Aortopathy and regurgitation in bicuspid valve patients increase the risk of aortopathy in relatives

Bicuspid Aortic Valve Consortium – BAVCON

Research output: Contribution to journalArticle

Abstract

Background: Bicuspid aortic valve (BAV) is the most frequent cardiac congenital valvular disease. Although the BAV risk of first degree relatives (FDR) has been assessed (7–9%), there is little information as to the heritable risk for aortopathy. Objective: Identify the specific risk for regional aortopathy in FDR with tricuspid aortic valve (TAV) of BAV patients according to their aortic phenotype and aortic regurgitation (AR). Methods: Using an international consortium, BAV probands were assessed for aortopathy of the root, ascending aorta and for AR. Aortopathy was defined by the presence of segmental dilatation. The presence of segmental aortopathy and AR in BAV probands was evaluated as predictor for aortopathy in FDR with TAV. Results: We identified 74 FDR related to 49 probands with aortopathy and 66 FDR related to 31 probands without aortopathy. Demographic variables were similar between proband groups. Among FDR, 16 individuals had BAV (11.4%). TAV-FDR of probands with ascending aortopathy had higher incidence of root aortopathy (18.8% vs. 3.6% p < 0.05) while TAV-FDR of probands with root aortopathy had higher incidence of aortopathy at all aortic segments (55%vs25%, 55%vs21%, and 4%vs29% at annulus, root and ascending respectively, p < 0.05 for all). Independent predictors for root aortopathy in TAV-FDR were: ascending (OR = 6.23;95%CI:1.27–30.5) and root aortopathy (OR = 9.00;95%CI:1.58–51.1) in probands; and for ascending aortopathy: root aortopathy (OR = 4.04;95%CI:1.33–12.3) and AR in probands (OR = 4.84; 95%CI:1.75–13.4). Conclusion: Root and ascending aortopathy in BAV probands are strong predictors of aortopathy in their TAV-FDR. AR in BAV patients has an independent effect on the risk for ascending aortopathy in TAV-FDR.

Original languageEnglish (US)
Pages (from-to)117-120
Number of pages4
JournalInternational Journal of Cardiology
Volume286
DOIs
StatePublished - Jul 1 2019

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Mitral Valve
Tricuspid Valve
Aortic Valve
Aortic Valve Insufficiency
Bicuspid Aortic Valve
Incidence
Aorta
Dilatation
Demography
Phenotype

Keywords

  • Aneurysm
  • Aorta
  • Bicuspid aortic valve

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Aortopathy and regurgitation in bicuspid valve patients increase the risk of aortopathy in relatives. / Bicuspid Aortic Valve Consortium – BAVCON.

In: International Journal of Cardiology, Vol. 286, 01.07.2019, p. 117-120.

Research output: Contribution to journalArticle

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title = "Aortopathy and regurgitation in bicuspid valve patients increase the risk of aortopathy in relatives",
abstract = "Background: Bicuspid aortic valve (BAV) is the most frequent cardiac congenital valvular disease. Although the BAV risk of first degree relatives (FDR) has been assessed (7–9{\%}), there is little information as to the heritable risk for aortopathy. Objective: Identify the specific risk for regional aortopathy in FDR with tricuspid aortic valve (TAV) of BAV patients according to their aortic phenotype and aortic regurgitation (AR). Methods: Using an international consortium, BAV probands were assessed for aortopathy of the root, ascending aorta and for AR. Aortopathy was defined by the presence of segmental dilatation. The presence of segmental aortopathy and AR in BAV probands was evaluated as predictor for aortopathy in FDR with TAV. Results: We identified 74 FDR related to 49 probands with aortopathy and 66 FDR related to 31 probands without aortopathy. Demographic variables were similar between proband groups. Among FDR, 16 individuals had BAV (11.4{\%}). TAV-FDR of probands with ascending aortopathy had higher incidence of root aortopathy (18.8{\%} vs. 3.6{\%} p < 0.05) while TAV-FDR of probands with root aortopathy had higher incidence of aortopathy at all aortic segments (55{\%}vs25{\%}, 55{\%}vs21{\%}, and 4{\%}vs29{\%} at annulus, root and ascending respectively, p < 0.05 for all). Independent predictors for root aortopathy in TAV-FDR were: ascending (OR = 6.23;95{\%}CI:1.27–30.5) and root aortopathy (OR = 9.00;95{\%}CI:1.58–51.1) in probands; and for ascending aortopathy: root aortopathy (OR = 4.04;95{\%}CI:1.33–12.3) and AR in probands (OR = 4.84; 95{\%}CI:1.75–13.4). Conclusion: Root and ascending aortopathy in BAV probands are strong predictors of aortopathy in their TAV-FDR. AR in BAV patients has an independent effect on the risk for ascending aortopathy in TAV-FDR.",
keywords = "Aneurysm, Aorta, Bicuspid aortic valve",
author = "{Bicuspid Aortic Valve Consortium – BAVCON} and Victor Dayan and Ana Zuasnabar and Rodolfo Citro and Eduardo Bossone and Michelena, {Hector I} and Gabriel Parma and Michele Bellino and Alicia Olascoaga and Lucia Florio and Simon Body",
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T1 - Aortopathy and regurgitation in bicuspid valve patients increase the risk of aortopathy in relatives

