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Practice Guideline
. 2023 Nov;166(5):e182-e331.
doi: 10.1016/j.jtcvs.2023.04.023. Epub 2023 Jun 28.

2022 ACC/AHA guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines

Writing Committee Members; Eric M Isselbacher, Ourania Preventza, James Hamilton Black 3rd, John G Augoustides, Adam W Beck, Michael A Bolen, Alan C Braverman, Bruce E Bray, Maya M Brown-Zimmerman, Edward P Chen, Tyrone J Collins, Abe DeAnda Jr, Christina L Fanola, Leonard N Girardi, Caitlin W Hicks, Dawn S Hui, William Schuyler Jones, Vidyasagar Kalahasti, Karen M Kim, Dianna M Milewicz, Gustavo S Oderich, Laura Ogbechie, Susan B Promes, Elsie Gyang Ross, Marc L Schermerhorn, Sabrina Singleton Times, Elaine E Tseng, Grace J Wang, Y Joseph Woo; Peer Review Committee Members; David P Faxon, Gilbert R Upchurch Jr, Aaron W Aday, Ali Azizzadeh, Michael Boisen, Beau Hawkins, Christopher M Kramer, Jessica G Y Luc, Thomas E MacGillivray, S Christopher Malaisrie, Kathryn Osteen, Himanshu J Patel, Parag J Patel, Wanda M Popescu, Evelio Rodriguez, Rebecca Sorber, Philip S Tsao, Annabelle Santos Volgman; AHA/ACC Joint Committee Members; Joshua A Beckman, Catherine M Otto, Patrick T O'Gara, Anastasia Armbruster, Kim K Birtcher, Lisa de las Fuentes, Anita Deswal, Dave L Dixon, Bulent Gorenek, Norrisa Haynes, Adrian F Hernandez, José A Joglar, W Schuyler Jones, Daniel Mark, Debabrata Mukherjee, Latha Palaniappan, Mariann R Piano, Tanveer Rab, Erica S Spatz, Jacqueline E Tamis-Holland, Y Joseph Woo
Practice Guideline

2022 ACC/AHA guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines

Writing Committee Members et al. J Thorac Cardiovasc Surg. 2023 Nov.

Abstract

Aim: The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

Methods: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.

Structure: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

Keywords: ACC/AHA Clinical Practice Guidelines; abdominal aortic aneurysm; aortic dissection; aortitis; aortopathy; bicuspid aortic valve; blunt traumatic aortic injury; cardiac surgery; endovascular aortic repair; guidelines; heritable thoracic aortic disease; intramural hematoma; loeys-dietz syndrome; malperfusion syndrome; marfan syndrome; penetrating atherosclerotic ulcer; thoracic aortic aneurysm; thoracic endovascular aortic repair; thoracoabdominal aortic aneurysm; vascular surgery.

