Diagnosis and Management of Aortic Disease

Publication Date: November 2, 2022

Overview

Overview

Top 10 Take-Home Messages for the Diagnosis and Management of Aortic Disease

  1. Because outcomes for patients with aortic disease are enhanced at programs with higher volumes, experienced practitioners, and extensive management capabilities, Multidisciplinary Aortic Team care is considered in determining the appropriate timing of intervention.
  2. Shared decision-making involving the patient and a multidisciplinary team is highly encouraged to determine the optimal medical, endovascular, and open surgical therapies. In patients with aortic disease who are contemplating pregnancy or who are pregnant, shared decision-making is especially important when considering the cardiovascular risks of pregnancy, the diameter thresholds for prophylactic aortic surgery, and the mode of delivery.
  3. Computed tomography, magnetic resonance imaging, and echocardiographic imaging of patients with aortic disease should follow recommended approaches for image acquisition, measurement and reporting of relevant aortic dimensions, and the frequency of surveillance before and after intervention.
  4. At centers with Multidisciplinary Aortic Teams and experienced surgeons, the threshold for surgical intervention for sporadic aortic root and ascending aortic aneurysms has been lowered from 5.5 cm to 5.0 cm in selected patients, and even lower in specific scenarios among patients with heritable thoracic aortic aneurysms.
  5. In patients who are significantly smaller or taller than average, surgical thresholds may incorporate indexing of the aortic root or ascending aortic diameter to either patient body surface area or height, or aortic cross-sectional area to patient height.
  6. Rapid aortic root growth or ascending aortic aneurysm growth, an indication for intervention, is defined as ≥0.5 cm in 1 year or ≥0.3 cm per year in 2 consecutive years for those with sporadic aneurysms and ≥0.3 cm in 1 year for those with heritable thoracic aortic disease or bicuspid aortic valve.
  7. In patients undergoing aortic root replacement surgery, valve-sparing aortic root replacement is reasonable if the valve is suitable for repair and when performed by experienced surgeons in a Multidisciplinary Aortic Team.
  8. Patients with acute type A aortic dissection, if clinically stable, should be considered for transfer to a high-volume aortic center to improve survival. The operative repair of type A aortic dissection should entail at least an open distal anastomosis rather than just a simple supracoronary interposition graft.
  9. There is an increasing role for thoracic endovascular aortic repair in the management of uncomplicated type B aortic dissection. Clinical trials of repair of thoracoabdominal aortic aneurysms with endografts are reporting results that suggest endovascular repair is an option for patients with suitable anatomy.
  10. In patients with aneurysms of the aortic root or ascending aorta, or those with aortic dissection, screening of first-degree relatives with aortic imaging is recommended.

Introduction

...Introductio...

...mbering of the following tables may differ...


...2. Normal Anatomy, Abnormal...

...atomy of the Aorta and Its Main Branches...

...A Simplified Diagram Depicting the Key Histologic...

...igure 3. Classification of Aortic Anatomic Segment...

...re 4. Freedom From Ascending Aortic Compli...

...5. Relative Risk of Aortic Dissection by...

.... Classification of Aortic Dissection Chronicity...

...6. Acute Aortic Syndromes In aorti...

Figure 7. Classification of Acute Aortic Dissectio...

...Anatomic Reporting of Aortic Dissection...

...anisms of Dynamic and Static Obstr...

...gure 10. Classification of Thoracoabdominal A...

...11. Classification of Endoleak Types...


Assessment

...3. Imaging and Mea...

...3.1 Aortic Imaging Te...

...In patients with known or suspected ao...

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

...tients with known or suspected aortic dise...

...In patients with known or suspected aortic di...

...nts with known or suspected aortic di...

...is reasonable to measure the aorta from lea...

...ng inner-edge to inner-edge measureme...

...ential Elements of CT and MRI Aortic Imaging ...

...gnostic Performance of Aortic Imaging Modalitie...

...e 12. Aortic Imaging Techniques to De...

Figure 13. Reformatted CT Image Orthogo...

...Observed Relationship Between Annual Instituti...

