What is the management approach for aortopathies in patients with bicuspid aortic valves (BAV)?

Management of Aortopathies in Bicuspid Aortic Valve Patients

Patients with bicuspid aortic valve (BAV) require lifelong surveillance and specific management of associated aortopathy, with surgical intervention recommended when aortic diameter reaches ≥55 mm in most cases, or ≥50 mm with additional risk factors. 1

Diagnostic Approach

Initial Evaluation

• Transthoracic echocardiography (TTE) is the primary imaging modality for initial assessment of BAV and aortic dimensions 1
• Cardiac MRI or CT is indicated when:
  • Aortic root/ascending aorta cannot be adequately assessed by TTE 1
  • At first diagnosis of BAV 1
  • When aortic diameter exceeds 45 mm 1
  • When discrepancies in measurements are found between subsequent TTE controls 1

Family Screening

• Screening of first-degree relatives (parents, siblings, children) of BAV patients is recommended, especially those with root phenotype aortopathy 1, 2
• Family screening has been shown to be cost-effective, with 9-20% of relatives having BAV 1

Surveillance Protocol

For BAV Patients with Aortic Dilation

• For aortic diameter >45 mm or growth rate >3 mm/year:

  • Annual imaging is indicated 1
  • Confirmation with CT or MRI when diameter >50 mm 1

• For aortic diameter >40 mm:

  • Serial imaging by TTE recommended after 1 year, then every 2-3 years if stability is observed 1
  • This applies to both patients without surgical indications and those who have undergone isolated aortic valve surgery 1

• For patients with previous AVR and aortic diameter ≥4.0 cm:

  • Lifelong surveillance imaging is recommended 1
  • Interval determined by aortic diameter and rate of growth 1

Surgical Management

Primary Indications for Aortic Replacement

1. Strong recommendation for surgery (Class I):

   • Aortic diameter >55 mm in asymptomatic or symptomatic patients 1
   • Aortic diameter >50 mm in patients with root phenotype aortopathy 1
   • Aortic diameter >45 mm when surgical aortic valve replacement is scheduled 1

2. Reasonable to consider surgery (Class IIa):

   • Aortic diameter 50-55 mm with additional risk factors 1:
     • Family history of aortic dissection
     • Aortic growth rate >3 mm per year (or >0.5 cm per year)
     • Aortic coarctation
     • Systemic hypertension
     • Age <50 years
     • Short stature
     • Ascending aortic length ≥11 cm
     • Resistant hypertension
     • Desire for pregnancy

3. May consider surgery (Class IIb):

   • Aortic diameter 50-55 mm without additional risk factors in low surgical risk patients 1
   • Valve-sparing surgery in patients meeting criteria for aortic sinus replacement 1

Medical Management

• Optimal blood pressure control targeting <140/90 mmHg 2
• Beta-blockers are preferred first-line agents for blood pressure control 2
• Regular moderate aerobic exercise is recommended 2
• Avoidance of strenuous isometric exercise, contact sports, and competitive sports (especially with borderline aortic diameters) 2

Special Considerations

Post-Surgical Follow-up

• Continued lifelong surveillance is essential even after aortic valve replacement 1
• Progressive aortic enlargement occurs in approximately 10% of patients after isolated AVR 1
• Subsequent aortic dissection occurs in about 1% of patients after isolated AVR 1

Risk Stratification

• Root phenotype and predominant aortic regurgitation are associated with greater risk of aortic growth and complications 1
• Patients with BAV who underwent AVR for aortic regurgitation have higher risk for late aortic events than those who underwent AVR for aortic stenosis 1

Common Pitfalls and Caveats

1. Measurement consistency: Use the same imaging modality for serial measurements to ensure accurate comparison 2

2. Continued surveillance after valve surgery: The aorta may continue to dilate even after isolated AVR 1

3. Phenotype matters: Root phenotype and aortic regurgitation are associated with higher risk of aortic complications 1

4. Comprehensive assessment: Evaluate the entire thoracic aorta, not just the ascending portion, as BAV aortopathy can affect multiple segments 1

5. Surgical expertise: Complex aortic surgeries should be performed at Comprehensive Valve Centers with experienced surgeons 1