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Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome.

ABSTRACT: BACKGROUND:Congenital aortic valve fusion is associated with aortic dilation, aneurysm, and rupture in girls and women with Turner syndrome. Our objective was to characterize aortic valve structure in subjects with Turner syndrome and to 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 MRI in 208 subjects with Turner syndrome in an institutional review board-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, 64% (n=133); partially fused aortic valve, 12% (n=25); bicuspid aortic valve, 23% (n=47); and unicuspid aortic valve, 1% (n=3). Age and body surface area were similar in the 4 valve morphology groups. There was a significant trend, independent of age, toward larger body surface area-indexed ascending aortic diameters with increasing valve fusion. Ascending aortic diameters were (mean±SD) 16.9±3.3, 18.3±3.3, and 19.8±3.9 mm/m(2) (P<0.0001) for tricuspid aortic valve, partially fused aortic valve, and bicuspid aortic valve+unicuspid aortic valve, respectively. Partially fused aortic valve, bicuspid aortic valve, and unicuspid aortic valve were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve. CONCLUSIONS:Aortic valve abnormalities in Turner syndrome 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 ascending aortic diameters.

SUBMITTER: Olivieri LJ

PROVIDER: S-EPMC4407276 | biostudies-literature | 2013 Nov

REPOSITORIES: biostudies-literature

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Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome.

Olivieri Laura J LJ Baba Ridhwan Y RY Arai Andrew E AE Bandettini W Patricia WP Rosing Douglas R DR Bakalov Vladimir V Sachdev Vandana V Bondy Carolyn A CA

Circulation. Cardiovascular imaging 20131001 6

<h4>Background</h4>Congenital aortic valve fusion is associated with aortic dilation, aneurysm, and rupture in girls and women with Turner syndrome. Our objective was to characterize aortic valve structure in subjects with Turner syndrome and to determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves.<h4>Methods and results</h4>The aortic valve and thoracic aorta were characterized by cardiovascular MRI in 208 subjects with Turner syndro ...[more]

PMID: 24084490

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Project description:BackgroundThe effect of different expressions of aortic valve disease on 3D aortic hemodynamics is unclear.PurposeTo investigate changes in aortic hemodynamics in patients with dilated ascending aorta (AAo) but different severity of aortic valve stenosis (AS) and/or regurgitation (AR).Study typeRetrospective.PopulationA total of 111 subjects (86 patients with AAo diameter ≥ 40 mm and 25 healthy controls, all with trileaflet aortic valve [TAV]). Patients were further stratified by TAV dysfunction: n = 9 with combined moderate or severe AS and AR (ASR, 56 ± 13 years), n = 14 with moderate or severe AS (AS, 64 ± 14 years), n = 33 with moderate or severe AR (AR, 62 ± 14 years), n = 30 with neither AS nor AR (no AS/AR, 63 ± 9 years).Field strength/sequence4D flow MRI on 1.5/3T systems for the in vivo analysis of aortic blood flow dynamics.AssessmentData analysis included grading of 3D AAo vortex/helix flow and AAo flow eccentricity as well as quantification of systolic peak velocities and wall shear stress (WSS).Statistical testsContinuous variables were compared by one-way analysis of variance or Kruskal-Wallis, followed by a pairwise Tukey or Dunn test if there was a significant difference.ResultsAll patients demonstrated markedly elevated vortex and helix flow compared with controls (P < 0.05). Peak velocities were significantly elevated in ASR, AS, and AR patients compared with controls (P < 0.05). Increased flow eccentricity was observed in entire AAo for AR, at the mid and distal AAo for ASR and AS, and at the proximal AAo for no AS/AR. Compared with controls, WSS in the AAo was significantly elevated in ASR and AS patients (P < 0.05) and reduced in no AS/AR patients (P < 0.05).Data conclusionThe presence of TAV dysfunction is associated with aberrant hemodynamics and altered WSS, which may play a role in the development of aortopathy.Level of evidence3 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:481-491.

Dataset Information

Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome.

Publications

Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome.

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