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Erschienen in: Pediatric Cardiology 2/2020

19.12.2019 | Original Article

Eccentric Enlargement of the Aortic Sinuses in Pediatric and Adult Patients with Bicuspid Aortic Valves: A Cardiac MRI Study

verfasst von: Heather A. Stefek, Kevin H. Lin, Cynthia K. Rigsby, Hector I. Michelena, Pascale Aouad, Alex J. Barker, Joshua D. Robinson

Erschienen in: Pediatric Cardiology | Ausgabe 2/2020

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Abstract

Aortic root size and cusp fusion pattern have been related to disease outcomes in bicuspid aortic valve (BAV). This study seeks to characterize symmetry of the aortic sinuses in adult and pediatric BAV patients and its relationship to valvulopathy and root aortopathy. Aortic sinus-to-commissure (S-C) lengths were measured on cardiac MRI of adult and pediatric BAV patients with right-and-left coronary (RL) or right-and-non-coronary (RN) leaflet fusion and tricuspid aortic valve (TAV) controls. Coefficient of variance (CoV) of S-C lengths was calculated to quantify sinus asymmetry, or eccentricity. BAV cohort included 149 adults (48 ± 15 years) and 51 children (15 ± 5 years). TAV cohort included 40 adults (60 ± 13 years) and 20 children (15 ± 5 years). In adult and pediatric BAV patients, the non-fused aortic sinus was larger than either fused sinus. In RL fusion, the non-coronary S-C distance was larger than right or left S-C distances in adults (n = 121, p < 0.001) and larger than the right S-C distance in children (n = 41, p = 0.013). Sinus eccentricity (CoV) in BAV patients was higher than in TAV patients (p < 0.001) and did not correlate with age (p = 0.12). CoV trended higher in RL adults with aortic regurgitation (AR) compared to those without AR (p = 0.081), but was lower in RN adults with AR than without AR (p = 0.006). CoV did not correlate to root Z scores (p = 0.06–0.55) or ascending aortic (AAo) Z scores in adults (p = 0.45–0.55) but correlated negatively to AAo Z score in children (p = 0.005–0.03). Most adult and pediatric BAV patients with RL and RN leaflet fusion demonstrate eccentric dominance of the non-fused aortic sinus irrespective of age. The degree of eccentricity varies with valve dysfunction and BAV phenotype but does not relate to the degree of aortic root dilatation, nor does eccentricity correlate with ascending aorta dilatation in adults.
Literatur
1.
Zurück zum Zitat Basso C, Boschello M, Perrone C et al (2004) An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol 93:661–663CrossRef Basso C, Boschello M, Perrone C et al (2004) An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol 93:661–663CrossRef
2.
Zurück zum Zitat Roberts WC (1970) The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 26:72–83CrossRef Roberts WC (1970) The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 26:72–83CrossRef
3.
Zurück zum Zitat Michelena HI, Khanna AD, Mahoney D et al (2011) Incidence of aortic complications in patients with bicuspid aortic valves. JAMA 306:1104–1112CrossRef Michelena HI, Khanna AD, Mahoney D et al (2011) Incidence of aortic complications in patients with bicuspid aortic valves. JAMA 306:1104–1112CrossRef
4.
Zurück zum Zitat Kang JW, Song HG, Yang DH et al (2013) Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: comprehensive evaluation using MDCT and echocardiography. JACC Cardiovasc Imaging 6:150–161CrossRef Kang JW, Song HG, Yang DH et al (2013) Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: comprehensive evaluation using MDCT and echocardiography. JACC Cardiovasc Imaging 6:150–161CrossRef
5.
Zurück zum Zitat Kong WK, Delgado V, Poh KK et al (2017) Prognostic implications of raphe in bicuspid aortic valve anatomy. JAMA Cardiol 2:285–292CrossRef Kong WK, Delgado V, Poh KK et al (2017) Prognostic implications of raphe in bicuspid aortic valve anatomy. JAMA Cardiol 2:285–292CrossRef
6.
