Management of Bicuspid Aortic Valve and Aortic Root Dilation in Pregnancy

Risk Assessment

Bicuspid aortic valve is associated with aortic dilation in approximately 50% of patients, with the dilation often maximal in the distal part of the ascending aorta 1, 2
An aortic root diameter >4.0 cm confers approximately a 10% risk of dissection during pregnancy 3
A diameter of 4.0-4.4 cm is associated with an 89-fold increased risk of dissection compared to normal aortic diameter 3
Aortic dissection can occur in patients with BAV, although less frequently than in Marfan syndrome patients 1

The European Society of Cardiology recommends prophylactic aortic root surgery in women desiring pregnancy with aortic diameters >45 mm 4
For patients with BAV planning pregnancy with an aortic root diameter of 4.5 cm, prophylactic aortic root surgery should be considered 4, 3
Complete imaging of the entire aorta using cardiac MRI or CT angiography is essential before pregnancy to fully assess the extent of aortic dilation 4, 3

Frequent echocardiographic monitoring (every 4-12 weeks) throughout pregnancy and 6 months postpartum is recommended for patients with BAV and aortic root diameter of 4.5 cm who pursue pregnancy without surgery 1, 7, 5
Treatment with beta-blockers throughout pregnancy to reduce risk of dissection is recommended for patients with BAV and aortic root diameter of 4.5 cm who pursue pregnancy without surgery 1, 4
The decision for surgery should take into account family history of aortic dissection 5, growth rate (≥0.5 cm/year indicates higher risk) 6, 3, and body surface area (especially important in women of small stature) 1, 7

For patients with BAV not planning pregnancy, surgery for bicuspid aortopathy is recommended when the maximum aortic diameter is ≥50 mm 4
For women planning pregnancy, a lower threshold (>45 mm) should be considered 4, 3
In patients with low surgical risk, surgery for bicuspid aortopathy of ascending phenotype should be considered when the maximum aortic diameter is >52 mm 4

Lifelong beta-blocker therapy should be considered to reduce the rate of aortic dilation 1, 3
More frequent imaging (every 6-12 months) is recommended if there are additional risk factors or if significant growth is detected 3