Skip to main content
SpringerLink
Log in

Clinical Outcome, Valve Dysfunction, and Progressive Aortic Dilation in a Pediatric Population With Isolated Bicuspid Aortic Valve

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The aim of this study was to explore the medium-term clinical outcome and the risk of progression of aortic valve disease and aortic dilation in pediatric patients with isolated bicuspid aortic valve (BAV). 179 pediatric patients with isolated BAV were prospectively followed from January 1995 to December 2010. Patients with severe valve dysfunction at baseline were excluded. Clinical outcome included cardiac death, infective endocarditis, aortic complications, cardiac surgery and percutaneous valvuloplasty. Echocardiographic endpoints were: progression of aortic stenosis (AS) or regurgitation (AR) and progressive aortic enlargement at different levels of the aortic root, evaluated as z-score. The median age at diagnosis was 7.8 [2.7–12.0] years. After a median followup of 5.4 [2.3–9.2] years, all patients were alive. The clinical endpoint occurred in 4 (2.2 %) patients (0.41 events per 100 patient-years). A progression of AS and AR was observed in 9 (5.0 %) and 29 (16.2 %) patients, respectively. The z-scores at the end of follow-up were not significantly different from baseline at the annulus, Valsalva sinuses and sinotubular junction, whereas a slight increase was observed at the level of the ascending aorta (1.9 vs 1.5, p = 0.046). Significant progressive aortic dilation occurred in a minority of patients (10.6, 5.6, 9.5, and 19.0 % respectively). The clinical outcome in pediatric patients with isolated BAV is favourable and the progression of aortic valve dysfunction and aortic dilation is relatively slow. These findings may be taken into account to better guide risk assessment and clinical follow-up in these patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Aicher D, Kunihara T, Abou Issa O, Brittner B, Gräber S, Shäfers HJ (2011) Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation 123:178–185

    Article  PubMed  Google Scholar 

  2. Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP, Iung B, Otto CM, Pellikka PA, Quiñones M (2009) Echocardiographic assessment of valve stenosis: American Society of Echocardiography/European Association of Echocardiography recommendations for clinical practice. J Am Soc Echocardiogr 22:1–23

    Article  PubMed  Google Scholar 

  3. Beppu S, Suzuki S, Matsude H et al (1993) Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves. Am J Cardiol 71:322–327

    Article  CAS  PubMed  Google Scholar 

  4. Beroukhim RS, Roosevelt G, Yetman AT (2006) Comparison of the pattern of aortic dilation in children with the Marfan’s syndrome versus children with a bicuspid aortic valve. Am J Cardiol 98:1094–1095

    Article  PubMed  Google Scholar 

  5. Cotrufo M, Della Corte A (2009) The association of bicuspid aortic valve disease with asymmetric dilation of the tubular ascending aorta: identification of a definite syndrome. J Cardiovasc Med 10:291–297

    Article  Google Scholar 

  6. Daniels CJ (2008) The adolescent and adult with congenital heart disease. In: Moss AJ, Adams LD (eds) Heart disease in infants, children and adolescents. Lippincott W&W, Philadelphia, pp 1370–1407

    Google Scholar 

  7. De Mozzi P, Longo UG, Galanti G, Maffulli N (2008) Bicuspid aortic valve: a literature review and its impact on sport activity. Br Med Bull 85:63–85

    Article  PubMed  Google Scholar 

  8. Evangelista A, Flachskampf FA, Erbel R, Antonini-Canterin F, Vlachopoulos C, Rocchi G, Sicari R et al (2010) Echocardiography in aortic diseases: European Association of Echocardiography recommendations for clinical practice. Eur J Echocardiogr 11:645–658

    Article  PubMed  Google Scholar 

  9. Fernandes SM, Sanders SP, Khairi P et al (2004) Morphology of bicuspid aortic valve in children and adolescents. J Am Coll Cardiol 44:1648–1651

    Article  PubMed  Google Scholar 

  10. Fernandes SM, Khairy P, Sanders SP, Colan SD (2007) Bicuspid aortic valve: morphology and interventions in the young. J Am Coll Cardiol 49:2211–2214

