Pregnancy in women with bicuspid aortic valve

Abstract Background Aortic dissection during pregnancy is uncommon, however, the risk of aortic dissection is increased if there is underlying aortopathy. Bicuspid aortic valve (BAV) is common in the general population and is associated with the presence of an aortic aneurysm, but this condition is mostly asymptomatic and ignored in women of childbearing age. Data on pregnancy in patients with BAV are scarce, and guidelines on this topic are based on the consensus opinion of experts. The risk of occurrence of aortic dissection as a function of aortic diameter during pregnancy remains poorly known in women with BAV. Purpose To investigate demographic and echocardiographic characteristics and aortic events associated with pregnancy in women with BAV and to estimate ascending aortic diameter at the time of pregnancy. Methods We performed a retrospective study using data from our tertiary centre. All women seen at our centre between 1996 and 2020 with BAV, at least 1 pregnancy, and no genetic syndrome were included. We have collected data from echocardiograms performed in and out of our centre and aortic events. Assuming from the literature an annual aortic dilation rate of 0.2 mm at the sinus of Valsalva and 0.4 mm at the tubular ascending aorta, we estimated ascending aortic size and Z-score at the time of pregnancy. Results We identified 47 women with BAV with occurrence of 103 pregnancies. The median age of BAV diagnosis was 43 years. The aorta was measured at a median of 13.3 years since the last delivery. At BAV diagnosis, the median largest ascending aortic diameter was 44mm, and the median Z-score was +4.3. Ascending aortic diameter was ≥40mm in 37/47 (79%) and Z-score ≥2 in 44/47 (94%). No aortic dissection was observed during pregnancy and postpartum in all 103 pregnancies. At the time of pregnancy, the estimated median diameter of the ascending aorta was 37mm and the estimated median Z-score was +3.3. The largest aortic diameter during pregnancy was estimated to be ≥40mm in 36/103 pregnancies, ≥45mm in 13/103, and ≥50mm in 1/103; Z-score was estimated to be ≥2 in 81/103 and ≥4 in 40/103. Type A aortic dissection occurred in 1 woman, 13 years after pregnancy, and type B aortic dissection in 1 woman, 14 years after pregnancy. Planned surgery was performed in 8 women at a median of 17.5 years after the last pregnancy: 1 isolated aortic valve replacement and 7 prophylactic aortic surgeries associated with aortic valve surgery. Conclusions In our populat