...dicted Risk of Mortality Derived From the Logi...


Treatment

...Treatment...

...4. Multidisciplinary Ao...

...4.0. Recommend...

...patients with acute aortic disease that...

...For patients who are asymptomatic with e...


5. Share...

...5.0. Recommendation...

...patients with aortic disease, shared d...

...ents with aortic disease who are cont...


...6. Aneurysms...

...16. Recommendations for Management of Aneurysms o...

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

...A Syndromes and Conditions Attributable...

...actors for Familial TAD TAD and syndrom...

...6.1.2.1. HTAD: Geneti...

...s with aortic root/ascending aortic aneurysms or...

...ents with aortic root/ascending aortic aneurysms...

...patients with an established pathogeni...

...ients with TAD who have a pathogeni...

...ith aortic root/ascending aortic aneurysms or...

...patients with aortic root/ascending...

...s with acute type A aortic dissection, the...

...Evaluation and Genetic Testing Protocol for Pat...

...6.1.2.1.1. Surgi...

...ic patients with aneurysms of the aortic root o...

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

...with aneurysms of the aortic root or asce...

...Table 9....

...Her...

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

...6.1.2.2.1. Diagnosti...

...Initial Dia...

...ic diameters are stable, an annual surveillance TT...

...root, ascending aorta, or both are not...

...dults with Marfan syndrome, after the...

...Imagi...

...patients with Marfan syndrome who...

...with Marfan syndrome who have undergone aortic...

...6.1....

...s with Marfan syndrome, treatment with eith...

...ients with Marfan syndrome, the use of both a...

...6.1.2.2.3. Ma...

...with Marfan syndrome and an aortic root d...

...tients with Marfan syndrome, an aortic root di...

...ts with Marfan syndrome and a maxim...

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

...10. Features Associated With Increased Risk...

...6.1.2.2.4. Marfan Syndrom...

...ents with Marfan syndrome and a nondissected aneu...

...6.1.2.3.1. Imagi...

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

...tients with Loeys-Dietz syndrome and a d...

...nts with Loeys-Dietz syndrome, a baseli...

...In patients with Loeys-Dietz syndrome without di...

...ients with Loeys-Dietz syndrome without dilation o...

...6.1.2.3....

...nts with Loeys-Dietz syndrome, treatment wit...

...6.1....

...with Loeys-Dietz syndrome and aortic dilation,...

...ients with Loeys-Dietz syndrome attributabl...

...ble 11. Surgical Thresholds for Prophyl...

...6.1.2.5. Tu...

...s with Turner syndrome, TTE and cardiac MRI are re...

...In patients with Turner syndrome w...

...s with Turner syndrome without risk factors for...

...In patients with Turner syndrome and an ASI >2.3 c...

...ith Turner syndrome and risk facto...

...isk factors for aortic dissection (Table...

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

...k Factors for Aortic Dissection in Patients...

...Suggested Aortic Monitoring Protocol for Girls...

...rgical Thresholds for Prophylactic A...

...6....

...patients with a BAV, TTE is indicated to evaluate...

...with a BAV, CT or MRI of the thora...

...In patients with a BAV and either HTAD or phe...

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

In patients with a BAV, screening of all fi...

...6.1.3...

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

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

...6.1.3.2. BA...

...tients with a BAV and a diameter of the aort...

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

...In patients with a BAV, a diameter of t...

...with a BAV who are undergoing surgical a...

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

...Factors for Aortic Dissection Family hi...

...6.2. AAA: Cause, Risk...

...ho are ≥65 years of age who have ever sm...

...n or women who are ≥65 years of age...

...ho are ≥65 years of age who have ever...

In men or wom...

...tomatic men or women >75 years who have had a ne...

...ithm for Identifying Patients to Screen for Abd...

...actors for Abdominal Aortic AneurysmStrong Ri...

...6.4.1.1. BP Man...

...tients with TAA and an average systolic BP (S...

...ients with TAA, regardless of caus...

In patients with TAA, regardless...

...6.4.1.2. Treatm...