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Figures

FIGURE 1.
FIGURE 1.
Applying American College of Cardiology/American Heart Association class of recommendation and level of evidence to clinical strategies, interventions, treatments, or diagnostic testing in patient care* (updated May 2019).
FIGURE 2.
FIGURE 2.
The anatomy of the aorta and its main branches.
FIGURE 3.
FIGURE 3.
A simplified diagram depicting the key histologic components of the aortic wall. The medial layer in human aortas contains >50 alternating layers of elastin and smooth muscle cells (whereas only 5 are shown in this simplified illustration). Adapted (cropped) from “Illustration of tunics of the arteries vs veins” by Malgosia Wilk-Blaszczak, used under CC-BY 4.0. “Illustration of tunics of the arteries vs veins” is adapted (cropped) from figure 20.3 in BC OpenStax Anatomy and Physiology used under CC-BY 4.0.
FIGURE 4.
FIGURE 4.
Classification of aortic anatomic segments by 11 landing zones. Zone 0 (involves the ascending to distal end of the origin of the innominate artery); Zone 1 (involves the origin of the left common carotid; between the innominate and the left carotid); Zone 2 (involves the origin of the left subclavian; between the left carotid and the left subclavian); Zone 3 (involves the proximal descending thoracic aorta down to the T4 vertebral body; the first 2 cm distal to the left subclavian); Zone 4 (the end of zone 3 to the mid-descending aorta - T6); Zone 5 (the mid-descending aorta to the celiac); Zone 6 (involves the origin of the celiac; the celiac to the superior mesenteric); Zone 7 (involves the origin of the superior mesenteric artery; the superior mesenteric to the renals); Zone 8 (involves the origin of the renal arteries; the renal to the infrarenal abdominal aorta); Zone 9 (the infrarenal abdominal aorta to the level of aortic bifurcation ); Zone 10 (the common iliac); Zone 11 (involves the origin of the external iliac arteries). From Czerny et al. Copyright 2019, with permission from Elsevier, Inc., Now Medical Studios, and Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.
FIGURE 5.
FIGURE 5.
Freedom from ascending aortic complications for patients with bicuspid aortic valve disease. Patients with moderate dilation of the ascending aorta (4.5 cm-4.9 cm) had a significantly increased risk of future aortic complications (aneurysm, dissection, or sudden death). Reprinted from Borger et al. Copyright 2004, with permission from Elsevier, Inc. and the American Association for Thoracic Surgery.
FIGURE 6.
FIGURE 6.
Relative risk of aortic dissection by size range. The relative risk of aortic dissection begins to increase appreciably at a diameter of 4.0 cm to ≥4.4 cm and then increases dramatically at a diameter of 4.5 cm. Reprinted from Paruchuri et al. Copyright 2005, with permission from Karger Publishers, Basel Switzerland.
FIGURE 7.
FIGURE 7.
Acute aortic syndromes. In aortic dissection, a tear in the aortic intima allows blood to penetrate the aortic media, pushing the dissection flap into the middle of the aorta, separating the true from the false lumen. In intramural hematoma, blood leaks into the aortic media at low pressure, forming a thrombus that pushes the outer wall of the aorta outward, leaving a relatively normal appearing aortic lumen. A penetrating atherosclerotic ulcer allows blood to enter the aortic media, but atherosclerotic scarring of the aorta typically confines the blood collection, often resulting in a localized dissection or pseudoaneurysm. Adapted with permission from Springer Nature Customer Service Centre GmbH: Springer Nature, Clough et al Copyright 2015.
FIGURE 8.
FIGURE 8.
Classification of acute aortic dissection. The DeBakey and Stanford classification systems are used most commonly. The DeBakey system offers greater anatomic detail, whereas the Stanford system is simpler, essentially distinguishing those dissections that involve the ascending thoracic aorta from those that do not.
FIGURE 9.
FIGURE 9.
Anatomic reporting of aortic dissection based on the 2020 SVS/STS reporting standards. SVS, Society for Vascular Surgery; STS, Society of Thoracic Surgeons. Reprinted from Lombardi et al. Copyright 2020, with permission from Elsevier, Inc., the Society of Thoracic Surgeons, and the Society for Vascular Surgery.
FIGURE 10.
FIGURE 10.
Mechanisms of dynamic and static obstruction in aortic dissection. (A) Static obstruction occurs when the dissection flap extends from the aortic lumen into the ostium of the affected branch vessel, leading to localized thrombosis of the branch false lumen that narrows or colludes the branch true lumen and, consequently, impairs distal branch perfusion. (B) Dynamic obstruction occurs when the false lumen becomes persistently pressurized and compresses the true lumen, in turn pushing the dissection flap up against the ostium of the affected branch vessel, significantly reducing or occluding its flow. (C) Sometimes, a branch vessel can suffer from both static and dynamic obstruction at the same time. Adapted with permission from Grewal et al. Copyright 2021, Elsevier, Inc.
FIGURE 11.
FIGURE 11.
Classification of thoracoabdominal aortic aneurysms. The classification of thoracoabdominal aortic aneurysms according to extent of aortic involvement as originally proposed by Crawford is as follows: Extent I, below the left subclavian to above the celiac axis or opposite the superior mesenteric and above the renal arteries; Extent II, below the left subclavian and including the infrarenal abdominal aorta to the level of the aortic bifurcation; Extent III, below T6 intercostal space, tapering to just above the infrarenal abdominal aorta to the iliac bifurcation; and Extent IV, below T12, tapering to above the iliac bifurcation. Safi et al proposed expanding the classification with the addition of Extent V, below T6, tapering to just above the renal arteries.
FIGURE 12.
FIGURE 12.
Classification of endoleak types. Endoleaks are classified by 5 types: Type Ia, proximal attachment site endoleak; Type Ib, distal attachment site endoleak; Type II, backfilling of the aneurysm sac through branch vessels of the aorta; Type III, graft defect or component misalignment; Type IV, leakage through the graft wall attributable to endograft porosity; and Type V, caused by “endotension,” possibly resulting from aortic pressure transmitted through the graft/thrombus to the aneurysm sac. Adapted from Rokosh et al. Copyright 2021, with permission from Elsevier, Inc., and the Society for Vascular Surgery.
FIGURE 13.
FIGURE 13.
Aortic imaging techniques to determine the presence and progression of aortic disease. (A) Schematic shows the leading-edge to leading-edge measurement technique used in echocardiography, from left to right: measurement of the aortic root (sinuses of Valsalva), sinotubular junction, and proximal tubular ascending aorta. (B) Inner-wall to inner-wall measurements of the aortic root used in MRI and CT. In addition, a consistent approach to measuring all 3 sinuses with MRI and CT is necessary. The sinus-to-commissure and sinus-to-sinus measurements can both be used, but consistency is necessary for interval surveillance. (C) Standard measurement locations for MRI and CTwith the inner-wall to inner-wall technique. Adapted from Borger et al. Copyright 2018, with permission from Elsevier, Inc. MRI, Magnetic resonance imaging; CT, computed tomography. *Leading-edge to leading-edge. †Inner-wall to inner-wall.
FIGURE 14.
FIGURE 14.
Reformatted CT image orthogonal to the aortic root at the level of the sinuses of valsalva. The root diameter can be measured from sinus-to-sinus (S-S) or sinus-to-commissure (S-C). The aortic root area (A) can also be measured. CT, Computed tomography; ROI, region-of-interest.
FIGURE 15.
FIGURE 15.
Observed relationship between annual institutional casevolume and risk-adjusted odds ratio for operative mortality ±2 standard deviations as assessed with regression analysis. The odds ratio for operative mortality decreased as institutional case volume increased. Adapted from Hughes et al. Copyright 2013, with permission from Elsevier Inc.
FIGURE 16.
FIGURE 16.
Predicted risk of mortality derived from the logistic regression model without center case volume as a covariate. Actual mortality and the ratio of actual mortality to predicted mortality (A/P ratio, the risk-adjusted mortality rate) are also shown. A similar predicted risk of mortality across the case volume strata and a decrease in the actual mortality at higher center casevolume are seen. Reprinted from Mori et al. Copyright 2018, with permission from Elsevier Inc.
FIGURE 17.
FIGURE 17.
Recommendations for management of aneurysms of the aortic root and ascending aorta according to known causative factors.
FIGURE 18.
FIGURE 18.
Evaluation and genetic testing protocol for patients with TAD. Genetic testing is recommended for individuals with syndromic features, family history of TAD, and/or early age of disease onset. Thoracic aortic imaging is recommended for first-degree relatives of all individuals with TAD, regardless of age of onset, to detect asymptomatic aneurysms. Positive genetic testing should trigger gene-based management and cascade testing of at-risk relatives. When testing is negative or reveals variants of unknown significance, first-degree relatives should undergo screening aortic imaging. Modified with permission from Milewicz et al. Copyright 2021, Minerva Medica. Blue (+), positive; green (−), negative; TAD, Thoracic aortic disease; MFS, Marfan syndrome; LDS, Loeys-Dietz syndrome; vEDS, vascular Ehlers-Danlos syndrome; VUS, variants of unknown significance; TAAD, thoracic aortic aneurysm and dissection. *Aneurysms are typically asymptomatic.
FIGURE 19.
FIGURE 19.
Suggested aortic monitoring protocol for girls and women with turner syndromewho are ≥15 years of age. *Surveillance frequency may vary depending on disease severity (ie, aortic valve dysfunction, severity of coarctation obstruction, hypertension, and left ventricular hypertrophy). Color corresponds to Class of Recommendations in Figure 1. TTE, Transthoracic echocardiography; CoA, coarctation of the aorta; BAV, bicuspid aortic valve; HTN, hypertension; ASI, aortic size index; MRI, magnetic resonance imaging. Modified from Silberbach et al. Copyright 2018, with permission from American Heart Association, Inc. Modified from Gravholt et al. Copyright 2017, with permission from Bioscientifica Limited.
FIGURE 20.
FIGURE 20.
Algorithm for identifying patients to screen for abdominal aortic aneurysm. Colors correspond to Class of Recommendations in Figure 1. AAA, Abdominal aortic aneurysm.
FIGURE 21.
FIGURE 21.
The Frequency of surveillance imaging of abdominal aortic aneurysms based on current aortic diameter. Color corresponds to Class of Recommendations in Figure 1.
FIGURE 22.
FIGURE 22.
Acute aortic dissection: Malperfusion treatment options. TEVAR, Thoracic endovascular aortic repair.
FIGURE 23.
FIGURE 23.
Dimensions of penetrating atherosclerotic ulcers. (A) Maximal aortic diameter at ulcer site diameter (from ulcer across to opposite aortic wall). (B) Depth of intramural blood pool. (C) Length of intimal defect at ulcer site. (D) Width of intramural blood pool. Adapted from Gifford et al, Copyright 2016, with permission from Elsevier, Inc., and from Cho et al Copyright 2004 with permission from the Society for Vascular Surgery.
FIGURE 24.
FIGURE 24.
Classification system for BTTAIs. Aortic injuries are classified according to severity, based on the findings of diagnostic imaging. Grade 1, intimal tear. intimal flap, or both. Grade 2, intramural hematoma. Grade 3, aortic wall disruption with pseudoaneurysm. Grade 4, aortic wall disruption with free rupture. BTTAI, Blunt traumatic thoracic aortic injury. Adapted from Azizzadeh et al, Copyright 2009, with permission from Elsevier, Inc. and the Society for Vascular Surgery.
FIGURE 25.
FIGURE 25.
Abdominal aortic zones of injury for surgical approaches and abdominal zones of injury based on trauma classification. (A) The abdominal aortic zones of injury described by Shalhub et al. (B) The abdominal zones of injury traditionally described in trauma. The abdominal aortic zones of injury may help in prognostication and deciding whether an endovascular or open repair is feasible. Shalhub et al found that the mortality rate was highest in zone 2 (see panel A) grade 4 aortic injuries (Table 32). Moreover, no zone 2 aortic injuries identified in a multicenter experience were managed by endovascular means. Panel A, adapted from Shalhub et al. Copyright 2014, with permission from Wolters Kluwer Health, Inc.
FIGURE 26.
FIGURE 26.
The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against Rheumatism) recommended algorithms for the pharmacological treatment of giant cell arteritis. GCA, Giant cell arteritis; GC, glucocorticoids. Modified from Hellmich et al. Copyright 2020, with permission from BMJ Publishing Group Limited.
FIGURE 27.
FIGURE 27.
The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against Rheumatism) recommended algorithms for the pharmacological treatment of Takayasu arteritis. GC, Glucocorticoids; csDMARD, conventional synthetic disease modifying antirheumatic drug; TNF, tumor necrosis factor. Modified from Hellmich et al. Copyright 2020, with permission from BMJ Publishing Group Limited.
FIGURE 28.
FIGURE 28.
Measurements of Kommerell’s diverticulum. Two diameter measurements should be obtained using cross-sectional imaging: the diverticulum orifice (radially and longitudinally at the aortic wall) and the combined diameter of the diverticulum and adjacent descending thoracic aorta (measured from the apex of the diverticulum to the opposite aortic wall). RCA, Right common carotid artery; LCA, left common carotid artery; LSA, left subclavian artery; ARSA, aberrant right subclavian artery. Adapted from Erben et al, Copyright 2020, with permission from Elsevier, Inc., and the Society for Vascular Surgery.
FIGURE 29.
FIGURE 29.
Normal and bovine aortic arch configurations. (A) Type I aortic arch: The normal aortic arch configuration. (B) Type II-A aortic arch: The left common carotid artery originates from the innominate artery. (C) Type II-B aortic arch: The innominate and left common carotid arteries share a common origin. RCA, Right common carotid artery; LCA, left common carotid artery; RVA, right vertebral artery; LVA, left vertebral artery; RSA, right subclavian artery; LSA, left subclavian artery. Adapted from Layton et al. Copyright 2006, American Society of Neuroradiology. Used with permission from Mayo Foundation for Medical Education and Research, all rights reserved.
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Multidisciplinary Aortic Teams

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Shared Decision-Making

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Thoracic Aortic Aneurysm (TAA) Cause

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Sporadic and Degenerative TAA

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HTAD: Genetic Testing and Screening of Family Members for TAD

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Surgical Considerations for Nonsyndromic Heritable TAAs and No Identified Genetic Cause

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Diagnostic and Surveillance Aortic Imaging in Marfan Syndrome

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Medical Therapy in Marfan Syndrome

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Marfan Syndrome Interventions: Replacement of the Aortic Root in Patients With Marfan Syndrome

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Marfan Syndrome Interventions: Replacement of Primary (Nondissected) Aneurysms of the Aortic Arch, Descending, and Abdominal Aorta in Patients With Marfan Syndrome

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Imaging in Loeys-Dietz Syndrome

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Medical Therapy in Loeys-Dietz Syndrome

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Loeys-Dietz Syndrome Surgical Interventions: Replacement of the Aorta in Patients With Loeys-Dietz Syndrome

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Vascular Ehlers-Danlos Syndrome: Imaging, Medical Therapy, and Surgical Intervention

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Turner Syndrome

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Pathogenic or Likely Pathogenic Variants in ACTA2, PRKG1, MYH11, MYLK, and LOX: Recommendations for Surveillance of Aorta, Medical Therapy, and Aortic Surgical Intervention