AU - Bicuspid Aortic Valve Consortium – BAVCON

AU - Dayan, Victor

AU - Zuasnabar, Ana

AU - Citro, Rodolfo

AU - Bossone, Eduardo

AU - Michelena, Hector I

AU - Parma, Gabriel

AU - Bellino, Michele

AU - Olascoaga, Alicia

AU - Florio, Lucia

AU - Body, Simon

PY - 2019/7/1

Y1 - 2019/7/1

N2 - Background: Bicuspid aortic valve (BAV) is the most frequent cardiac congenital valvular disease. Although the BAV risk of first degree relatives (FDR) has been assessed (7–9%), there is little information as to the heritable risk for aortopathy. Objective: Identify the specific risk for regional aortopathy in FDR with tricuspid aortic valve (TAV) of BAV patients according to their aortic phenotype and aortic regurgitation (AR). Methods: Using an international consortium, BAV probands were assessed for aortopathy of the root, ascending aorta and for AR. Aortopathy was defined by the presence of segmental dilatation. The presence of segmental aortopathy and AR in BAV probands was evaluated as predictor for aortopathy in FDR with TAV. Results: We identified 74 FDR related to 49 probands with aortopathy and 66 FDR related to 31 probands without aortopathy. Demographic variables were similar between proband groups. Among FDR, 16 individuals had BAV (11.4%). TAV-FDR of probands with ascending aortopathy had higher incidence of root aortopathy (18.8% vs. 3.6% p < 0.05) while TAV-FDR of probands with root aortopathy had higher incidence of aortopathy at all aortic segments (55%vs25%, 55%vs21%, and 4%vs29% at annulus, root and ascending respectively, p < 0.05 for all). Independent predictors for root aortopathy in TAV-FDR were: ascending (OR = 6.23;95%CI:1.27–30.5) and root aortopathy (OR = 9.00;95%CI:1.58–51.1) in probands; and for ascending aortopathy: root aortopathy (OR = 4.04;95%CI:1.33–12.3) and AR in probands (OR = 4.84; 95%CI:1.75–13.4). Conclusion: Root and ascending aortopathy in BAV probands are strong predictors of aortopathy in their TAV-FDR. AR in BAV patients has an independent effect on the risk for ascending aortopathy in TAV-FDR.

AB - Background: Bicuspid aortic valve (BAV) is the most frequent cardiac congenital valvular disease. Although the BAV risk of first degree relatives (FDR) has been assessed (7–9%), there is little information as to the heritable risk for aortopathy. Objective: Identify the specific risk for regional aortopathy in FDR with tricuspid aortic valve (TAV) of BAV patients according to their aortic phenotype and aortic regurgitation (AR). Methods: Using an international consortium, BAV probands were assessed for aortopathy of the root, ascending aorta and for AR. Aortopathy was defined by the presence of segmental dilatation. The presence of segmental aortopathy and AR in BAV probands was evaluated as predictor for aortopathy in FDR with TAV. Results: We identified 74 FDR related to 49 probands with aortopathy and 66 FDR related to 31 probands without aortopathy. Demographic variables were similar between proband groups. Among FDR, 16 individuals had BAV (11.4%). TAV-FDR of probands with ascending aortopathy had higher incidence of root aortopathy (18.8% vs. 3.6% p < 0.05) while TAV-FDR of probands with root aortopathy had higher incidence of aortopathy at all aortic segments (55%vs25%, 55%vs21%, and 4%vs29% at annulus, root and ascending respectively, p < 0.05 for all). Independent predictors for root aortopathy in TAV-FDR were: ascending (OR = 6.23;95%CI:1.27–30.5) and root aortopathy (OR = 9.00;95%CI:1.58–51.1) in probands; and for ascending aortopathy: root aortopathy (OR = 4.04;95%CI:1.33–12.3) and AR in probands (OR = 4.84; 95%CI:1.75–13.4). Conclusion: Root and ascending aortopathy in BAV probands are strong predictors of aortopathy in their TAV-FDR. AR in BAV patients has an independent effect on the risk for ascending aortopathy in TAV-FDR.

KW - Aneurysm

KW - Aorta

KW - Bicuspid aortic valve

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