...nts with TAA and imaging or clinical evidenc...

...patients with TAA who have no evide...

...6.4.1.3....

...with TAA who smoke cigarettes, smoking cessa...

...6.4.1.4. An...

...tients with atherosclerotic TAA and concomitant a...

...6.4.2.1. BP Ma...

...nts with AAA and an average SBP of ≥130...

6.4.2.2...

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

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

In patients with AAA who smoke...

...with AAA with concomitant atheroma and/or PA...

...ts with a dilated thoracic aorta, a TTE is recom...

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

...ith a dilated thoracic aorta, follow-up im...

...6.4.3.2. Surveillanc...

...patients with an AAA of 3.0 cm to...

...with an AAA of 4.0 cm to 4.9 cm and in women wi...

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

...veillance CT is recommended. ( C-EO ,...

...h patients, when there is a contrai...

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

...e 20. The Frequency of Surveillance...

...6.5.1. Surgery for...

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

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

...atients with an aneurysm of the aortic root or as...

...matic patients with aneurysms of the aortic root o...

...ts undergoing repair or replacemen...

...atients undergoing repair or replacement of a...

...ergoing cardiac surgery for indicati...

...patients with a height >1 standard deviation ab...

...ic patients with aneurysms of the aortic...

...6.5.1.1. Su...

...ients with an aneurysm isolated to the...

...s undergoing aortic valve repair or replac...

...tients undergoing aortic root replacemen...

...In patients undergoing aortic root rep...

...6.5.2. Aor...

...In patients with an aortic arch aneurysm...

...ents with an isolated aortic arch aneurysm who a...

...In patients undergoing open surgical...

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

...s with an aortic arch aneurysm who are a...

...6.5.3....

...with intact descending TAA, repair is recom...

In patients with intact descending TAA an...

...atients at increased risk for periopera...

...dverse Aortic Events at 1 Year, Based...

...Ta...

...isk Features for Rupture Aneurysm growth of ...

...able 18. Patient Characteristics Associated With...

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

...tients with a descending TAA that...

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

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

...n patients with descending TAA who have u...

...6.5.3...

...nts with descending TAA undergoing TEVAR in whom...

...atients with ruptured descending TAA who...

...In patients with ruptured descen...

...6.5...

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

...ients with descending TAA undergoing T...

...patients with descending TAA undergoing TEV...

...6.5.4.1...

...ents with intact degenerative TAAA, r...

...ith intact degenerative TAAA, repair i...

...s with intact degenerative TAAA who ha...

...Features Associated With an Incre...

...6.5.4.2. Open Ve...

...Ruptured TAAA...

...In patients with ruptured TAAA requirin...

...nts with ruptured TAAA requiring intervention,...

...Int...

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

...ts with intact degenerative TAAA and suitable anat...

...6.5.4.3. TAAA Spi...

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

...In patients who experience delayed spina...

...es to Optimize Spinal Cord and End-O...

...6.5.4.4. TA...

...In patients undergoing open repair of T...

...n patients undergoing open or endovas...

...6...

...atients undergoing endovascular repair of AA...

...6.5.5.2...

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

...patients presenting with ruptured AAA...

...undergoing endovascular repair for ru...

...nts with ruptured AAA, permissive hy...

...6.5.5.3. Th...

...n patients with unruptured AAA, repair is reco...

...tients with unruptured AAA who have symptoms t...

...ith unruptured saccular AAA, intervention to...

...In patients with unruptured AAA and aneur...

...6.5...

...tients with nonruptured AAA with low to moderate...

...patients undergoing elective endovascular repa...

...patients with nonruptured AAA and a...

...ents with nonruptured AAA, a moderate to high...

...6.5.5...

...ents with asymptomatic small AAA an...

...reating common iliac artery aneurysms or ectasia a...

...6.5.6.1....

...nts treated with TEVAR, surveillance imaging wit...

...In patients treated with TEVAR, longit...

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

...In patients treated with open repai...

...6.5.6.2...

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

...In patients with AAA treated with E...

...In patients with AAA treated wit...

...ents with AAA treated with complex EVAR,...

