Look for the Bulge!

Ascending Aortic

Aneurysms in BAV

Families

In 2007, a paper was published about 13 families with BAV: “Familial thoracic aortic dilation and bicommissural aortic valve: A prospective analysis of natural history and inheritance”. It is important to note in this paper that in all 13 families, at least one person had an ascending aortic aneurysm with a three- leaflet aortic valve (considered normal). Dissection or rupture occurred in some of those tri-leaflet valve individuals.

Bicuspid Aortic Valves are Present at Birth

The Most Common Birth Defect in the Heart

A “Fish Mouth” Aortic Valve

If you were born with a bicuspid aortic valve, you are not alone. Better statistics are needed, but it is estimated that about 1 in 50 people are born with a bicuspid aortic valve. In the United States, a child is born with BAV every 7 minutes. A bicuspid aortic valve is an aortic valve with only two leaflets instead of three. Current clinical and scientific studies are revealing that bicuspid aortic disease is not a simple valve condition. It increasingly appears to be a genetically based connective tissue disorder. There are other malformations of the aortic valve leaflets also, which are more rare. Sometimes the leaflet is just a circle of tissue - a unicuspid valve. Four leaflets have also been found - a quadricuspid aortic valve. Sometimes the fusion of the leaflets is so slight it is hard to detect. However, any malformation of the aortic valve leaflets is abnormal, however slight. As a genetic condition, it may affect multiple family members. Some affected family members have bicuspid aortic valves, and some have normal-appearing trileaflet aortic valves. As highlighted in the information on the right, regardless of the number of aortic valve leaflets, all family members should be checked periodically to avoid serious, potentially life-threatening complications of the aorta and vessels of the head and neck.

An estimated 1 in 50

people are born with

BAV

It is very likely that you know someone with BAV. It could even exist in your family or extended family without you knowing about it. A child is born with BAV every 7 minutes in the United States.