Aortic Disease Management

Normal Aortic Diameter

• A normal ascending aorta diameter is within the range of 2.5-3.8 cm by transthoracic echocardiography (TTE) and <3.7 cm by transesophageal echocardiography (TEE) for most adults, with an ascending aorta diameter of 3.5 cm being within normal limits and not requiring intervention or immediate concern, as recommended by the European Society of Cardiology 1

Monitoring and Intervention

• Annual measurement is recommended for patients with a bicuspid aortic valve (BAV) and an ascending aorta diameter of 3.5 cm, with cardiac MRI or CT also being indicated if the aorta cannot be accurately assessed by TTE, according to the European Society of Cardiology 2, 3
• Intervention thresholds are ≥5.5 cm for the general population with a tricuspid valve, and ≥5.0 cm with risk factors for patients with a bicuspid aortic valve, as recommended by the American Heart Association 2, 4
• The following table outlines the surgical thresholds for different patient populations:

Aortic Growth Rate

• The normal expansion rate of the aorta is approximately 1-2 mm over 10 years, and an increase of >3 mm per year is considered rapid growth and requires more frequent monitoring, as stated by the European Society of Cardiology 1, 2
• The following table outlines the imaging frequency based on aortic diameter:

Imaging and Evaluation

• Initial imaging should include TTE with suprasternal notch acoustic windows, as recommended by the American Heart Association 8
• Complete evaluation requires at least one cardiovascular MRI or CT scan of the thoracic aorta and intracranial vessels, according to the American College of Cardiology 8
• Complete assessment of the entire aorta is recommended at baseline and during follow-up by the European Society of Cardiology 2
• Yearly imaging surveillance is recommended for patients with aortic diameter of both the sinus of Valsalva and ascending aorta ≥4.0 cm, using consistent imaging modalities such as Transthoracic echocardiography (TTE), Cardiac CT, and Cardiac MRI for accurate comparison over time 4

Medical Management

• Beta blockers should be used to reduce aortic wall stress as first-line therapy, with a target heart rate ≤60 bpm, and if systolic blood pressure remains >120 mmHg after adequate heart rate control, add vasodilators (ACE inhibitors) 9
• Blood pressure control should target <140/90 mmHg, with beta-blockers as first-line therapy for blood pressure control in patients with aortic dilation, and lipid management should target LDL-C <1.4 mmol/L (<55 mg/dL) 2
• Optimal cardiovascular risk management to reduce major adverse cardiovascular events is recommended, including blood pressure control, beta-blockers, and lipid management 2, 4
• Regular moderate aerobic exercise is recommended, but avoid strenuous isometric exercise and contact sports, and smoking cessation is strongly advised, and weight management is recommended to maintain a healthy body weight and optimize cardiovascular health 2, 10

Surgical Management

• Open surgical replacement is recommended at an arch diameter ≥5.5 cm for asymptomatic patients, as suggested by the American College of Cardiology 4
• Symptomatic patients with low/intermediate operative risk should undergo immediate open surgical replacement, according to the American Heart Association 4
• High-risk patients should consider a hybrid or endovascular approach, as recommended by the American College of Cardiology 4
• Surgical intervention is indicated if the aortic diameter reaches ≥5.0 cm, growth rate ≥0.5 cm in one year, or development of symptoms attributable to aortic pathology, as suggested by the American Heart Association 3

Special Populations

• Patients with risk factors such as bicuspid aortic valve, family history of aortic dissection, genetic syndromes (Marfan, Loeys-Dietz), hypertension, and smoking require more aggressive management 4, 6
• The European Society of Cardiology advises caesarean delivery if aortic root diameter is ≥4.5 cm 11
• Joint cardiac and obstetric management with regular echocardiograms before, during, and after pregnancy is recommended 11
• Patients with Ehlers-Danlos syndrome require close monitoring as aortic dissection may occur without dilatation 11
• Patients with Loeys-Dietz syndrome may experience dissection at smaller aortic diameters, requiring more aggressive management 4, 5
• Patients with Marfan syndrome require surgical intervention at a diameter of 4.0-5.0 cm, and patients with Loeys-Dietz syndrome require surgical intervention at an ascending aorta diameter of ≥4.2 cm (internal diameter) 9, 4

Follow-up and Screening

• Lifelong cardiology follow-up is required for all patients with aortic coarctation (repaired or not), as emphasized by the American Heart Association 8
• Post-repair evaluation should be done at least yearly, according to the American College of Cardiology 8
• Late postoperative thoracic aortic imaging is necessary to assess for aortic dilatation or aneurysm formation, as recommended by the American Heart Association 8
• MRI/CT evaluation of the repair site should be done at intervals of 5 years or less, as suggested by the American College of Cardiology 8
• Screening of family members is recommended for patients with bicuspid aortic valve, according to the American Heart Association 12