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BAV Aortopathy

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Routine Follow-Up of BAV Disease Aortopathy

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BAV Aortopathy Interventions: Replacement of the Aorta in Patients With BAV

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AAA: Cause, Risk Factors, and Screening

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Growth and Natural History of Aortic Aneurysms

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Medical Management of Sporadic and Degenerative Aortic Aneurysm Disease

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BP Management in Sporadic TAA

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Treatment of TAA With Statins

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Smoking Cessation in TAA

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Antiplatelet Therapy in TAA

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BP Management in AAA

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Treatment of AAA With Statins

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Smoking Cessation in AAA

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Antithrombotic Therapy in AAA

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Surveillance of Thoracic Aortic Dilation and Aneurysm

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Surveillance of Abdominal Aortic Dilation and Aneurysm

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Surgical and Endovascular Management of Aortic Aneurysms

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Surgery for Sporadic Aneurysms of the Aortic Root and Ascending Aorta

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Surgical Approach for Patients With Sporadic Aneurysms of the Aortic Root and Ascending Aorta Meeting Criteria for Surgery

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Aortic Arch Aneurysms

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Size Thresholds for Repair of Descending TAA

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Endovascular Versus Open Repair of Descending TAA

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Left Subclavian Artery Management

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Celiac Artery Management

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Ruptured Descending TAA

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Access Issues for TEVAR in Descending TAA

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Size Thresholds for Open Surgical Repair of TAAA

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    1. Zafar MA, Chen JF, Wu J, et al. Natural history of descending thoracic and thoracoabdominal aortic aneurysms. J Thorac Cardiovasc Surg. 2021;161:498–511.e1. - PubMed
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    1. Gambardella I, Gaudino MFL, Rahouma M, et al. Impact of left ventricular ejection fraction on the outcomes of open repair of descending thoracic and thoracoabdominal aneurysms. J Thorac Cardiovasc Surg. 2021;161:534–41.e5. - PubMed

Open Versus Endovascular Repair of TAAA

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TAAA Spinal Cord Protection

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TAAA Renal and Visceral Organ Protection

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Access During Endovascular Repair of AAA

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Repair of Ruptured AAA

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Threshold for AAA Repair

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Open Versus Endovascular Repair of AAA

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Treatment of Concomitant Common Iliac Aneurysms

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Surveillance After TAA Repair

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Surveillance After AAA Repair

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Presentation

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AAS: Diagnostic Evaluation (Imaging, Laboratory Testing)

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Acute Medical Management of AAS

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Subsequent Medical Management of AAS

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Initial Surgical Considerations in Acute Type A Aortic Dissection

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Management of Malperfusion

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Surgical Repair Strategies in Acute Type A Aortic Dissection

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Management of Acute Type B Aortic Dissection

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Management of IMH

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PAU With IMH, Rupture, or Both

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Isolated PAU

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PAU Open Surgical Repair Versus Endovascular Repair

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Initial Management of BTTAI in the Emergency Department

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Approach to the Initial Management of BTTAI

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Endovascular Versus Open Surgical Repair

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Initial Management of Blunt Traumatic Abdominal Aortic Injury (BAAI)

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Long-Term Management and Surveillance After Blunt Traumatic Aortic Injury (BTAI)

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Long-Term Surveillance Imaging After Aortic Dissection and IMH

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Long-Term Management After Acute Aortic Dissection and IMH

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Long-Term Management and Surveillance for PAUs

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Counseling and Management of Aortic Disease in Pregnancy and Postpartum

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Delivery in Pregnant Patients With Aortopathy

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Surgery Before Pregnancy in Women With Aortic Disease

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Inflammatory Aortitis: Diagnosis and Treatment of Takayasu Arteritis and Giant Cell Arteritis

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Atherosclerotic Disease

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Aortic Thrombus

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Aortic Occlusion

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Porcelain Aorta

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Coarctation of the Aorta (CoA) and Congenital Abnormalities of the Arch

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Coarctation of the Aorta

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ASCA, Kommerell’s Diverticulum

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Aberrant Left Vertebral Artery Origin

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Bovine Arch (Common Innominate and Left Carotid Artery)

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Tumors

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Physical Activity and Quality of Life

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Cost and Value Considerations

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Biomarker Studies

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Genetic and Nongenetic Factors

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Biomechanics of the Aorta

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Sex, Race, and Ethnicity

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Quality of Life in Patients With Aortic Disease

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New Endovascular Technology

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Optimal Exercise and Rehabilitation Protocols

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Equitable Care and Training Opportunities

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