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)

Mary J. Roman, Norma L. Pugh, Richard B. Devereux, Kim A. Eagle, Kathryn Holmes, Scott A. LeMaire, Rita K. Milewski, Shaine A. Morris, Siddharth K. Prakash, Reed E. Pyeritz, William J. Ravekes, Ralph V. Shohet, Howard Song, Federico M. Asch

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Abstract

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.

Original languageEnglish (US)
JournalAmerican Journal of Cardiology
DOIs
StateAccepted/In press - 2017

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National Heart, Lung, and Blood Institute (U.S.)
Thoracic Aortic Aneurysm
Aortic Valve Insufficiency
Aortic Valve Stenosis
Sinus of Valsalva
Registries
Dilatation
Hemodynamics
Bicuspid Aortic Valve
Population

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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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). / Roman, Mary J.; Pugh, Norma L.; Devereux, Richard B.; Eagle, Kim A.; Holmes, Kathryn; LeMaire, Scott A.; Milewski, Rita K.; Morris, Shaine A.; Prakash, Siddharth K.; Pyeritz, Reed E.; Ravekes, William J.; Shohet, Ralph V.; Song, Howard; Asch, Federico M.

In: American Journal of Cardiology, 2017.

Research output: Contribution to journalArticle

Roman, Mary J. ; Pugh, Norma L. ; Devereux, Richard B. ; Eagle, Kim A. ; Holmes, Kathryn ; LeMaire, Scott A. ; Milewski, Rita K. ; Morris, Shaine A. ; Prakash, Siddharth K. ; Pyeritz, Reed E. ; Ravekes, William J. ; Shohet, Ralph V. ; Song, Howard ; Asch, Federico M. / 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). In: American Journal of Cardiology. 2017.
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title = "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)",
abstract = "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.",
author = "Roman, {Mary J.} and Pugh, {Norma L.} and Devereux, {Richard B.} and Eagle, {Kim A.} and Kathryn Holmes and LeMaire, {Scott A.} and Milewski, {Rita K.} and Morris, {Shaine A.} and Prakash, {Siddharth K.} and Pyeritz, {Reed E.} and Ravekes, {William J.} and Shohet, {Ralph V.} and Howard Song and Asch, {Federico M.}",
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T2 - 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)

AU - Roman, Mary J.

AU - Pugh, Norma L.

AU - Devereux, Richard B.

AU - Eagle, Kim A.

AU - Holmes, Kathryn

AU - LeMaire, Scott A.

AU - Milewski, Rita K.

AU - Morris, Shaine A.

AU - Prakash, Siddharth K.

AU - Pyeritz, Reed E.

AU - Ravekes, William J.

AU - Shohet, Ralph V.

AU - Song, Howard

AU - Asch, Federico M.

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

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