Hostname: page-component-586b7cd67f-dsjbd Total loading time: 0 Render date: 2024-11-27T20:18:37.972Z Has data issue: false hasContentIssue false
  • English
  • Français

Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve

Published online by Cambridge University Press:  06 August 2018

Elif Erolu ,
Figen Akalın ,
Nilüfer Çetiner  and
Berna Ç. Şaylan
Elif Erolu*
Affiliation:
Divisions of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey
Figen Akalın
Affiliation:
Divisions of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey
Nilüfer Çetiner
Affiliation:
Divisions of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey
Berna Ç. Şaylan
Affiliation:
Divisions of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey
*
Author for correspondence: Elif Erolu, Divisions of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Marmara University Fevzi Çakmak Mah. Muhsin Yazıcıoğlu Cad. No: 10 Üst Kaynarca, Pendik, Istanbul, Turkey. Tel: +90 505 816 9456; Fax: +90 216 625 4639; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Aim

We investigated dimensions and elasticity of whole aorta in patients with bicuspid aortic valve and influence of valve phenotype.

Method

The study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal – fusion between right and left coronary cusps – and vertical – fusion between right–non-coronary cusps; according to age they were divided into younger (5–10 years) and older patients (11–16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic–diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending–descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied.

Results

z-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn−1.10−6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group.

Conclusion

Aortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.

