Bicuspid Aortic Valve Management

Diagnostic Imaging for BAV

The European Society of Cardiology recommends initial screening with transthoracic echocardiography (TTE) to evaluate valve morphology, measure severity of aortic stenosis/regurgitation, and assess the shape and diameter of the aortic sinuses and ascending aorta in patients with known or suspected bicuspid aortic valve 1, 2, 3
When a BAV is first diagnosed, initial TTE to assess diameters of the aorta at several levels is recommended by the European Heart Journal 1, 4
Cardiac MRI or CT is indicated in patients with BAV when the morphology of the aortic root and ascending aorta cannot be accurately or fully assessed by TTE, as recommended by the European Heart Journal and Circulation 1, 2, 3
The European Heart Journal recommends CCT or CMR of the entire thoracic aorta 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, 4
MRI imaging is preferred over CT when possible due to absence of ionizing radiation exposure in patients who will likely require multiple imaging studies over their lifetime, as recommended by Circulation 2, 5

For patients with BAV without significant aortic dilation, the American Heart Association recommends reimaging approximately every 2 years for aortic diameter <40 mm 6, 7
For patients with BAV with aortic diameter >40 mm, annual measurement of aortic diameter is indicated, as recommended by the European Heart Journal 1, 4
For patients with BAV with aortic diameter >45 mm or an increase of >3 mm per year, annual measurement of aortic diameter is indicated, as recommended by the European Heart Journal 1, 4

The American Heart Association recommends echocardiographic screening for the presence of BAV in first-degree relatives of patients with BAV 6, 7
The European Heart Journal recommends screening by TTE in first-degree relatives of BAV patients with root phenotype aortopathy and/or isolated aortic regurgitation 4

The European Society of Cardiology recommends surgery when the maximum aortic diameter is ≥55 mm 4
Surgery is recommended when aortic root or ascending aortic diameter >50 mm in the presence of risk factors, as recommended by the European Heart Journal 1, 4
The European Heart Journal recommends considering surgery at a maximum diameter ≥50 mm if any of the following exist: age <50 years, short stature, ascending aortic length ≥11 cm, aortic diameter growth rate >3 mm per year, family history of acute aortic syndrome, aortic coarctation, resistant hypertension, concomitant non-aortic-valve cardiac surgery, or desire for pregnancy 1, 4

The American Heart Association recommends counseling women with BAV and ascending aorta diameter greater than 4.5 cm about the high risks of pregnancy 6, 7
The European Heart Journal recommends prophylactic aortic root surgery in women desiring pregnancy with aortic diameters >45 mm 4
Patients with moderate to severe aortic stenosis should be counseled against participation in competitive athletics and strenuous isometric exercise, as recommended by the American Heart Association 6, 7
Physical activity should be individualized based on aortic diameter, family history of aortic dissection, and pre-existing fitness, as recommended by the European Heart Journal 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, as reported in Circulation 2, 3
Patients with BAV require lifelong cardiology follow-up, even after intervention, as they may develop progressive or recurrent aortic stenosis, regurgitation, or aortic enlargement, as recommended by the American Heart Association 6, 7