Thoracic aortic aneurysm medical therapy

Thoracic aortic aneurysm Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3] Mohammad Salih, MD. Hibatullah Abdul Aleem, M.B.B.S[4]

Overview

Medical therapy for patients with a thoracic aortic aneurysm includes aggressive blood pressure control targeting a goal of 130/80 mmHg for all patients, heart rate reduction with beta-adrenergic blocking agents, lipid management, and smoking cessation. For patients with heritable thoracic aortic disease (HTAD), including Marfan syndrome, Loeys-Dietz syndrome, and bicuspid aortic valve-associated aortopathy, gene-specific pharmacotherapy is a cornerstone of management. Medical therapy is continued in parallel with serial surveillance imaging and remains the primary treatment strategy until aneurysm size or growth rate reaches thresholds warranting surgical or endovascular intervention.

Medical Therapy

Initial Resuscitation

• Airway and breathing: Secure the airway and provide oxygenation/ventilation as needed.
• Vascular access: Place two large-bore IV catheters for fluids, medications, and possible transfusion.
• Urine output: Insert a Foley catheter and monitor hourly urine output as a marker of renal/end-organ perfusion.

Emergency Care

• Aggressive blood pressure and heart rate control
  • Target systolic blood pressure 100 to 120 mmHg and heart rate less than 60 bpm
  • IV esmolol (preferred first-line titratable agent)
  • IV labetalol (combined alpha/beta blockade)
  • IV sodium nitroprusside (adjunct vasodilator; must not be used without concurrent beta-blocker due to reflex tachycardia)
• IV opioid analgesia for pain control as needed
• Avoid aggressive fluid resuscitation in the absence of hypotension
• Immediate cardiac surgery consultation

Medications

• Antihypertensives
  • Beta-adrenergic blocking agents (first-line): atenolol, metoprolol succinate, propranolol
  • Angiotensin receptor blockers (ARBs): losartan, telmisartan
  • ACE inhibitors: acceptable alternative to ARBs in non-HTAD patients
  • Calcium channel blockers or thiazide diuretics: adjunct agents if blood pressure target not achieved
• Statins: high-intensity statin therapy for atherosclerotic TAA

2022 ACC/AHA Guidelines for the Diagnosis and Management of Aortic Disease

Multidisciplinary Aortic Team and Shared Decision-Making

Class I

1. Patients with thoracic aortic aneurysm approaching surgical thresholds, those with heritable thoracic aortic disease, or those requiring complex aortic interventions should be referred to a Multidisciplinary Aortic Team at a high-volume aortic center for evaluation and management. (Level of Evidence: B)

2. Shared decision-making between the patient, the multidisciplinary aortic team, and the referring clinician is recommended when discussing the timing of surgical intervention, type of repair, and the risks and benefits of medical versus surgical management. (Level of Evidence: C)

Class IIb

1. In patients with sporadic ascending aortic aneurysm who meet additional risk criteria (e.g., rapid growth, family history, or female sex with smaller body size), surgical repair at a maximum aortic diameter of ≥5.0 cm may be reasonable when performed at a high-volume aortic center with established expertise. (Level of Evidence: B)

Medical Treatment of Patients With Thoracic Aortic Disease

Class I

1. Stringent control of hypertension, lipid profile optimization, smoking cessation, and other atherosclerosis risk-reduction measures should be instituted for all patients with thoracic aortic aneurysm not requiring immediate surgical intervention, as well as for patients who are not considered surgical or stent graft candidates. (Level of Evidence: C)

Blood Pressure and Heart Rate Control

Class I

1. Antihypertensive therapy is recommended for all patients with thoracic aortic disease to achieve a blood pressure goal of ≤130/80 mmHg, regardless of the presence of diabetes or chronic kidney disease, to reduce the risk of aortic growth, dissection, stroke, myocardial infarction, heart failure, and cardiovascular death. (Level of Evidence: B)

2. Beta-adrenergic blocking agents are recommended as the preferred first-line antihypertensive drug class in patients with thoracic aortic aneurysm to reduce aortic wall stress through reduction of both blood pressure and the rate of pressure rise (dP/dt). (Level of Evidence: B)

3. Beta-adrenergic blocking drugs are recommended for all patients with Marfan syndrome and aortic aneurysm to reduce the rate of aortic dilatation unless contraindicated. (Level of Evidence: B)

4. Angiotensin receptor blockers are recommended for patients with Marfan syndrome and aortic aneurysm as an alternative or additive therapy to beta-adrenergic blocking agents to reduce the rate of aortic dilatation unless contraindicated. (Level of Evidence: B)

Class IIa

1. For patients with thoracic aortic aneurysm, it is reasonable to reduce blood pressure and heart rate with beta-adrenergic blocking agents and/or ACE inhibitors or angiotensin receptor blockers, titrated to the lowest tolerated levels without adverse effects. (Level of Evidence: B)

Class III (Harm)

