Aortic Dilatation Associated With Bicuspid Aortic Valve: Relation to Sex, Hemodynamics, and Valve Morphology (the National Heart Lung and Blood Institute-Sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions)

Am J Cardiol. 2017 Oct 1;120(7):1171-1175. doi: 10.1016/j.amjcard.2017.06.061. Epub 2017 Jul 14.


This study analyzed the impact of sex, hemodynamic profile, and valve fusion pattern on aortopathy associated with bicuspid aortic valve (BAV). The National Heart Lung and Blood Institute-sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) provided comprehensive information on a large population of well-characterized patients with BAV. Of 969 enrolled patients with BAV, 551 (57%, 77% male) had already undergone valvular and/or aortic surgery. Echocardiographic imaging data were available on 339 unoperated or preoperative participants who formed the basis of this study. BAV function was normal in 45 (14%), with a predominant aortic regurgitation (AR) in 127 (41%) and a predominant aortic stenosis (AS) in 76 (22%). Moderate-severe AR was associated with larger sinus of Valsalva (SOV) diameters compared with normal function and AS (all p <0.01). Moderate-severe AS was associated with a larger ascending aortic (AscAo) diameter compared with normal function (p = 0.003) but not with AR. The SOV diameter was larger in men than in women (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001), whereas AscAo diameters were comparable (3.9 ± 0.9 vs 3.7 ± 0.9 cm, p = 0.08). Right-left commissural fusion was associated with a larger SOV diameter (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001) compared with a right-noncoronary fusion pattern. Predominant AR was more common in men (45% vs 27%, p = 0.004), whereas AS was more common in women (29% vs 18%, p = 0.04). In conclusion, in the GenTAC Registry, AR was associated with diffuse (annular, SOV, and AscAo) enlargement, whereas moderate-severe AS was only associated with AscAo enlargement. Male sex and right-left cusp pattern of cusp fusion were associated with larger SOV diameters and a greater likelihood of AR, whereas women had a higher prevalence of AS.

Publication types

  • Multicenter Study
  • Observational Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aorta, Thoracic / diagnostic imaging*
  • Aorta, Thoracic / physiopathology
  • Aortic Aneurysm, Thoracic / epidemiology
  • Aortic Aneurysm, Thoracic / etiology*
  • Aortic Aneurysm, Thoracic / physiopathology
  • Aortic Valve / abnormalities*
  • Aortic Valve / physiopathology
  • Bicuspid Aortic Valve Disease
  • Child
  • Child, Preschool
  • Echocardiography
  • Female
  • Follow-Up Studies
  • Heart Valve Diseases / complications*
  • Heart Valve Diseases / epidemiology
  • Heart Valve Diseases / physiopathology
  • Hemodynamics / physiology*
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Prevalence
  • Registries*
  • Retrospective Studies
  • Risk Factors
  • Sex Factors
  • United States / epidemiology
  • Young Adult