Aortic dilatation patterns and rates in adults with bicuspid aortic valves

A comparative study with Marfan syndrome and degenerative aortopathy

Delphine Detaint, Hector I Michelena, Vuyisile T Nkomo, Alec Vahanian, Guillaume Jondeau, Maurice E Sarano

Research output: Contribution to journalArticle

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Abstract

Background Bicuspid aortic valve (BAV) is related to aortic dilatation, but patterns/rates are conflicting with no comparison among aneurysms of different aetiology. We sought to define ascending aorta dilatation patterns/ progression rates in BAV versus other aortopathies (Marfan syndrome (MFS), degenerative aortopathy (DA)). Design and setting Retrospective, observational study. Aortic dilatation progression was evaluated in two tertiary care centres (US and European) by repeated echocardiography ≥2 years apart in adults with BAV (n=353), matched to MFS (n=50) and DA (n=51) for gender, blood pressure, and minimum follow-up time. Results At baseline, ascending aortic dilatation was present in 87% of BAV cases: tubular ascending aorta in 60% (irrespective of BAV morphology), and Valsalva sinuses dilatation in 27% (independently linked to typical BAV morphology and male gender (p=0.0001)). After 3.6±1.2 years, the aortic dilatation rate in BAV was higher than expected for the population for all aortic levels (p=0.005) and was maximal at the tubular ascending aorta for BAV (0.42±0.6 mm/year) and DA (0.20±0.3 mm/year), and was maximal at the Valsalva sinuses for MFS (0.49±0.5 mm/year). Maximal aortic dilatation rate was similar between BAV and MFS (p>0.40) and lower in DA (p=0.02) but was heterogeneous in BAV, with 43% of BAV not progressing (vs 20% of MFS, p=0.01). Aortic dilatation rate was not proportionally related to baseline aortic size or BAV type (all models p>0.40). Conclusions In patients with BAV, tubular ascending aorta dilatation is the most common pattern and exhibits the fastest growing rate, irrespective of valve morphology and function. Dilatation of the Valsalva sinuses is less common and associated with typical BAV morphology and male gender. Aortic dilatation progresses equally fast in BAV (tubular segment) and MFS (Valsalva sinuses), but a significantly higher proportion of BAV patients does not progress at all, irrespective of BAV type. Baseline aortic diameter does not proportionally predict progression rate; systematic follow-up is therefore warranted in patients with BAV.

Original languageEnglish (US)
Pages (from-to)126-134
Number of pages9
JournalHeart
Volume100
Issue number2
DOIs
StatePublished - Jan 2014

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Marfan Syndrome
Dilatation
Sinus of Valsalva
Aorta
Bicuspid Aortic Valve

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Aortic dilatation patterns and rates in adults with bicuspid aortic valves : A comparative study with Marfan syndrome and degenerative aortopathy. / Detaint, Delphine; Michelena, Hector I; Nkomo, Vuyisile T; Vahanian, Alec; Jondeau, Guillaume; Sarano, Maurice E.

In: Heart, Vol. 100, No. 2, 01.2014, p. 126-134.

Research output: Contribution to journalArticle

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title = "Aortic dilatation patterns and rates in adults with bicuspid aortic valves: A comparative study with Marfan syndrome and degenerative aortopathy",
abstract = "Background Bicuspid aortic valve (BAV) is related to aortic dilatation, but patterns/rates are conflicting with no comparison among aneurysms of different aetiology. We sought to define ascending aorta dilatation patterns/ progression rates in BAV versus other aortopathies (Marfan syndrome (MFS), degenerative aortopathy (DA)). Design and setting Retrospective, observational study. Aortic dilatation progression was evaluated in two tertiary care centres (US and European) by repeated echocardiography ≥2 years apart in adults with BAV (n=353), matched to MFS (n=50) and DA (n=51) for gender, blood pressure, and minimum follow-up time. Results At baseline, ascending aortic dilatation was present in 87{\%} of BAV cases: tubular ascending aorta in 60{\%} (irrespective of BAV morphology), and Valsalva sinuses dilatation in 27{\%} (independently linked to typical BAV morphology and male gender (p=0.0001)). After 3.6±1.2 years, the aortic dilatation rate in BAV was higher than expected for the population for all aortic levels (p=0.005) and was maximal at the tubular ascending aorta for BAV (0.42±0.6 mm/year) and DA (0.20±0.3 mm/year), and was maximal at the Valsalva sinuses for MFS (0.49±0.5 mm/year). Maximal aortic dilatation rate was similar between BAV and MFS (p>0.40) and lower in DA (p=0.02) but was heterogeneous in BAV, with 43{\%} of BAV not progressing (vs 20{\%} of MFS, p=0.01). Aortic dilatation rate was not proportionally related to baseline aortic size or BAV type (all models p>0.40). Conclusions In patients with BAV, tubular ascending aorta dilatation is the most common pattern and exhibits the fastest growing rate, irrespective of valve morphology and function. Dilatation of the Valsalva sinuses is less common and associated with typical BAV morphology and male gender. Aortic dilatation progresses equally fast in BAV (tubular segment) and MFS (Valsalva sinuses), but a significantly higher proportion of BAV patients does not progress at all, irrespective of BAV type. Baseline aortic diameter does not proportionally predict progression rate; systematic follow-up is therefore warranted in patients with BAV.",
author = "Delphine Detaint and Michelena, {Hector I} and Nkomo, {Vuyisile T} and Alec Vahanian and Guillaume Jondeau and Sarano, {Maurice E}",
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doi = "10.1136/heartjnl-2013-304920",
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T1 - Aortic dilatation patterns and rates in adults with bicuspid aortic valves

