Introduction:
Bicuspid aortic valve (BAV) is the most common congenital heart disease,
with a prevalence of 0.5-1-2% and a slight male
predominance1-5. Many patients with BAV are
asymptomatic and often present in adulthood as an incidental finding on
echocardiogram. While survival in adult patients with BAV may not differ
significantly from that of the general population (potentially due to
the efficacy of AVR and similar interventions)3,6-8,
patients with BAV are at an increased risk for various aortic
pathologies including aortic stenosis (AS), aortic regurgitation (AR),
aortic root dilation, aortic aneurysm, and aortic
dissection1,3,9. A systematic review of 11,000
patients during 2-12 years of follow-up reported that aortic aneurisms
were presents in 20-40% of patients with BAV, though less than 0.5%
suffered a dissection5. Other studies report that up
to 84% of patients with BAV may eventually develop an aortic aneurysm
over the course of their lifetime, though less than 5% will have an
aortic dissection10,11. The risk of various aortic
pathologies has prompted guidelines to recommend surveillance of
patients with BAV to guide timely intervention, but how closely these
recommendations are being followed remains unknown.
The 2018 American Association for Thoracic Surgery (AATS) guidelines for
the management of BAV recommend serial evaluations of the aorta by
transthoracic echocardiogram (TTE) with intervals tailored to the
presence and severity of aortic dilation9. The 2020
American College of Cardiology/American Heart Association (ACC/AHA)
guidelines suggest lifelong surveillance in patients with aortic
dilation ≥4.0cm, and MRI or CT evaluation of difficult to assess
structures12. Additionally, surgery is often based on
the severity of valvular pathologies7, for which the
American Society of Echocardiography gives specific guidelines to assess
and categorize13. Overall, all major cardiology/
cardiac surgery societies recommend careful clinical and imaging
surveillance in BAV patients.
Although there is consensus on the necessity to carefully follow these
patients, it is unknown how well current guidelines are adopted into
clinical practice for incidentally detected BAV. Therefore, we aimed to
understand the extent of clinical gap in implementation of
guidelines-based surveillance for BAV patients at a tertiary care health
system.
Materials and Methods: