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BAV is often identified in otherwise healthy, asymptomatic individuals, but it is associated with serious long term health risks including progressive aortic valve disease (stenosis or regurgitation) and thoracic aortic aneurysm and dissection. BAV and other LVOTO defects have high heritability. Although recommendations for cardiac screening of BAV in at\u2010risk relatives exist, there are no standard guidelines for providing genetic counseling to patients and families with BAV. This review describes current knowledge of BAV and associated aortopathy and provides guidance to genetic counselors involved in the care of patients and families with these malformations. The heritability of BAV and recommendations for screening are highlighted. While this review focuses specifically on BAV, the principles are applicable to counseling needs for other LVOTO defects.<\/jats:p>","DOI":"10.1007\/s10897-016-0002-6","type":"journal-article","created":{"date-parts":[[2016,8,22]],"date-time":"2016-08-22T04:16:39Z","timestamp":1471839399000},"page":"1171-1178","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["Bicuspid Aortic Valve: a Review with Recommendations for Genetic Counseling"],"prefix":"10.1002","volume":"25","author":[{"given":"Samantha L.","family":"Freeze","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children at IU Health Indiana University School of Medicine Indianapolis IN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin J.","family":"Landis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children at IU Health Indiana University School of Medicine Indianapolis IN USA"},{"name":"Department of Medical &amp; Molecular Genetics, Riley Hospital for Children at IU Health Indiana University School of Medicine 975 West Walnut Street, IB\u2010130 Indianapolis IN 46202 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie M.","family":"Ware","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children at IU Health Indiana University School of Medicine Indianapolis IN USA"},{"name":"Department of Medical &amp; Molecular Genetics, Riley Hospital for Children at IU Health Indiana University School of Medicine 975 West Walnut Street, IB\u2010130 Indianapolis IN 46202 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin M.","family":"Helm","sequence":"additional","affiliation":[{"name":"Department of Medical &amp; Molecular Genetics, Riley Hospital for Children at IU Health Indiana University School of Medicine 975 West Walnut Street, IB\u2010130 Indianapolis IN 46202 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,8,22]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.592089"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2012.08.017"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)34382-X"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2008.04.044"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.03.027"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/pr.2014.67"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cpcardiol.2005.06.002"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1540-8175.2003.03012.x"},{"key":"e_1_2_14_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-009-9616-x"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clp.2015.02.009"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.03.050"},{"key":"e_1_2_14_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10897-005-0519-6"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2006.10.074"},{"key":"e_1_2_14_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcts.2006.12.006"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.57.5.1022"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2350-14-44"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.72.5.668"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature03940"},{"key":"e_1_2_14_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(94)90018-3"},{"key":"e_1_2_14_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.eurheartj.a062276"},{"key":"e_1_2_14_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2007.6"},{"key":"e_1_2_14_23_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2013-3051"},{"key":"e_1_2_14_24_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/926975"},{"key":"e_1_2_14_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2007.07.021"},{"key":"e_1_2_14_26_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0b013e3181d4739e"},{"key":"e_1_2_14_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.11.006"},{"key":"e_1_2_14_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(97)00372-0"},{"issue":"19","key":"e_1_2_14_29_1","first-page":"1626","article-title":"Left ventricular outflow tract obstruction: should cardiac screening be offered to first\u2010degree relatives? 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