Bicuspid Aortic Valve

• Bicuspid aortic valve (BAV) disease is the most common congenital cardiac defect.
  • Affects 0.5% to 2.0% of adults
  • 3:1 male-to-female predominance, or as Dr. Skolnick put it “70% are men.”
• While the BAV can be found in isolation, it is often associated with other congenital cardiac lesions.¹
  • Dilation of the proximal ascending aorta secondary to abnormalities of the aortic media (most common)
    • Aortic aneurysms is 20% to 40%
    • Aortopathy is independent of valve function and consists of dilation of the aortic sinuses, the ascending aorta, or the arch.
    • ∴, patients with BAV require careful evaluation of the aorta throughout their lifetimes
• Per Dr. Magalski:
  • If mom has it → 6% chance kid has it.
  • If dad has it → 3% chance for kid.
• Important clinical implications
  • Valve dysfunction
  • aortic dilatation
  • aortic dissection
  • endocarditis
• Despite the potential complications, 2 large contemporary series have demonstrated that life expectancy in adults with BAV disease is not shortened when compared with the general population.¹
• Turner syndrome
  • BAV prevlance is ~30% in women with Turner syndrome; is 30-60x more frequent in women with Turner syndrome than in women with 46,XX.
  • By contrast, 7-18% of women with Turner syndrome have aortic coarctation.

Management

• Many undergoing aortic valve replacement will also need aortic root surgery b/c BAV is a disease of both the aortic valve and aorta ¹

Screening

• For patients with BAV, you should (at least once) scan entire aorta to screen for aortopathy with an MRA or CT
• Screen first-degree relatives for BAV or aortopathy with a TTE

Surveillance

Aortic Dilatation in BAV

• Prophylactic ascending aorta replacement is indicated when
  • Aorta ≥ 5.5 cm
  • Aorta ≥ 5 cm
    • if high risk features also present: uncontrolled hypertension, personal history of coarctation, family hx of aortic dissection or early unexplained SCD, and confirmed aortic diameter increase of > 0.3 cm/yr
  • Aorta ≥ 4.5 cm
    • if already going for aortic valve replacement (AVR) for another indicated reason, e.g. for AR or AS
    • to prevent progressive dilatation of the aorta after you replace the valve

Footnotes

1. Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. Journal of the American College of Cardiology. 2010;55(25):2789-2800. doi:10.1016/j.jacc.2009.12.068 ↩ ↩² ↩³