Keywords

Bicuspid aortic valvechildrenaortic distensibilityaortic stiffnessflow-mediated dilatation
Type
Original Article
Information
Cardiology in the Young , Volume 28 , Issue 11 , November 2018 , pp. 1338 - 1344
Copyright
© Cambridge University Press 2018 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Cecconi, M, Nistri, S, Quarti, A, et al. Aortic dilatation in patients with bicuspid aortic valve. J Cardiovasc Med 2006; 7: 11–20.Google Scholar
2. Gurvitz, M, Chang, RK, Drant, S, Allada, V. Frequency of aortic root dilatation in children with a bicuspid aortic valve. Am J Cardiol 2004; 94: 1337–1340.Google Scholar
3. Della Corta, A, Bancone, C, Quarto, C, et al. Predictors of ascending aortic dilataiton with bicuspid aortic valve: a wide spectrum of disease expression. Eur J Cardiothorac Surg 2007; 31: 397–404.Google Scholar
4. Pagé, M, Mongeon, FP, Stevens, LM, Soulière, V, Khairy, P, El-Hamamsy, I. Aortic dilation rates in patients with biscuspid aortic valve: correlations with cusp fusion phenotype. J Heart Valve Dis 2014; 23: 450–457.Google Scholar
5. Kim, JS, Ko, SM, Chee, HK, Shin, JK, Song, MG, Shin, HJ. Relationship between bicuspid aortic valve phenotype, valvular function, and ascending aortic dimensions. J Heart Valve Dis 2014; 23: 406–413.Google Scholar
6. Shin, HJ, Shin, JK, Chee, HK, Kim, JS, Ko, SM. Characteristics of aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve morphology. Eur Radiol 2015; 25: 2103–2114.Google Scholar
7. Forte, A, Della Corte, A, Grossi, M, et al. Early cell changes and TGFbeta pathway alterations in the aortapathy associated with bicuspid aortic valve stenosis. Clin Sci 2013; 124: 97–108.Google Scholar
8. Grewal, N, Gittenberger-de Groot, AC, DeRuiter, MC, et al. Bicuspid aortic valve: phosphorylation of c-Kit and downstream targets are prognostic for future aortopathy. Eur J Cardiothorac Surg 2014; 46: 831–839.Google Scholar
9. Wang, YB, Li, Y, Deng, YB, et al. Enlarged size and impaired elastic properties of the ascending aorta are associated with endothelial dysfunction and elevated plasma matrix metalloproteinase-2 level in patients with bicuspid aortic valve. Ultrasound Med Biol 2018; 44: 955–962.Google Scholar
10. Lang, RM, Badano, LP, Mor-Avi, V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 2015; 16: 233–270.Google Scholar
11. Pettersen, MD, Du, W, Skeens, ME, Humes, RA. Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr 2008; 21: 922–934.Google Scholar
12. Nistri, S, Grande-Allen, J, Noale, M, et al. Aortic elasticity and size in bicuspid aortic valve syndrome. Eur Heart J 2008; 29: 472–479.Google Scholar
13. Celermajer, DS, Sorensen, KE, Gooch, VM, et al. Non-invasive detection of endothelial dysfunction in children and adults at risk of atherosclerosis. Lancet 1992; 340: 1111–1115.Google Scholar
14. Braverman, AC, Güven, H, Beardslee, MA, et al. The bicuspid aortic valve. Outcome of isolated bicuspid aortic valve in childhood. J Pediatr 2010; 157: 445–449.Google Scholar
15. Papagiannis, J. Sudden death due to aortic pathology. Cardiol Young 2017; 27 (S1): S36–S42.Google Scholar
16. Disha, K, Rouman, M, Secknus, MA, Kuntze, T, Girdauskas, E. Are normal-sized ascending aortas at risk of late aortic events after aortic valve replacement for bicuspid aortic valve disease? Interact Cardiovasc Thorac Surg 2016; 22: 465–471.Google Scholar
17. Cury, M, Zeidan, F, Lobato, AC. Aortic disease in the young: genetic aneurysm syndromes, connective tissue disorders, and familial aortic aneurysms and dissections. Int J Vasc Med 2013: 267215.Google Scholar
18. Sievers, HH, Schmidtke, C. A classification system for the bicuspid aortic valve from 304 specimens. J Thorac Cardiovasc Surg 2007; 133: 1226–1233.Google Scholar
19. De Mozzi, P, Longo, UG, Galanti, G, Maffuli, N. Bicuspid aortic valve: a literature review and its impact on sport activity. Br Med Bull 2008; 85: 63–85.Google Scholar
20. Fernandes, SM, Khairy, P, Sanders, SP, Colan, SD. Bicuspid aortic valve morphology and interventions in the young. J Am Coll Cardiol 2007; 22: 2211–2214.Google Scholar
21. Prakash, SK, Bossé, Y, Muehlschlegel, JD, et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications. J Am Coll Cardiol 2014; 64: 832–839.Google Scholar
22. Fernandes, S, Khairy, P, Graham, AD, Colan, DS, Galvin, CS, Sanders, PS. Bicuspid aortic valve and associated aortic dilation in the young. Heart 2012; 98: 1014–1019.Google Scholar
23. Mart, CR, McNerny, BE. Shape of dilated aorta in children with bicuspid aortic valve. Ann Pediatr Cardiol 2013; 6: 126–131.Google Scholar
24. Nistri, S, Sorbo, MD, Marin, M, Palisi, M, Scognamiglio, R, Thiene, G. Aortic root dilation in young men with normally functioning bicuspid aortic valves. Heart 1999; 82: 19–22.Google Scholar
25. Cecconi, M, Manfrin, M, Moraca, A, et al. Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction. Am J Cardiol 2005; 95: 292–294.Google Scholar
26. Tadros, TM, Klein, MD, Shapira, OM. Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119: 880–890.Google Scholar
27. Cotrufo, M, Della Corte, A, De Santo, LS, et al. Different patterns of extracellular matrix protein expression in the convexity and the concavity of the dilated aorta with bicuspid aortic valve: preliminary results. J Thorac Cardiovasc Surg 2005; 130: 504–511.Google Scholar
28. Della Corte, A, Quarto, C, Bancone, C, et al. Spatiotemporal patterns of smooth muscle cell changes in ascending aortic dilatation with bicuspid and tricuspid aortic valve stenosis: focus on cell-matrix signaling. J Thorac Cardiovasc Surg 2008; 135: 8–18; 18.e1–e2.Google Scholar
29. Cripe, L, Andelfinger, G, Martin, LJ, Shooner, K, Benson, DW. Bicuspid aortic valve is heritable. J Am Coll Cardiol 2004; 44: 138–143.Google Scholar
30. Padang, R, Bannon, PG, Jeremy, R, et al. The genetic and molecular basis of bicuspid aortic valve associated thoracic aortopathy: a link to phenotype heterogeneity. Ann Cardiothorac Surg 2013; 2: 83–91.Google Scholar
31. Padang, R, Bagnall, RD, Richmond, DR, Bannon, PG, Semsarian, C. Rare non-synonymous variations in the transcriptional activation domains of GATA5 in bicuspid aortic valve disease. J Mol Cell Cardiol 2012; 53: 277–281.Google Scholar
32. Nathan, DP, Xu, C, Plappert, T, et al. Increased ascending aortic wall stress in patients with bicuspid aortic valves. Ann Thorac Surg 2011; 92: 1384–1389.Google Scholar
33. Girdauskas, E, Disha, K, Borger, MA, Kuntze, T. Relation of bicuspid aortic valve morphology to the dilatation pattern of the proximal aorta: focus on the transvalvular flow. Cardiol Res Pract 2012: 478259.Google Scholar
34. Viscardi, F, Vergara, C, Antiga, L, et al. Comparative finite element model analysis of ascending aortic flow in bicuspid and tricuspid aortic valve. Artif Organs 2010; 34: 1114–1120.Google Scholar
35. John, AS, McDonald-McGinn, DM, Zackai, EH, Goldmuntz, E. Aortic root dilation in patients with 22q11 deletion syndrome. Am J Med Genet A 2009; 149A: 939–942.Google Scholar
36. John, AS, Rychik, J, Khan, M, Yang, W. Goldmuntz E. 22q11.2 deletion syndrome as a risk factor for aortic root dilation in tetralogy of Fallot. Cardiol Young 2014; 24: 303–310.Google Scholar
37. Pisano, C, Maresi, E, Balistreri, CR, et al. Histological and genetic studies in patients with bicuspid aortic valve and ascending aorta complications. Interact Cardiovasc Thorac Surg 2012; 14: 300–306.Google Scholar
38. Cecconi, M, Manfrin, M, Moraca, A, et al. Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction. Am J Cardiol 2005; 95: 292–294.Google Scholar
39. Yasuda, H, Nakatani, S, Stugaard, M, et al. Failure to prevent progressive dilation of ascending aorta by aortic valve replacement in patients with bicuspid aortic valve: comparison with tricuspid aortic valve. Circulation 2003; 108 (Suppl 1): 291–294.Google Scholar
40. Huang, FQ, Le Tan, J. Pattern of aortic dilatation in different bicuspid aortic valve phenotypes and its association with aortic valvular dysfunction and elasticity. Heart Lung Circ 2014; 23: 32–38.Google Scholar
41. Schafer, BM, Lewin, MB, Stout, KK, et al. The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart 2008; 94: 1634–1638.Google Scholar
42. Ikonomidis, JS, Ruddy, JM, Benson, SM, Arroya, J, Brinsa, TA, Stroud, RE. Aortic dilatation with bicuspid aortic valves: cusp fusion correlates to matrix metalloproteinases and inhibitors. Ann Thorac Surg 2012; 93: 457–463.Google Scholar
43. O’Rourke, MF, Hashimoto, J. Mechanical factors in arterial aging: a clinical perspective. J Am Coll Cardiol 2007; 50: 1–13.Google Scholar
44. Pees, C, Michel-Behnke, I. Morphology of the bicuspid aortic valve and elasticity of the adjacent aorta in children. Am J Cardiol 2012; 110: 1354–1360.Google Scholar
45. Weismann, CG, Lombardi, KC, Grell, BS, Northrup, V, Sugeng, L. Aortic stiffness and left ventricular diastolic function in children with well-functioning bicuspid aortic valves. Eur Heart J Cardiovasc Imaging 2016; 17: 225–230.Google Scholar
46. Nistr, S, Grande-Allen, J, Noale, M, et al. Aortic elasticity and size in bicuspid aortic valve syndrome. Eur Heart J 2008; 29: 472–479.Google Scholar
47. Schaefer, BM, Lewin, MB, Stout, KK, Byers, HP, Otto, CM. Usefulness of bicuspid aortic valve phenotype to predict elastic properties of the ascending aorta. Am J Cardiol 2007; 99: 686–690.Google Scholar
48. Ali, OA, Chapman, M, Nguyen, TH, et al. Interactions between inflammatory activation and endothelial dysfunction selectively modulate valve disease progression in patients with bicuspid aortic valve. Heart 2014; 100: 800–805.Google Scholar
49. Tzemos, N, Lyseggen, E, Silversides, C, et al. Endothelial function, carotid-femoral stiffness, and plasma matrix metalloproteinase-2 in men with bicuspid aortic valve and dilated aorta. J Am Coll Cardiol 2010; 55: 660–668.Google Scholar
50. Bonderman, D, Gharehbaghi-Schnell, E, Wollenek, G, Maurer, G, Baumgartner, H, Lang, IM. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation 1999; 99: 2138–2143.Google Scholar
51. Forte, A, Della Corte, A, Grossi, M, et al. Early cell changes and TGFß pathway alterations in the aortopathy associated with bicuspid aortic valve stenosis. Clin Sci 2013; 6: 150–161.Google Scholar
52. Aicher, D, Urbich, C, Zeiher, A, Dimmeler, S, Schäfers, HJ. Endothelial nitric oxide synthase in bicuspid aortic valve disease. Ann Thorac Surg 2007; 83: 1290–1294.Google Scholar