Open BAV

The opening of a BAV resembles the open mouth of a fish, leading to the term “fish mouth” valve.

BAV and Families - Look for the Bulge! Bicuspid Aortic Valve is A Red Flag for All Family Members Including Those With Normal Aortic Valves Blood relatives of someone with a bicuspid aortic valve may have other aspects of this condition, even though their aortic valve has three normal-appearing leaflets. The followings study of the risk of thoracic aortic disease in BAV family members with normal-appearing aortic valves was published in 2007 by several geneticists: Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance, Loscalzo ML, Goh DL, Loeys B, Kent KC, Spevak PJ, Dietz HC, Am J Med Genet A. 2007 Sep 1;143A(17):1960-7 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. The authors wrote that a single defective gene may cause these abnormalities of the valve and aorta independently of each other. The Bicuspid Aortic Foundation has heard from families with a variety of manifestations of bicuspid aortic disease. For example, in one family, two daughters had clearly identifiable bicuspid aortic valves and ascending aortic aneurysms (one of the daughters had a brain aneurysm also). The mother was told she had a normal, tri-leaflet aortic valve. However, the mother has an ascending aortic aneurysm. Much more study and research is needed to understand the complexity of families where bicuspid aortic valves are found. Learning must come from these families themselves, under the careful observation and guidance of physicians. What these BAV families are teaching us now, is that having a normal-appearing aortic valve is not enough to insure safety. It is still possible to be at risk for aneurysm and dissection.

Closed BAV

When closed, it may be difficult to distinguish a BAV from an aortic valve with all three leaflets.

Tricuspid Aortic Valve

Closed TAV

A normal aortic valve has three leaflets or cusps. Pictured here is a TAV with the leaflets closed. The opening of a TAV is shaped like a triangle.