LymphedemaV1, Main, Exploration, bibRecord, 003142

Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

Identifieur interne : 003142 ( Main/Exploration ); précédent : 003141; suivant : 003143

Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

Auteurs : Laura J. Olivieri [États-Unis] ; Ridhwan Y. Baba [États-Unis] ; Andrew E. Arai [États-Unis] ; W. Patricia Bandettini [États-Unis] ; Douglas R. Rosing [États-Unis] ; Vladimir Bakalov [États-Unis] ; Vandana Sachdev [États-Unis] ; Carolyn A. Bondy [États-Unis]

Source :

Circulation. Cardiovascular imaging [ 1941-9651 ] ; 2013.

RBID : PMC:4407276

Abstract

Background

Congenital aortic valve fusion is associated with aortic dilation, aneurysm and rupture in girls and women with Turner syndrome (TS). Our objective was to characterize aortic valve structure in subjects with TS, and determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves.

Methods and Results

The aortic valve and thoracic aorta were characterized by cardiovascular magnetic resonance imaging in 208 subjects with TS in an IRB-approved natural history study. Echocardiography was used to measure peak velocities across the aortic valve, and the degree of aortic regurgitation. Four distinct valve morphologies were identified: tricuspid aortic valve (TAV) 64%(n=133), partially fused aortic valve (PF) 12%(n=25), bicuspid aortic valve (BAV) 23%(n=47), and unicuspid aortic valve (UAV) 1%(n=3). Age and body surface area (BSA) were similar in the 4 valve morphology groups. There was a significant trend, independent of age, towards larger BSA-indexed ascending aortic diameters (AADi) with increasing valve fusion. AADi were (mean +/− SD) 16.9 +/− 3.3 mm/m², 18.3 +/− 3.3 mm/m², and 19.8 +/− 3.9 mm/m² (p<0.0001) for TAV, PF and BAV+UAV respectively. PF, BAV, and UAV were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve.

Conclusions

Aortic valve abnormalities in TS occur with a spectrum of severity, and are associated with aortic root dilation across age groups. Partial fusion of the aortic valve, traditionally regarded as an acquired valve problem, had an equal age distribution and was associated with an increased AADi.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4407276

DOI: 10.1161/CIRCIMAGING.113.000526
PubMed: 24084490
PubMed Central: 4407276

Affiliations:

Le document en format XML

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<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background</title>
<p id="P1">Congenital aortic valve fusion is associated with aortic dilation, aneurysm and rupture in girls and women with Turner syndrome (TS). Our objective was to characterize aortic valve structure in subjects with TS, and determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves.</p>
</sec>
<sec id="S2"><title>Methods and Results</title>
<p id="P2">The aortic valve and thoracic aorta were characterized by cardiovascular magnetic resonance imaging in 208 subjects with TS in an IRB-approved natural history study. Echocardiography was used to measure peak velocities across the aortic valve, and the degree of aortic regurgitation. Four distinct valve morphologies were identified: tricuspid aortic valve (TAV) 64%(n=133), partially fused aortic valve (PF) 12%(n=25), bicuspid aortic valve (BAV) 23%(n=47), and unicuspid aortic valve (UAV) 1%(n=3). Age and body surface area (BSA) were similar in the 4 valve morphology groups. There was a significant trend, independent of age, towards larger BSA-indexed ascending aortic diameters (AADi) with increasing valve fusion. AADi were (mean +/− SD) 16.9 +/− 3.3 mm/m<sup>2</sup>
, 18.3 +/− 3.3 mm/m<sup>2</sup>
, and 19.8 +/− 3.9 mm/m<sup>2</sup>
 (p<0.0001) for TAV, PF and BAV+UAV respectively. PF, BAV, and UAV were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve.</p>
</sec>
<sec id="S3"><title>Conclusions</title>
<p id="P3">Aortic valve abnormalities in TS occur with a spectrum of severity, and are associated with aortic root dilation across age groups. Partial fusion of the aortic valve, traditionally regarded as an acquired valve problem, had an equal age distribution and was associated with an increased AADi.</p>
</sec>
</div>
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<tree><country name="États-Unis"><region name="Maryland"><name sortKey="Olivieri, Laura J" sort="Olivieri, Laura J" uniqKey="Olivieri L" first="Laura J." last="Olivieri">Laura J. Olivieri</name>
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<name sortKey="Arai, Andrew E" sort="Arai, Andrew E" uniqKey="Arai A" first="Andrew E." last="Arai">Andrew E. Arai</name>
<name sortKey="Baba, Ridhwan Y" sort="Baba, Ridhwan Y" uniqKey="Baba R" first="Ridhwan Y." last="Baba">Ridhwan Y. Baba</name>
<name sortKey="Bakalov, Vladimir" sort="Bakalov, Vladimir" uniqKey="Bakalov V" first="Vladimir" last="Bakalov">Vladimir Bakalov</name>
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<name sortKey="Bondy, Carolyn A" sort="Bondy, Carolyn A" uniqKey="Bondy C" first="Carolyn A." last="Bondy">Carolyn A. Bondy</name>
<name sortKey="Rosing, Douglas R" sort="Rosing, Douglas R" uniqKey="Rosing D" first="Douglas R." last="Rosing">Douglas R. Rosing</name>
<name sortKey="Sachdev, Vandana" sort="Sachdev, Vandana" uniqKey="Sachdev V" first="Vandana" last="Sachdev">Vandana Sachdev</name>
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Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

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Abstract

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