Management of Bicuspid Aortic Stenosis

Bicuspid aortic valve (BAV) disease is the most common congenital heart lesion affecting 1 to 2% of the population. BAV leads to premature leaflet degeneration, increasing the risk of aortic stenosis (AS) and aortic regurgitation (AR), and is associated with increased risk of endocarditis and ascending aorta pathologies including aneurysm and dissection. Patients with BAV experience a 70% lifetime risk of aortic valve surgery, often at a younger age than those with a trileaflet anatomy. BAV is present in up to 40% of patients undergoing surgical aortic valve replacement (SAVR), which remains the standard of care in low-risk patients. Transcatheter aortic valve implantation (TAVI) has emerged as a less invasive treatment alternative with favorable short- and mid-term outcomes in appropriately selected BAV patients. Randomized controlled trials (RCTs) comparing TAVI and SAVR have systematically excluded BAV patients, except for the UK TAVI trial and recent NOTION-2 trial, the latter signalling a potentially increased risk of all-cause mortality, stroke, and re-hospitalization rates at 1 year among low-risk BAV patients undergoing TAVI. Dedicated RCTs comparing TAVI and SAVR for BAV stenosis are in preparation and will inform future guidelines. Heart Teams need to individualize treatment plans based on periprocedural risk, comorbidities, aortic root anatomy, and life expectancy. The aim of the current article is to review and summarize those issues frequently associated with BAV and how they affect treatment decision pathways, with a particular focus on SAVR vs. TAVI in BAV patients presenting with aortic stenosis.