...with AAA who have undergone open repair, surveil...

.... Abnormal Findings on Duplex Imaging After EVAR...


...7. Acute Aor...

...and Symptoms of AAS Clinical...

...7.2....

...s with a suspected AAS, CT is recomm...

...atients with a suspected AAS, TEE and MRI are r...

...Table 23. Pla...

...rtic Dissection on Chest X-Ray Mediastinal wide...

...ic Dissection Detection Risk Score...

...ta Simplified Score (AORTAs) Pretest Probabil...

...7.3.1. Acute Medi...

...nts presenting to the hospital with AAS, prompt...

Patients with AAS should be treated t...

...nagement should include intravenous b...

...hose with contraindications or intolerance...

...ients with AAS, initial management should include...

...atients with AAS should be treated with pa...

...cute Aortic Dissection: Malperfusio...

...7.3.2. Subsequent Medical...

...n patients with AAS, it is recommen...

...7.4.1.1. I...

...atients presenting with suspected or...

...tients presenting with acute type A aorti...

...ents presenting with non-hemorrhagic stroke compli...

...7.4.1.2. Management of Mal...

...s with acute type A aortic dissectio...

...with acute type A aortic dissection pres...

...ical Evidence of Malperfusion (“Malperfusio...

...7.4.1.3. S...

...Aortic Repair Strategie...

...s with acute type A aortic dissectio...

...acement is recommended with a mechanical or biolo...

...atients who are stable, valve-sparing root...

...atients with acute type A aortic d...

...patients with acute type A aortic disse...

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

...Perfu...

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

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

...ients with acute type A aortic dissection u...

...7.4.2. Management o...

In all patients with uncomplicated acut...

...ntion is recommended. ( C-LD ,...

In patients with rupture, in the pres...

...ents with other complications, in th...

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

...able 27. Consensus Features of Com...

...Table 28. High-R...

...High-Risk Im...

...mal aortic diameter >40 mm False-lumen di...

...High-Risk...

...hypertension despite >3 different classes...

...7.5....

...atients with complicated (Table 29) acute t...

...pt open surgical repair is recommend...

...cted patients with uncomplicated acute type...

...tients with uncomplicated acute ty...

...n patients with type B IMH who require repair of...

...nts with type B IMH who require repair of the dis...

...patients with uncomplicated type B IMH and hi...

...Table 29. Features...

...Malperfusion Periaortic hematoma Peri...

...igh-Risk Imaging Features of IMHFor Type A...

...7.6.1. PA...

In patients with PAU of the aorta with rupture...

...s with PAU of the ascending aorta with...

...patients with PAU of the aortic arch or de...

...with PAU of the abdominal aorta with associate...

...7.6.2. Isol...

...patients with isolated PAU who are symptomatic...

...ients with isolated PAU who are asymptoma...

...Table 31. H...

...aximum PAU diameter ≥13–20 mm...

...Dimensions of Penetrating Atherosclerotic Ulc...

...7.6.3. PAU Open Su...

...nts who require repair of a PAU in the ascending a...

...ents who require repair of a PAU in the distal...

...7.7.1.1. Ini...

...with BTTAI, management and treatment at a traum...

...In patients with BTTAI, anti-impulse thera...

...7.7.1.2. App...

...ents with grade 1 BTTAI (Figure 23), nonoper...

...ith grade 3 to 4 BTTAI (Figure 23) and nonp...

...n patients with grade 2 BTTAI (Figure 23) and wit...

...with grade 2 BTTAI (Figure 23) and without...

...ssification System for BTTAIs Aortic...

...le 32. High-Risk Imaging Features of BTTAI...

...In patients with BTTAI who meet indicat...

...7.7.2. Initial...

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

...with grade 4 BAAI (Table 33), repair...

...tients with grade 2 BAAI (Table 33) and...

...nts with BAAI, treatment with either endo...

...with grade 3 BAAI (Table 33), it may b...

...ts with BAAI, the usefulness of routin...

...able 33. Descriptions of Blunt Aortic Injury Gra...

