Editor's Note: Please see Part 2 of the Aortic Disease Guideline Key Perspectives.

The 2022 American College of Cardiology/American Heart Association (ACC/AHA) aortic disease guideline provides recommendations on the diagnosis, evaluation, medical therapy, endovascular and surgical intervention, and long-term surveillance of patients with aortic disease across its multiple clinical presentations. The following is the first of two sets of key perspectives on the guideline:

1. Anatomy:
   • In addition to defining normal anatomy, the guideline defines thoracic and abdominal aorta landing zones pertinent to the use of aortic endovascular repair.
   • Among patients who are smaller than average or taller than average, indications for surgical intervention may incorporate measures of aorta size indexed to body size, including the aortic diameter indexed to body surface area (the aortic size index [cm/m²]) or to the square of height (the aortic height index [cm/m²]), or the aortic cross-sectional area indexed to height (cm²/m).
2. Imaging:
   • Computed tomography (CT), magnetic resonance imaging (MRI), and echocardiography should use standardized measurement techniques and include measurements at reproducible anatomic landmarks.
   • CT and MRI reports should include essential elements including maximal aortic diameters, wall changes, luminal stenosis, findings suggestive of acute aortic syndrome, involvement of branch vessels, presence of prior repair, comparison with previous examinations, and impressions regarding disease classification.
   • The use of ionizing radiation should be kept as low as feasible while maintaining diagnostic image quality.
3. Multidisciplinary aortic teams, high-volume centers:
   • A multidisciplinary team should determine the most suitable intervention for patients with acute aortic disease who require urgent intervention.
   • Referral to a high-volume center with experienced surgeons as part of a multidisciplinary aortic team is reasonable for asymptomatic patients with extensive aortic disease, for patients who might benefit from complex open and endovascular repairs, and for patients with multiple comorbidities in whom intervention is considered.
   • Transfer from a low-volume to a high-volume center is reasonable for patients with acute type A aortic dissection who are stable for transfer.
4. Shared decision-making:
   • Shared decision-making is recommended in determining thresholds for intervention, deciding on the type of surgical repair, choosing between open surgical and endovascular approaches, and medical management and surveillance decisions in patients with aortic disease.
   • Among patients with aortic disease who are pregnant or contemplating pregnancy, shared decision-making is recommended in considering the risks of pregnancy, thresholds for prophylactic aortic surgery, and the mode of delivery.
5. Aneurysms, general considerations:
   • The causes of thoracic aortic aneurysm (TAA) include:
     • Syndromic heritable thoracic aortic disease (HTAD), including Marfan syndrome (MFS), Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome.
     • Nonsyndromic HTAD (nsHTAD).
     • Congenital conditions, including bicuspid aortic valve (BAV), Turner syndrome, and aortic coarctation.
     • Degenerative conditions, influenced by hypertension and atherosclerosis.
     • Inflammatory aortitis, including giant cell arteritis, Takayasu arteritis, and Behçet disease.
     • Infectious aortitis, including bacterial, fungal, and syphilitic.
     • Prior aortic dissection.
     • Prior traumatic aortic injury.
   • Patients with thoracic aortic disease (TAD) should undergo a thorough family history for aortic, intracranial, and peripheral aneurysm, and for unexplained sudden death; and for risk factors for HTAD.
   • Genetic testing is recommended for people with syndromic features, family history of TAD, or early age of disease onset (<60 years).
   • Thoracic aortic imaging of family members is recommended to screen for asymptomatic TAA:
     • Based on genetic testing in relatives of patients with TAD and a pathologic or likely pathologic genetic variant.
     • In a family with history of TAA or dissection but a disease-causing variant is not identified.
     • In first-degree relatives of all other patients with TAD (with no known family history of TAD, or with no identified pathologic or likely pathologic genetic variant).
   • Rapid growth is defined as:
     • nsHTAD or sporadic TAA: ≥0.5 cm/yr or ≥0.3 cm/yr for 2 consecutive years.
     • MFS: ≥0.3 cm/yr.
     • BAV: ≥0.3 cm/yr.
   • Other specific recommendations for different conditions are summarized separately.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Valvular Heart Disease, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Angiography, Computed Tomography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging

Keywords: Aortic Aneurysm, Aortic Aneurysm, Thoracic, Aortic Rupture, Aortic Valve Disease, Aortic Valve Stenosis, Aortitis, Arterial Occlusive Diseases, Bicuspid Aortic Valve Disease, Cardiac Surgical Procedures, Diagnostic Imaging, Echocardiography, Ehlers-Danlos Syndrome, Endovascular Procedures, Heart Defects, Congenital, Heart Valve Diseases, Loeys-Dietz Syndrome, Magnetic Resonance Angiography, Magnetic Resonance Imaging, Interventional, Marfan Syndrome, Practice Guideline, Pregnancy, Tomography, Emission-Computed, Vascular Diseases

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