Bicuspid Aortic Valve (BAV) — The #1 Congenital Heart Defect
About 1–2% of people are born with only two aortic valve leaflets instead of three. This bicuspid valve works fine for decades, but tends to calcify and stiffen in the early 50s — earlier than most patients or their doctors expect. I have lost count of how many patients I've seen who were told for years, “it's just a murmur,” only to finally get a proper echo and discover significant aortic stenosis from an undiagnosed bicuspid valve.
BAV is also an aorta problem, not just a valve problem. Up to 80% of BAV patients have some degree of aortic root or ascending aorta dilation. The 2022 ACC/AHA guidelines recommend lifelong aortic surveillance and screening of all first-degree relatives. An ascending aorta ≥4.5 cm in a BAV patient requires urgent specialist evaluation.