...7.7.3...

...In patients with BTAI who have undergone aort...

...atients with BTAI who have not undergon...

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

...7.8.1. Long-Term Surveilla...

...In patients who have had an acute aort...

...s who have had an acute aortic dissection and IMH...

...7.8.2....

...with a previous acute aortic dissection and IMH, w...

...7.8.3. Long-...

...with a PAU who have undergone aortic repair,...

...ith a PAU that is being managed medically,...


...8. Pregnancy in Patie...

...8.1. Counseling...

...Prepregnancy...

...ients with genetic aortopathies att...

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

...nts with syndromic and nsHTAD, Turne...

...During Pregnancy...

...ents with aortic aneurysms, or at in...

...n patients with aortopathies who are pregn...

...atients with syndromic and nsHTAD,...

In pregnant patients with an aortopathic con...

...gnant patients with aortic disease who requ...

...8.2. Delivery in Pregna...

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

...patients with an aortopathy and an aortic diame...

...n pregnant patients with a diameter...

...gnant patients with a diameter of t...

...In pregnant patients with syndromic and n...

...8.3. Surgery Before...

...nd an aortic root diameter of >4.5 cm, aortic s...

...oot diameter is 4.0 cm to 4.5 cm,...

...TGFB3 and an aortic diameter of ≥4.5 cm,...

...BR2, or SMAD3, and the aortic diameter is...

...nd an aortic diameter of ≥4.5 cm, surgery before...

...aortic diameter is 4.0 cm to 4.4 cm, surger...

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

...atients with a BAV (in the absence...

In patients with sporadic aortic root aneurys...

...phylactic Aortic Surgery Before Pregnancy in...

...8.4. Pre...

...ents experiencing an acute type A aor...

...patients experiencing an acute type A aortic d...

...In patients experiencing an acute typ...

...patients with progressive aortic dila...


...9. Other Aortic Cond...

...9.1. Infla...

...Diagnosis...

...tients with large vessel vasculitis (LVV), p...

...T...

...s with active GCA or Takayasu arteri...

...ients with GCA who have evidence of active a...

...patients with Takayasu arteritis, nonbiologi...

...nts with active GCA or Takayasu arteriti...

...In patients with GCA or Takayasu a...

In patients with GCA or Takayasu a...

...nostic Criteria for Inflammatory Aortitis...

...9.2.1. Diagnosis and Manage...

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

...lect patients, treatment with endovascular repair...

...tients with infectious aortitis complic...

...ith infectious aortitis, intravenous antimicrobial...

...e 2018 European Alliance of Associations for...

...The 2018 European Alliance of Associations for Rh...

...Management of Aortic Mycotic Aneurysm: Comp...

...9.2.2....

...Diagnosis...

...s with a prosthetic aortic graft, who have signs a...

T...

...In patients with an infected prosthetic...

...patients with an infected prosthetic aortic graf...

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

...Late Manageme...

...tients who have undergone treatment of an acute pr...

...n patients with an infected prosthetic...

...9.3. Atheroscl...

...atients with aortic atherosclerotic...

...In patients with aortic atheroscleroti...

...ts with aortic atheromas of a thicknes...

...9.4....

...patients with CoA, including those who have under...

...with CoA, BPs should be measured in both ar...

...with significant native or recurrent Co...

...patients with CoA, guideline-directed me...

...ents with CoA, screening for intracran...

...Table 37. Criteri...

...resence of significant CoA is based on eviden...

...A noninvasive blood pressure diff...

...9.4.2.1. Aberrant...

...s discovered to have an ASCA in the absenc...

...with Kommerell’s diverticulum,...

...9.4....

...In patients with an aberrant left ve...

...27. Measurements of Kommerell’s Div...

...9.4.2.3. Bovine...

...atients with bovine arch (common innominate a...

Figure 28. Normal and Bovine Aortic Arch...


...10...

...Recommendations for Phys...

...or patients with significant aortic dis...

...who have undergone surgery for aortic aneurysm...

...s with thoracic or abdominal aortic...

...tients with clinically significant aortic diseas...