What is the follow-up for a dilated aortic root?

Follow-up Protocol for Dilated Aortic Root

For patients with a dilated aortic root, follow-up should include regular imaging surveillance with frequency determined by aortic diameter, growth rate, and underlying etiology, with imaging intervals ranging from 6 months to 5 years depending on risk factors.

Initial Evaluation

• Transthoracic echocardiography (TTE) is recommended at diagnosis to assess aortic valve anatomy, function, aortic root, and ascending aorta diameters 1
• Cardiovascular computed tomography (CCT) or cardiovascular magnetic resonance (CMR) is recommended to confirm TTE measurements, rule out aortic asymmetry, and determine baseline diameters for follow-up 1
• Complete assessment of the entire aorta is recommended at baseline to identify additional aneurysms 1
• Assessment of aortic valve morphology (especially for bicuspid aortic valve) is essential 1

Follow-up Intervals Based on Aortic Diameter (Non-Heritable Disease)

For Aortic Root/Proximal Ascending Aorta:

• 40-44 mm: Baseline CCT/CMR and reimaging by TTE in one year 1, 2
• 45-49 mm: Confirm by CCT or CMR, then annual imaging 1, 2
• 50-54 mm: Imaging every 6 months 1
• ≥55 mm: Consider surgical intervention 1

Based on Growth Rate:

• ≥3 mm/year: More frequent monitoring (every 6 months) and consider intervention 1, 2
• <3 mm/year: Reimage 6-12 months later to define projected growth rate 1

Follow-up for Heritable Thoracic Aortic Disease

Marfan Syndrome:

• Aortic root <45 mm without risk factors: TTE at least annually 1
• Aortic root <45 mm with risk factors: TTE at least every 6 months 1
• Aortic root ≥45 mm: TTE every 6-12 months 1
• Complete vascular imaging (CMR/CCT) every 3-5 years if stable 1
• Post-aortic root replacement: Surveillance imaging at least every 3 years 1

Loeys-Dietz Syndrome:

• TTE at baseline and subsequently every 6-12 months, depending on aortic diameter and growth 1
• Baseline arterial imaging from head to pelvis with CMR or CCT 1
• Subsequent surveillance with CMR, CCT, or DUS every 1-3 years 1

ACTA2-Related Heritable Thoracic Aortic Disease:

• Annual monitoring of aortic root/ascending aorta with TTE 1
• Imaging of the entire aorta with CMR/CCT every 3-5 years 1

Imaging Modality Selection

• TTE is appropriate for aortic root and proximal ascending aorta follow-up 1
• TTE is not recommended for surveillance of aneurysms in the distal ascending aorta, aortic arch, or descending thoracic aorta 1
• CMR or CCT is recommended for surveillance of aneurysms at the distal ascending aorta, aortic arch, or descending thoracic aorta 1
• CMR is preferred over CCT for long-term follow-up in young patients to minimize radiation exposure 1, 2
• Follow-up should be conducted with the same imaging technique and at the same center for consistency 1

Medical Management During Surveillance

• Beta-blockers or angiotensin receptor blockers (ARBs) in maximally tolerated doses are recommended for patients with Marfan syndrome to reduce the rate of aortic dilatation 1
• Consider combination therapy with both beta-blockers and ARBs in Marfan syndrome 1
• Celiprolol should be considered in patients with vascular Ehlers-Danlos syndrome 1
• Optimize cardiovascular risk management for all patients with aortic aneurysms 1

Special Considerations

• Pregnancy: Women with Marfan syndrome desiring pregnancy should have pre-conception evaluation and imaging of the whole aorta 1
• Physical activity: Individualize physical activity recommendations based on aortic diameter, family history of aortic dissection, and pre-existing fitness 1
• Bicuspid aortic valve: Consider more frequent monitoring due to associated aortopathy 1
• Post-surgical follow-up: After open repair, early CCT within 1 month, then yearly for first 2 years, then every 5 years if stable 1

Common Pitfalls and Caveats

• TTE may underestimate aortic root size compared to CT measurements, especially as the diameter of the aortic root increases 3
• Echocardiography alone may not be sufficient for comprehensive assessment; cross-sectional imaging with CT or MRI is often necessary 1, 4
• Rapid expansion (≥3 mm per year) requires closer evaluation every 6 months 1
• Non-compliance with follow-up programs is associated with higher rupture rates and should be addressed 2
• Patients with generalized aortic root dilatation (versus localized to sinuses of Valsalva) may have more severe abnormalities of left ventricular size and function and higher rates of subsequent valve replacement 5

When to Consider Surgical Intervention

• Aortic root/ascending aorta diameter ≥55 mm in patients with tricuspid aortic valve 1
• Aortic root diameter ≥50 mm in patients with Marfan syndrome 1
• Aortic root diameter ≥45 mm in women with Marfan syndrome desiring pregnancy 1
• Aortic root diameter ≥45 mm (or lower with risk factors) in ACTA2-related heritable thoracic aortic disease 1
• Rapid expansion (≥10 mm/year or ≥5 mm/6 months) 2