Editor's Note: Please see Part 1 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 second of two sets of key perspectives on the guideline:

6. Aneurysms, specific recommendations:
   • Nonsyndromic heritable thoracic aortic disease (nsHTAD). For patients with nsHTAD and no identifiable genetic cause, repair of the aorta is recommended when the diameter is ≥5.0 cm in the absence of high-risk features or ≥4.5 cm in the presence of high-risk features (family history of dissection at an aortic diameter <5.0 cm or unexplained sudden death at age <50 years, or rapid aortic growth).
   • Marfan syndrome (MFS).
     • Treatment is recommended with either a beta-blocker or an angiotensin receptor antagonist (ARB) in maximally tolerated doses.
     • Surgery to replace the aortic root and ascending aorta is recommended with an aortic root diameter ≥5.0 cm; surgery with an experienced surgeon is reasonable with either an aortic root diameter ≥4.5 cm plus high-risk features (family history of dissection, rapid growth, diffuse aortic root and ascending aorta dilation, or marked vertebral artery tortuosity), or with a cross-sectional aortic root area to patient height ratio ≥10 cm²/m.
   • Loeys-Dietz syndrome (LDS).
     • Treatment is reasonable with either a beta-blocker or an ARB in maximally tolerated doses.
     • Surgery for the prophylactic repair of the aortic root and ascending aorta should be based on the specific genetic variant, aortic diameter, aortic growth rate, extra-aortic features, family history, patient age and sex, and physician and patient preferences.
   • Bicuspid aortic valve (BAV) aortopathy.
     • All first-degree relatives should be screened with transthoracic echocardiography (TTE) for BAV and dilation of the aortic root and/or ascending aorta; computed tomography (CT) or magnetic resonance imaging (MRI) should be used if assessment with TTE is incomplete. It is reasonable to screen all first-degree relatives with TTE in the setting of a patient with BAV without aortopathy.
     • Surgery to replace the aortic root and/or ascending aorta is recommended with an aortic diameter ≥5.5 cm. Surgical intervention is reasonable with a cross-sectional area to height ratio ≥10 cm²/m, with an aortic diameter 5.0-5.4 cm plus a risk factor for dissection (family history of dissection, rapid growth, aortic coarctation, or "root phenotype" aortopathy), or with an aortic diameter ≥4.5 cm at the time of aortic valve replacement or repair.
   • Abdominal aortic aneurysm (AAA).
     • Ultrasound screening for AAA is recommended in men ≥65 years who have ever smoked and in men or women ≥65 years with a first-degree relative with AAA. Ultrasound screening is reasonable in women ≥65 years who have ever smoked.
     • Moderate- or high-intensity statin therapy is recommended in patients with AAA and evidence of aortic atherosclerosis. Low-dose aspirin may be considered in patients with AAA and concomitant atheroma or penetrating aortic ulcer (PAU).
     • Surveillance should be every 3 years in patients with AAA diameter 3.0-3.9 cm, annually in men with AAA diameter 4.0-4.9 cm or women with AAA diameter 4.0-4.4 cm, and every 6 months in men with AAA diameter ≥5.0 cm or women with AAA diameter ≥4.5 cm.
     • Repair is recommended in men with AAA diameter ≥5.5 cm or women with AAA diameter ≥5.0 cm, and in the setting of symptoms attributable to the aneurysm.
   • Sporadic/degenerative thoracic aortic aneurysm (TAA).
     • The use of antihypertensive medications is recommended among patients with sporadic TAA and blood pressure (BP) ≥130/80 mm Hg. In the absence of contraindications, the use of beta-blockers and/or ARB therapy is reasonable. Moderate- or high-intensity statin therapy is reasonable in patients with TAA and clinical or imaging evidence of atherosclerosis. Low-dose aspirin is reasonable in patients with TAA and concomitant atherosclerosis or PAU.
     • TTE is recommended at the time of diagnosis to assess aortic valve anatomy and function and thoracic aortic diameters. CT or MRI is reasonable at the time of diagnosis. Surveillance imaging with TTE, CT, or MRI is reasonable after 6-12 months, and then, if stable, every 6-24 months.
     • For aortic root or ascending aorta aneurysm, surgery is recommended for symptoms attributable to the aneurysm, diameter ≥5.5 cm, or rapid growth; and reasonable at the time of tricuspid aortic valve replacement with an aortic diameter ≥5.0 cm. With an experienced surgeon, surgery is reasonable with an aortic diameter ≥5.0 cm (or in a patient with height >1 standard deviation above or below the mean with a cross-sectional aortic area to height ratio ≥10 cm²/m), or at the time of tricuspid aortic valve replacement with an aortic diameter ≥4.5 cm.
     • Thoracic endovascular aortic repair (TEVAR) is recommended over open surgery in patients without MFS, LDS, or vascular Ehlers-Danlos syndrome who have descending TAA meeting criteria for intervention and suitable anatomy; consideration for alternative vascular access is recommended for patients with smaller or diseased access vessels.
   • Surveillance after TAA repair.
     • After treatment of TAA with TEVAR, surveillance imaging with CT is recommended after 1 month and 12 months, and then annually if stable; MRI is a reasonable alternative to CT.
     • After open repair of TAA, CT or MRI within 1 year and then every 5 years is reasonable in the absence of residual aortopathy; annual imaging is reasonable if there is residual aortopathy or abnormal findings on surveillance imaging.
7. Acute aortic syndrome (AAS):
   • Medical therapy. Patients with AAS should be treated to a systolic BP <120 mm Hg or to the lowest BP that maintains end-organ perfusion, and to a heart rate of 60-80 bpm. Unless contraindicated, intravenous beta-blockers are recommended as initial management, and vasodilators if BP is not adequately controlled with beta-blockers. Long-term beta-blockers are recommended after the initial treatment of AAS.
   • Type A acute aortic dissection.
     • Immediate operative repair is recommended for patients with acute type A aortic dissection with renal, mesenteric, or lower extremity malperfusion.
     • Aortic valve resuspension is recommended over replacement in patients with type A dissection and no significant aortic valve leaflet pathology; aortic valve replacement with a mechanical or biological valved conduit is recommended if there is extensive destruction of the aortic root, a root aneurysm, or a known genetic disorder. An open distal anastomosis is recommended in the aortic repair.
   • Type B acute aortic dissection.
     • Medical therapy is recommended as the initial management in patients with uncomplicated acute type B dissection.
     • Intervention is recommended in patients with acute type B dissection and rupture, branch artery occlusion and malperfusion, dissection extension, progressive aortic enlargement, intractable pain, or uncontrolled hypertension. In patients with rupture and suitable anatomy, endovascular stent-grafting is recommended over open surgical repair.
   • Intramural hematoma (IMH). Urgent repair is recommended in patients with acute type A or type B IMH and complications (malperfusion, periaortic hematoma, pericardial effusion with tamponade, refractory or recurrent pain, or rupture). Prompt surgical repair is recommended in uncomplicated acute type A IMH. Medical therapy is recommended as the initial treatment in patients with uncomplicated acute type B IMH.
   • PAU. Urgent repair is recommended in patients with PAU and rupture; urgent repair is recommended for ascending aorta PAU with associated IMH; repair is recommended in patients with uncomplicated PAU and persistent pain.
   • Long-term surveillance imaging after AAS.
     • Acute aortic dissection and IMH. In patients with acute aortic dissection or IMH treated with open or endovascular repair and who have residual disease, and in patients managed only with medical therapy, CT (or MRI) is recommended after 1 month, 6 months, 12 months, and then annually if stable.
     • PAU. Imaging follow-up in patients with PAU who underwent repair should follow the same guidelines as for patients who underwent repair of TAA. In patients with PAU managed medically, CT is reasonable after 1 month and, if stable, every 6 months for 2 years; and then based on patient age and PAU characteristics.
8. Pregnancy in patients with aortopathy:
   • In patients with syndromic HTAD, nsHTAD, Turner syndrome, BAV with aortic dilation, or another aortopathy, both counseling and aortic imaging (with TTE or CT or MRI) is recommended before pregnancy.
   • Surgery prior to pregnancy is recommended in patients with MFS and aortic root diameter >4.5 cm, nsHTAD and aortic diameter ≥4.5 cm, BAV and aortic diameter ≥5.0 cm, Turner syndrome and ASI ≥2.5 cm/m², and sporadic aortic root or ascending aorta aneurysm diameter ≥5.0 cm. Surgery prior to pregnancy is reasonable in patients with LDS attributable to pathologic variants in TGFB2 or TGFB3 and aortic diameter ≥4.5 cm.
   • During pregnancy, patients with aortic aneurysm or increased risk of dissection should be managed by a multidisciplinary team including a maternal fetal medicine specialist and a cardiologist; and hypertension should be treated using guideline-directed therapy. Among patients with syndromic HTAD or nsHTAD, beta-blocker therapy is recommended during therapy and post-partum. Surveillance TTE is recommended during each trimester and several weeks post-partum; MRI without gadolinium is recommended over CT among patients in whom surveillance imaging of the arch, descending thoracic aorta, or abdominal aorta is required.
   • Delivery by cesarean section is recommended in patients with chronic aortic dissection, and is reasonable in patients with aortic root or ascending aorta aneurysm diameter ≥4.5 cm; if otherwise appropriate, vaginal delivery is recommended in patients with aortopathy and aortic diameter <4.0 cm.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Dyslipidemia, 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, Nonstatins, Novel Agents, Statins, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Angiography, Computed Tomography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging

Keywords: Adrenergic beta-Antagonists, 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, Hydroxymethylglutaryl-CoA Reductase Inhibitors, Loeys-Dietz Syndrome, Magnetic Resonance Angiography, Magnetic Resonance Imaging, Interventional, Marfan Syndrome, Practice Guideline, Pregnancy, Tomography, Emission-Computed, Ulcer, Ultrasonography, Interventional, Vascular Diseases

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