@Article{chd.12520,
AUTHOR = {Joseph M. Krepp, Mary J. Roman, Richard B. Devereux, Adrienne Bruce, Siddharth K. Prakash, Shaine A. Morris, Dianna M. Milewicz, Kathryn W. Holmes, William Ravekes, Ralph V. Shohet, Reed E. Pyeritz, Cheryl L. Maslen, Barbara L. Kroner, Kim A. Eagle, Liliana Preiss, GenTAC Investigators, Federico M. Asch},
TITLE = {Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {12},
YEAR = {2017},
NUMBER = {6},
PAGES = {740--745},
URL = {http://www.techscience.com/schd/v12n6/39228},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> 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.<br/>
<b>Methods:</b> 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 and 17 matched-controls with BAV.<br/>
<b>Results:</b> Baseline characteristics including demographics, clinical findings including family history
of BAV and aortic aneurysm/coarctation, and echocardiographic variables were similar between
BAV and UAV patients; aortic stenosis was more common and more severe in patients with UAV.
This was evidenced by higher mean and peak gradient, smaller aortic valve area, and more
advanced valvular degeneration (all P < .05). There were no significant differences in aortic dimensions, with a similar pattern of enlargement of the ascending aorta.<br/>
<b>Conclusions:</b> The similar baseline characteristics with more accelerated aortic valve degeneration
and stenosis suggest that UAV represents an extreme in the spectrum of BAV syndromes. Therefore, it is reasonable to consider application of recommendations for the management of patients
with BAV to those with the rarer UAV.},
DOI = {10.1111/chd.12520}
}