Zurück zum Zitat Barker AJ, Markl M, Burk J et al (2012) Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging 5:457–466CrossRef Barker AJ, Markl M, Burk J et al (2012) Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging 5:457–466CrossRef
7.
Zurück zum Zitat Mahadevia R, Barker AJ, Schnell S et al (2014) Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation 129:673–682CrossRef Mahadevia R, Barker AJ, Schnell S et al (2014) Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation 129:673–682CrossRef
8.
Zurück zum Zitat Capoulade R, Teoh JG, Bartko PE et al (2018) Relationship between proximal aorta morphology and progression rate of aortic stenosis. J Am Soc Echocardiogr 31:561–569.e561CrossRef Capoulade R, Teoh JG, Bartko PE et al (2018) Relationship between proximal aorta morphology and progression rate of aortic stenosis. J Am Soc Echocardiogr 31:561–569.e561CrossRef
9.
Zurück zum Zitat Michelena HI, Della Corte A, Prakash SK et al (2015) Bicuspid aortic valve aortopathy in adults: incidence, etiology, and clinical significance. Int J Cardiol 201:400–407CrossRef Michelena HI, Della Corte A, Prakash SK et al (2015) Bicuspid aortic valve aortopathy in adults: incidence, etiology, and clinical significance. Int J Cardiol 201:400–407CrossRef
10.
Zurück zum Zitat Detaint D, Michelena HI, Nkomo VT et al (2014) Aortic dilatation patterns and rates in adults with bicuspid aortic valves: a comparative study with Marfan syndrome and degenerative aortopathy. Heart (British Cardiac Society) 100:126–134 Detaint D, Michelena HI, Nkomo VT et al (2014) Aortic dilatation patterns and rates in adults with bicuspid aortic valves: a comparative study with Marfan syndrome and degenerative aortopathy. Heart (British Cardiac Society) 100:126–134
11.
Zurück zum Zitat Torres FS, Windram JD, Bradley TJ et al (2013) Impact of asymmetry on measurements of the aortic root using cardiovascular magnetic resonance imaging in patients with a bicuspid aortic valve. Int J Cardiovasc Imaging 29:1769–1777CrossRef Torres FS, Windram JD, Bradley TJ et al (2013) Impact of asymmetry on measurements of the aortic root using cardiovascular magnetic resonance imaging in patients with a bicuspid aortic valve. Int J Cardiovasc Imaging 29:1769–1777CrossRef
12.
Zurück zum Zitat Vis JC, Rodriguez-Palomares JF, Teixido-Tura G et al (2019) Implications of asymmetry and valvular morphotype on echocardiographic measurements of the aortic root in bicuspid aortic valve. J Am Soc Echocardiogr 32:105–112CrossRef Vis JC, Rodriguez-Palomares JF, Teixido-Tura G et al (2019) Implications of asymmetry and valvular morphotype on echocardiographic measurements of the aortic root in bicuspid aortic valve. J Am Soc Echocardiogr 32:105–112CrossRef
13.
Zurück zum Zitat Chubb H, Simpson JM (2012) The use of Z-scores in paediatric cardiology. Ann Pediatr Cardiol 5:179–184CrossRef Chubb H, Simpson JM (2012) The use of Z-scores in paediatric cardiology. Ann Pediatr Cardiol 5:179–184CrossRef
14.
Zurück zum Zitat Campens L, Demulier L, De Groote K et al (2014) Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories. Am J Cardiol 114:914–920CrossRef Campens L, Demulier L, De Groote K et al (2014) Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories. Am J Cardiol 114:914–920CrossRef
15.
Zurück zum Zitat Michelena HI, Prakash SK, Della Corte A et al (2014) Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon). Circulation 129:2691–2704CrossRef Michelena HI, Prakash SK, Della Corte A et al (2014) Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon). Circulation 129:2691–2704CrossRef
16.
Zurück zum Zitat Entezari P, Schnell S, Mahadevia R et al (2014) From unicuspid to quadricuspid: influence of aortic valve morphology on aortic three-dimensional hemodynamics. J Magn Reson Imaging 40:1342–1346CrossRef Entezari P, Schnell S, Mahadevia R et al (2014) From unicuspid to quadricuspid: influence of aortic valve morphology on aortic three-dimensional hemodynamics. J Magn Reson Imaging 40:1342–1346CrossRef
17.
