What is the recommended follow-up and management plan for a patient with a bicuspid aortic valve?

Follow-up and Management Plan for Bicuspid Aortic Valve

Patients with bicuspid aortic valve (BAV) require lifelong cardiology follow-up with regular imaging surveillance, as they may develop progressive or recurrent aortic stenosis, regurgitation, or aortic enlargement.

Initial Diagnostic Evaluation

• When a BAV is first diagnosed, initial transthoracic echocardiography (TTE) is recommended to assess diameters of the aorta at several levels 1, 2
• Cardiac MRI or CT of the entire thoracic aorta is recommended at first diagnosis, particularly when the morphology of the aortic root and ascending aorta cannot be accurately assessed by TTE 1, 2
• MRI is preferred over CT when possible due to absence of ionizing radiation exposure in patients who will require multiple imaging studies over their lifetime 2, 3

Surveillance Imaging Recommendations

• For patients with BAV without significant aortic dilation (aortic diameter <40 mm), reimaging should be performed approximately every 2 years 1, 2
• For patients with BAV with aortic diameter ≥40 mm, annual imaging is recommended 1, 2
• For patients with BAV with maximum aortic diameter >40 mm, surveillance serial imaging by TTE is recommended after 1 year, then if stability is observed, every 2–3 years 1
• If the aortic diameter exceeds 45 mm or shows an increase of >3 mm per year, more frequent monitoring is indicated 2, 3

Family Screening

• Echocardiographic screening is recommended for first-degree relatives of patients with BAV 1, 2
• Screening by TTE is particularly important for first-degree relatives of BAV patients with root phenotype aortopathy and/or isolated aortic regurgitation 1

Surgical Management Thresholds

• Surgery for bicuspid aortopathy is recommended when the maximum aortic diameter is ≥55 mm 1
• Surgery for bicuspid aortopathy of the root phenotype is recommended when the maximum aortic diameter is ≥50 mm 1
• In patients with low surgical risk, surgery for bicuspid aortopathy of ascending phenotype should be considered when the maximum aortic diameter is >52 mm 1

Special Considerations

Pregnancy Planning

• Women with BAV and ascending aorta diameter greater than 4.5 cm should be counseled about the high risks of pregnancy 1, 2
• Prophylactic aortic root surgery is recommended in women desiring pregnancy with aortic diameters >45 mm 1

Physical Activity

• Physical activity should be individualized based on aortic diameter, family history of aortic dissection, and pre-existing fitness 1, 2
• Patients with moderate to severe aortic stenosis should be counseled against participation in competitive athletics and strenuous isometric exercise 1, 2

Clinical Pitfalls and Management Challenges

• Despite guidelines recommending regular follow-up, surveillance imaging is often sparse in clinical practice, with studies showing that only 72.7% of BAV patients receive follow-up by cardiovascular specialists 4
• Different BAV phenotypes may have different risks of aortic dilation, with fusion involving the noncoronary cusp associated with higher risk of ascending aorta dilation 2, 5
• Patients with BAV may be asymptomatic for long periods despite progressive valve dysfunction; therefore, regular monitoring is essential even in asymptomatic patients 6, 5
• Echocardiographic valve degeneration at diagnosis can help identify higher-risk patients who require more frequent assessment versus lower-risk patients who may need only episodic follow-up 6

Long-term Outcomes

• Asymptomatic patients with BAV and no or minimal hemodynamic abnormality have excellent long-term survival but incur frequent cardiovascular events, particularly with progressive valve dysfunction 6
• Twenty years after diagnosis, approximately 24% of patients require aortic valve surgery, 5% need ascending aortic surgery, and overall 42% experience cardiovascular medical or surgical events 6
• No aortic dissection occurred in a 20-year community follow-up study, suggesting that with proper surveillance, catastrophic complications can be prevented 6