascular Versus Open Surgical...
In patients with BTTAI who meet i...
...tial Management of Blunt Traumatic Abdominal Aor...
...patients with grade 1 to 2 BAAI (Table 3...
...patients with grade 4 BAAI (Table 33), repair...
...with grade 2 BAAI (Table 33) and associated...
...In patients with BAAI, treatment wi...
...nts with grade 3 BAAI (Table 33), it may be...
...s with BAAI, the usefulness of routine...
...ble 33. Descriptions of Blunt Aortic Injury...
...Long-Term Management and Surveillance Aft...
...ts with BTAI who have undergone aortic repair, s...
...In patients with BTAI who have not underg...
...4. Abdominal Aortic Zones of Injury for Surg...
...g-Term Surveillance Imaging After Aortic Dissec...
...nts who have had an acute aortic dissection and...
...ts who have had an acute aortic dissection...
....2. Long-Term Management After Acute...
...ts with a previous acute aortic dissection and IMH...
...3. Long-Term Management and Surveillance f...
...patients with a PAU who have undergone aortic re...
...ients with a PAU that is being managed...
...y in Patients With Aortopathy...
...Counseling and Management of Aorti...
...regnancy...
...In patients with genetic aortopath...
...ients with syndromic and nsHTAD, Turner...
...with syndromic and nsHTAD, Turner syn...
...uring Pregnan...
In patients with aortic aneurysms, or...
...s with aortopathies who are pregnant, gu...
...In patients with syndromic and nsHTAD, beta-b...
...patients with an aortopathic condit...
...In pregnant patients with aortic disease who...
...ery in Pregnant Patients With Aortopathy...
...nt patients with a history of chronic aortic...
...pregnant patients with an aortopathy and a...
...patients with a diameter of the aortic root,...
...gnant patients with a diameter of the aorti...
...egnant patients with syndromic and nsHTAD...
...Before Pregnancy in Women With Aortic Disease...
...ic root diameter of >4.5 cm, aortic s...
...ortic root diameter is 4.0 cm to 4....
...TGFB3 and an aortic diameter of ≥4.5 cm, surge...
...1, TGFBR2, or SMAD3, and the aortic diameter is â‰...
...diameter of ≥4.5 cm, surgery before pregnanc...
...c diameter is 4.0 cm to 4.4 cm, surgery before pr...
...tients with Turner syndrome and ASI...
...patients with a BAV (in the absence of Turner...
...tients with sporadic aortic root ane...
...hylactic Aortic Surgery Before Pregnan...
...n Patients With Aortopathy: Aortic Dissection...
...ts experiencing an acute type A ao...
In patients experiencing an acute ty...
...n patients experiencing an acute type B...
...patients with progressive aortic dilati...
...Other Aortic Condi...
....1. Inflammatory Aortitis: Diagnosis an...
...iagnosis...
...with large vessel vasculitis (LVV), pro...
...atment...
...ith active GCA or Takayasu arteritis,...
...In patients with GCA who have evidence o...
...patients with Takayasu arteritis, non...
...atients with active GCA or Takayasu arteritis,...
...patients with GCA or Takayasu arterit...
...ents with GCA or Takayasu arteritis an...
.... Diagnostic Criteria for Inflammatory Aorti...
...s and Management of Infection of th...
...pair is recommended. ( C-EO , I )70...
...select patients, treatment with endovascu...
...atients with infectious aortitis complic...
...ith infectious aortitis, intraveno...
...018 European Alliance of Associations for Rheumat...
...2018 European Alliance of Associations for R...
...36. Management of Aortic Mycotic Aneurysm: Com...
...osis and Management of Prosthetic Aort...
Diagnos...
...ith a prosthetic aortic graft, who h...
...atment
...with an infected prosthetic aortic graft who...
...ith an infected prosthetic aortic gra...
...with an infected prosthetic aortic gra...
Late Manageme...
...ts who have undergone treatment of an acute...
...ents with an infected prosthetic aortic graft a...
...herosclerotic Disease
...patients with aortic atherosclerotic disease and c...
...ients with aortic atherosclerotic disease and...
...In patients with aortic atheromas of a thicknes...
...1. Coarctation of the Aorta
...ients with CoA, including those who have u...
...ients with CoA, BPs should be measured...
...n patients with significant native or recurrent Co...
...atients with CoA, guideline-directed medical...
...In adult patients with CoA, screening for intrac...
...37. Criteria for Significa...
...e presence of significant CoA is based on evide...
...vasive blood pressure difference of >20...
....4.2.1. Aberrant Subclavian Artery, Kommerell’...
...patients discovered to have an ASCA in the abs...
In patients with Kommerell’s div...
....2.2. Aberrant Left Vertebral Artery Ori...
...ients with an aberrant left vertebral...
...rements of Kommerell’s DiverticulumTwo diame...
....4.2.3. Bovine Arch (Common Innominate a...
...s with bovine arch (common innominat...
...re 28. Normal and Bovine Aortic Arch Conf...
...Physical Activity and Quality of Life...
...tions for Physical Activity and Quality of...
...s with significant aortic disease, educ...
...atients who have undergone surgery for a...
...In patients with thoracic or abdominal...
...ts with clinically significant aortic disease, it...