Citation: Congenital Heart Disease. 12(6):740-745, 2017 Dec.Journal: Congenital heart disease.Published: 2017ISSN: 1747-079X.Full author list: Krepp JM; Roman MJ; Devereux RB; Bruce A; Prakash SK; Morris SA; Milewicz DM; Holmes KW; Ravekes W; Shohet RV; Pyeritz RE; Maslen CL; Kroner BL; Eagle KA; Preiss L; Asch FM; GenTAC Investigators.UI/PMID: 28805011.Subject(s): Adolescent | Adult | Aorta/dg [Diagnostic Imaging] | *Aortic Valve/ab [Abnormalities] | Aortic Valve/dg [Diagnostic Imaging] | Child | Child, Preschool | Diagnosis, Differential | Echocardiography | Female | Heart Valve Diseases/cn [Congenital] | Heart Valve Diseases/di [Diagnosis] | *Heart Valve Diseases/ge [Genetics] | Humans | Infant | Male | Middle Aged | Phenotype | *Registries | Retrospective Studies | Young AdultInstitution(s): MedStar Heart & Vascular InstituteActivity type: Journal Article.Medline article type(s): Journal ArticleDigital Object Identifier: https://dx.doi.org/10.1111/chd.12520 (Click here)ORCID: Krepp, Joseph M http://orcid.org/0000-0003-4626-9200 (Click here)Abbreviated citation: Congenit. heart dis.. 12(6):740-745, 2017 Dec.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.Abstract: 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 and 17 matched-controls with BAV.Abstract: RESULTS: 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.Abstract: CONCLUSIONS: 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. Copyright (c) 2017 Wiley Periodicals, Inc.