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Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry.
Krepp, Joseph M; Roman, Mary J; Devereux, Richard B; Bruce, Adrienne; Prakash, Siddharth K; Morris, Shaine A; Milewicz, Dianna M; Holmes, Kathryn W; Ravekes, William; Shohet, Ralph V; Pyeritz, Reed E; Maslen, Cheryl L; Kroner, Barbara L; Eagle, Kim A; Preiss, Liliana; Asch, Federico M.
Afiliação
  • Krepp JM; MedStar Health Research Institute, Washington, District of Columbia, USA.
  • Roman MJ; Weill Medical College of Cornell University, New York, New York, USA.
  • Devereux RB; Weill Medical College of Cornell University, New York, New York, USA.
  • Bruce A; MedStar Health Research Institute, Washington, District of Columbia, USA.
  • Prakash SK; University of Texas, Houston, Texas, USA.
  • Morris SA; Baylor College of Medicine, Houston, Texas, USA.
  • Milewicz DM; University of Texas, Houston, Texas, USA.
  • Holmes KW; Johns Hopkins University, Baltimore, Maryland, USA.
  • Ravekes W; Johns Hopkins University, Baltimore, Maryland, USA.
  • Shohet RV; The Queen's Medical Center, Honolulu, Hawaii, USA.
  • Pyeritz RE; University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Maslen CL; Oregon Health & Science University, Portland, Oregon, USA.
  • Kroner BL; RTI International, Research Triangle Park, North Carolina, USA.
  • Eagle KA; University of Michigan, Ann Arbor, Michigan, USA.
  • Preiss L; RTI International, Research Triangle Park, North Carolina, USA.
  • Asch FM; MedStar Health Research Institute, Washington, District of Columbia, USA.
Congenit Heart Dis ; 12(6): 740-745, 2017 Dec.
Article em En | MEDLINE | ID: mdl-28805011
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 and 17 matched-controls with BAV. 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. 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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Valva Aórtica / Sistema de Registros / Doenças das Valvas Cardíacas Tipo de estudo: Diagnostic_studies / Guideline / Observational_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged Idioma: En Revista: Congenit Heart Dis Assunto da revista: CARDIOLOGIA Ano de publicação: 2017 Tipo de documento: Article País de afiliação: Estados Unidos País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Valva Aórtica / Sistema de Registros / Doenças das Valvas Cardíacas Tipo de estudo: Diagnostic_studies / Guideline / Observational_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged Idioma: En Revista: Congenit Heart Dis Assunto da revista: CARDIOLOGIA Ano de publicação: 2017 Tipo de documento: Article País de afiliação: Estados Unidos País de publicação: Estados Unidos