What monitoring is recommended for patients with an asymptomatic bicuspid aortic valve (BAV)?

From the Guidelines

Patients with an asymptomatic bicuspid aortic valve (BAV) should undergo initial transthoracic echocardiogram (TTE) to assess diameters of the aorta at several levels, and subsequent surveillance serial imaging by TTE is recommended in BAV patients with a maximum aortic diameter >40 mm, either with no indication for surgery or after isolated aortic valve surgery, after 1 year, then if stability is observed, every 2–3 years 1.

Monitoring Recommendations

When a BAV is first diagnosed, the following monitoring is recommended:

• Initial TTE to assess diameters of the aorta at several levels 1
• CCT or CMR of the entire thoracic aorta is recommended at first diagnosis and when important discrepancies in measurements are found between subsequent TTE controls during surveillance, or when the diameter of the aorta exceeds 45 mm 1

Surveillance

Surveillance serial imaging by TTE is recommended in BAV patients with a maximum aortic diameter >40 mm, either with no indication for surgery or after isolated aortic valve surgery, after 1 year, then if stability is observed, every 2–3 years 1.

Additional Considerations

• Screening by TTE in first-degree relatives (FDRs) of BAV patients with root phenotype aortopathy and/or isolated aortic regurgitation is recommended 1
• Screening by TTE in FDRs of all BAV patients should be considered 1 The most recent and highest quality study 1 provides the basis for these recommendations, prioritizing morbidity, mortality, and quality of life as the outcome.

From the Research

Monitoring for Asymptomatic Bicuspid Aortic Valve (BAV)

• Patients with asymptomatic BAV should undergo regular monitoring to assess the progression of valve dysfunction and aortic dilatation 2, 3.
• The monitoring should include:
  • Yearly surveillance transthoracic echocardiography to assess left ventricular function, jet velocity, gradient, and valve area 3.
  • Yearly clinical evaluation for change in symptom status or functional capacity 3.
  • Evaluation of the aorta with MRI or CT angiography at some point, as 50% of BAV patients have aortic root involvement 3.
  • Cardiac MRI is preferred unless there is a contraindication, particularly in younger patients, given the cumulative radiation exposure from surveillance CT scans 3.
• For patients with no aortic dilatation, echocardiography can be used for follow-up 3.
• For patients with mild aortic dilation, surveillance aortic imaging is usually performed every 3-5 years 3.
• For patients with greater degrees of aortic dilation (aortic diameters >4.0 cm) or notable interval change in dimensions, aortic imaging should be performed every year 3.
• NT-pro BNP levels can be used to help assess patients, particularly those in whom the anatomic severity does not match the clinical symptoms 3.

Predictors of Cardiovascular Events

• Independent predictors of cardiovascular events in patients with asymptomatic BAV include:
  • Age > 50 years 2.
  • Valve degeneration at diagnosis 2.
  • Baseline ascending aorta > 40 mm 2.
• These predictors can help identify higher-risk patients who require regular assessment and closer monitoring 2.

Familial Screening

• Familial screening is recommended for first-degree relatives of patients with BAV disease, as the condition is often familial and associated with dilatation of the aortic root 4.
• The screening should include aortic valve and root assessment by standard two-dimensional echocardiography 4.