Joseph M. Krepp1, Mary J. Roman2, Richard B. Devereux2, Adrienne Bruce1, Siddharth K. Prakash3, Shaine A. Morris4, Dianna M. Milewicz3, Kathryn W. Holmes5, William Ravekes5, Ralph V. Shohet6, Reed E. Pyeritz7, Cheryl L. Maslen8, Barbara L. Kroner9, Kim A. Eagle10, Liliana Preiss9, GenTAC Investigators*, Federico M. Asch1
Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520
Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from
bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe,
leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously
performed.
Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions.
Clinical and echocardiographic data from the GenTAC Registry were compared between 17
patients with UAV… More >
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