Zurück zum Zitat Vis JC, Rodriguez-Palomares JF, Teixido-Tura G et al (2018) Implications of asymmetry and valvular morphotype on echocardiographic measurements of the aortic root in bicuspid aortic valve. J Am Soc Echocardiogr 32:105–112CrossRef Vis JC, Rodriguez-Palomares JF, Teixido-Tura G et al (2018) Implications of asymmetry and valvular morphotype on echocardiographic measurements of the aortic root in bicuspid aortic valve. J Am Soc Echocardiogr 32:105–112CrossRef
18.
Zurück zum Zitat Fernandez B, Duran AC, Fernandez-Gallego T et al (2009) Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 54:2312–2318CrossRef Fernandez B, Duran AC, Fernandez-Gallego T et al (2009) Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 54:2312–2318CrossRef
19.
Zurück zum Zitat Phillips HM, Mahendran P, Singh E et al (2013) Neural crest cells are required for correct positioning of the developing outflow cushions and pattern the arterial valve leaflets. Cardiovasc Res 99:452–460CrossRef Phillips HM, Mahendran P, Singh E et al (2013) Neural crest cells are required for correct positioning of the developing outflow cushions and pattern the arterial valve leaflets. Cardiovasc Res 99:452–460CrossRef
20.
Zurück zum Zitat Beroukhim RS, Kruzick TL, Taylor AL et al (2006) Progression of aortic dilation in children with a functionally normal bicuspid aortic valve. Am J Cardiol 98:828–830CrossRef Beroukhim RS, Kruzick TL, Taylor AL et al (2006) Progression of aortic dilation in children with a functionally normal bicuspid aortic valve. Am J Cardiol 98:828–830CrossRef
21.
Zurück zum Zitat Spaziani G, Ballo P, Favilli S et al (2014) Clinical outcome, valve dysfunction, and progressive aortic dilation in a pediatric population with isolated bicuspid aortic valve. Pediatr Cardiol 35:803–809CrossRef Spaziani G, Ballo P, Favilli S et al (2014) Clinical outcome, valve dysfunction, and progressive aortic dilation in a pediatric population with isolated bicuspid aortic valve. Pediatr Cardiol 35:803–809CrossRef
22.
Zurück zum Zitat Merkx R, Duijnhouwer AL, Vink E et al (2017) Aortic diameter growth in children with a bicuspid aortic valve. Am J Cardiol 120:131–136CrossRef Merkx R, Duijnhouwer AL, Vink E et al (2017) Aortic diameter growth in children with a bicuspid aortic valve. Am J Cardiol 120:131–136CrossRef
23.
Zurück zum Zitat Hope MD, Hope TA, Meadows AK et al (2010) Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flow patterns. Radiology 255:53–61CrossRef Hope MD, Hope TA, Meadows AK et al (2010) Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flow patterns. Radiology 255:53–61CrossRef
24.
Zurück zum Zitat Rodriguez-Palomares JF, Dux-Santoy L, Guala A et al (2018) Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson 20:28CrossRef Rodriguez-Palomares JF, Dux-Santoy L, Guala A et al (2018) Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson 20:28CrossRef
25.
Zurück zum Zitat Aicher D, Kunihara T, Abou Issa O et al (2011) Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation 123:178–185CrossRef Aicher D, Kunihara T, Abou Issa O et al (2011) Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation 123:178–185CrossRef
26.
Zurück zum Zitat Ugur M, Schaff HV, Suri RM et al (2014) Late outcome of noncoronary sinus replacement in patients with bicuspid aortic valves and aortopathy. Ann Thorac Surg 97:1242–1246CrossRef Ugur M, Schaff HV, Suri RM et al (2014) Late outcome of noncoronary sinus replacement in patients with bicuspid aortic valves and aortopathy. Ann Thorac Surg 97:1242–1246CrossRef
Metadaten
Titel
Eccentric Enlargement of the Aortic Sinuses in Pediatric and Adult Patients with Bicuspid Aortic Valves: A Cardiac MRI Study
verfasst von
Heather A. Stefek
Kevin H. Lin
Cynthia K. Rigsby
Hector I. Michelena
Pascale Aouad
Alex J. Barker
Joshua D. Robinson
Publikationsdatum
19.12.2019
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 2/2020
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-019-02264-3

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