T2 - A comparative study with Marfan syndrome and degenerative aortopathy

AU - Detaint, Delphine

AU - Michelena, Hector I

AU - Nkomo, Vuyisile T

AU - Vahanian, Alec

AU - Jondeau, Guillaume

AU - Sarano, Maurice E

PY - 2014/1

Y1 - 2014/1

N2 - Background Bicuspid aortic valve (BAV) is related to aortic dilatation, but patterns/rates are conflicting with no comparison among aneurysms of different aetiology. We sought to define ascending aorta dilatation patterns/ progression rates in BAV versus other aortopathies (Marfan syndrome (MFS), degenerative aortopathy (DA)). Design and setting Retrospective, observational study. Aortic dilatation progression was evaluated in two tertiary care centres (US and European) by repeated echocardiography ≥2 years apart in adults with BAV (n=353), matched to MFS (n=50) and DA (n=51) for gender, blood pressure, and minimum follow-up time. Results At baseline, ascending aortic dilatation was present in 87% of BAV cases: tubular ascending aorta in 60% (irrespective of BAV morphology), and Valsalva sinuses dilatation in 27% (independently linked to typical BAV morphology and male gender (p=0.0001)). After 3.6±1.2 years, the aortic dilatation rate in BAV was higher than expected for the population for all aortic levels (p=0.005) and was maximal at the tubular ascending aorta for BAV (0.42±0.6 mm/year) and DA (0.20±0.3 mm/year), and was maximal at the Valsalva sinuses for MFS (0.49±0.5 mm/year). Maximal aortic dilatation rate was similar between BAV and MFS (p>0.40) and lower in DA (p=0.02) but was heterogeneous in BAV, with 43% of BAV not progressing (vs 20% of MFS, p=0.01). Aortic dilatation rate was not proportionally related to baseline aortic size or BAV type (all models p>0.40). Conclusions In patients with BAV, tubular ascending aorta dilatation is the most common pattern and exhibits the fastest growing rate, irrespective of valve morphology and function. Dilatation of the Valsalva sinuses is less common and associated with typical BAV morphology and male gender. Aortic dilatation progresses equally fast in BAV (tubular segment) and MFS (Valsalva sinuses), but a significantly higher proportion of BAV patients does not progress at all, irrespective of BAV type. Baseline aortic diameter does not proportionally predict progression rate; systematic follow-up is therefore warranted in patients with BAV.

AB - Background Bicuspid aortic valve (BAV) is related to aortic dilatation, but patterns/rates are conflicting with no comparison among aneurysms of different aetiology. We sought to define ascending aorta dilatation patterns/ progression rates in BAV versus other aortopathies (Marfan syndrome (MFS), degenerative aortopathy (DA)). Design and setting Retrospective, observational study. Aortic dilatation progression was evaluated in two tertiary care centres (US and European) by repeated echocardiography ≥2 years apart in adults with BAV (n=353), matched to MFS (n=50) and DA (n=51) for gender, blood pressure, and minimum follow-up time. Results At baseline, ascending aortic dilatation was present in 87% of BAV cases: tubular ascending aorta in 60% (irrespective of BAV morphology), and Valsalva sinuses dilatation in 27% (independently linked to typical BAV morphology and male gender (p=0.0001)). After 3.6±1.2 years, the aortic dilatation rate in BAV was higher than expected for the population for all aortic levels (p=0.005) and was maximal at the tubular ascending aorta for BAV (0.42±0.6 mm/year) and DA (0.20±0.3 mm/year), and was maximal at the Valsalva sinuses for MFS (0.49±0.5 mm/year). Maximal aortic dilatation rate was similar between BAV and MFS (p>0.40) and lower in DA (p=0.02) but was heterogeneous in BAV, with 43% of BAV not progressing (vs 20% of MFS, p=0.01). Aortic dilatation rate was not proportionally related to baseline aortic size or BAV type (all models p>0.40). Conclusions In patients with BAV, tubular ascending aorta dilatation is the most common pattern and exhibits the fastest growing rate, irrespective of valve morphology and function. Dilatation of the Valsalva sinuses is less common and associated with typical BAV morphology and male gender. Aortic dilatation progresses equally fast in BAV (tubular segment) and MFS (Valsalva sinuses), but a significantly higher proportion of BAV patients does not progress at all, irrespective of BAV type. Baseline aortic diameter does not proportionally predict progression rate; systematic follow-up is therefore warranted in patients with BAV.

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