1. ACE inhibitors and angiotensin receptor blockers are contraindicated during pregnancy in patients with heritable thoracic aortic disease due to teratogenicity; beta-adrenergic blocking agents (labetalol or metoprolol) are the preferred antihypertensive agents in this setting. (Level of Evidence: C)

Medical Management of Heritable Thoracic Aortic Disease

Class I

1. Genetic testing is recommended for patients with aortic root or ascending aortic aneurysm presenting at age <60 years and for any patient with a family history of thoracic aortic disease or sudden death, to guide diagnosis, surgical timing, and family screening. (Level of Evidence: B)

2. Genetic counseling and cascade screening of first-degree relatives are recommended for patients with a confirmed pathogenic or likely pathogenic variant in a gene associated with heritable thoracic aortic disease. (Level of Evidence: B)

3. Gene-specific surgical thresholds should be applied for patients with confirmed pathogenic variants (e.g., TGFBR1, TGFBR2, SMAD3, ACTA2, MYH11), as the risk of aortic dissection varies substantially by genotype. (Level of Evidence: B)

Class IIa

1. In patients with Loeys-Dietz syndrome and aortic aneurysm, it is reasonable to treat with angiotensin receptor blockers to reduce the rate of aortic dilatation. (Level of Evidence: C)

2. In patients with bicuspid aortic valve-associated aortopathy and aortic aneurysm, it is reasonable to treat with beta-adrenergic blocking agents or angiotensin receptor blockers to reduce blood pressure and slow aortic dilatation. (Level of Evidence: C)

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Dyslipidemia Treatment

Class IIa

1. Treatment with a statin is reasonable for patients with atherosclerotic thoracic aortic aneurysm or aortic arch atheroma to reduce cardiovascular risk and the risk of stroke. (Level of Evidence: B)

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Smoking Cessation

Class I

1. Smoking cessation and avoidance of exposure to environmental tobacco smoke at work and home are recommended for all patients with thoracic aortic disease. Follow-up, referral to special programs, and/or pharmacotherapy (including nicotine replacement, bupropion, or varenicline) is useful, as is adopting a stepwise strategy aimed at smoking cessation (the 5 As: Ask, Advise, Assess, Assist, and Arrange). (Level of Evidence: B)

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Surveillance Imaging During Medical Management

Class I

1. In patients with known thoracic aortic aneurysm managed medically, cross-sectional imaging with CTA or MRA of the entire aorta is recommended at the time of initial diagnosis to establish baseline aortic dimensions using a standardized measurement technique. (Level of Evidence: B)

2. Repeat aortic imaging is recommended at 6 months after the initial study to assess the rate of aortic growth before establishing a long-term surveillance interval. (Level of Evidence: B)

3. Annual surveillance imaging is recommended for patients with stable thoracic aortic aneurysm to detect interval growth that may warrant surgical referral. (Level of Evidence: B)

Class IIa

1. MRA is reasonable to use in preference to CTA for serial surveillance of thoracic aortic aneurysm when feasible, to minimize cumulative radiation exposure, particularly in younger patients and those with heritable aortopathies. (Level of Evidence: C)

2. Surveillance imaging every 3 to 6 months is reasonable for patients with aortic aneurysms approaching the surgical threshold size or with a prior documented growth rate of ≥0.5 cm per year. (Level of Evidence: C)

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Takayasu Arteritis and Giant Cell Arteritis

Class I

1. Initial therapy for active Takayasu arteritis and active giant cell arteritis should be corticosteroids at a high dose (prednisone 40 to 60 mg daily at initiation or its equivalent) to reduce the active inflammatory state. (Level of Evidence: B)

2. The success of treatment of patients with Takayasu arteritis and giant cell arteritis should be periodically evaluated to determine disease activity by repeated physical examination and measurement of either an erythrocyte sedimentation rate or C-reactive protein level. (Level of Evidence: B)

3. Elective revascularization of patients with Takayasu arteritis and giant cell arteritis should be delayed until the acute inflammatory state is treated and quiescent. (Level of Evidence: B)

Class IIa

1. It is reasonable to add a steroid-sparing immunosuppressive agent (e.g., methotrexate, azathioprine, or mycophenolate mofetil) in patients with Takayasu arteritis receiving corticosteroids who demonstrate progression of vascular disease, recurrence of constitutional symptoms, or re-elevation of inflammatory markers. (Level of Evidence: C)

Class IIb

1. In patients with Takayasu arteritis refractory to or unable to tolerate corticosteroids, tumor necrosis factor inhibitors (e.g., infliximab or etanercept) or other biologic immunosuppressive agents may be considered to achieve and maintain disease remission. (Level of Evidence: C)

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Aortic Arch and Thoracic Aortic Atheroma and Atheroembolic Disease

Class IIa

1. Treatment with a statin is a reasonable option for patients with aortic arch atheroma to reduce the risk of stroke and systemic embolism. (Level of Evidence: B)

Class IIb

1. Antiplatelet therapy or oral anticoagulation with warfarin (INR 2.0 to 3.0) may be considered in patients with stroke attributable to aortic arch atheroma ≥4 mm in thickness to prevent recurrent stroke. (Level of Evidence: C)