    Article  PubMed  Google Scholar 

  11. Fernandes S, Khairy P, Graham DA, Colan SD et al (2012) Bicuspid aortic valve and associated aortic dilation in the young. Heart 98:1014–1019

    Article  PubMed  Google Scholar 

  12. Gautier M, Detaint D, Fermanian C, Aegerter P, Delorme G, Arnoult F, Milleron O, Raoux F, Stheneur C, Boileau C, Vahanian A, Jondeau G (2010) Nomograms for aortic root diameters in children using two-dimensional echocardiography. Am J Cardiol 105:888–894

    Article  PubMed  Google Scholar 

  13. Guntheroth WG (2008) A critical review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta. Am J Cardiol 102:107–110

    Article  PubMed  Google Scholar 

  14. Gurvitz M, Chang RK, Drant S, Allada V (2004) Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol 94:1337–1340

    Article  PubMed  Google Scholar 

  15. Hahn RT, Roman MJ, Mogtader AH, Devereux RB (1992) Association of aortic dilation with regurgitant, stenotic, and functionally normal bicuspid aortic valves. J Am Coll Cardiol 19:283–288

    Article  CAS  PubMed  Google Scholar 

  16. Hohmann D, Bertram H, Schieffer B, Wessel A (2011) Acute myocardial infarction in a 16-year-old girl caused by infective endocarditis of a bicuspid aortic valve. Pediatr Cardiol 32:534–535

    Article  PubMed  Google Scholar 

  17. Keane MG, Wiegers SE, Pleppert T, Pochettino A, Bavaria JE, Sutton MG (2000) Bicuspid aortic valves are associated with aortic dilation out of proportion to coexistent valvular lesions. Circulation 102:35–39

    Article  Google Scholar 

  18. Kim Woo Shin, Kang Seok Hyung, Lee Shin A, Ryu Min Sun, Park Seong-Hoon (2011) A case of staphylococcal tricuspid valve endocarditis with para-aortic abscess in a patient with bicuspid aortic valve. Korean Circ J 41:482–485

    Article  PubMed Central  PubMed  Google Scholar 

  19. La Canna G, Ficarra E, Tsagalau E, Nardi M, Morandini A, Chieffo A, Maisano F, Alfieri O (2006) Progressive rate of ascending aortic dilation in patients with normally functioning bicuspid and tricuspid aortic valves. Am J Cardiol 98:249–253

    Article  PubMed  Google Scholar 

  20. Lancellotti P, Tribouilloy C, Hagendorff A, Moura L, Popescu BA, Agricola E, Monin JL, Pierard LA, Badano L, Zamorano JL (2010) European Association of Echocardiography recommendations for the assessment of valvular regurgitation: Part 1. Aortic and pulmonary regurgitation (native valve disease). Eur J Echocardiogr 11:223–244

    Article  PubMed  Google Scholar 

  21. Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA et al (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18:1440–1463

    Article  PubMed  Google Scholar 

  22. Mahle WT, Sutherland JL, Frias PA (2010) Outcome of isolated bicuspid valve in childhood. J Pediatr 157:445–449

    Article  PubMed  Google Scholar 

  23. Mi Y, Rädle-Hurst T, Rentzsch A, Aicher D, Schäfers HJ, Abdul-Khaliq H (2013) The novel two-dimensional strain reflects improvement and remodeling of left-ventricular function better than conventional echocardiographic parameters after aortic valve repair in pediatric patients. Pediatr Cardiol 34:30–38

    Article  PubMed  Google Scholar 

  24. Michelena HL, Desjardins VA, Avierinos J-F, Russo A, Nkomo VT, Sundt TM, Pellikka PA, Tajik AJ, Enriquez-Sarano M (2008) Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 117:2776–2784

    Article  PubMed Central  PubMed  Google Scholar 

  25. Mordi I, Tzemos N (2012) Bicuspid aortic valve disease: a comprehensive review. Cardiol Res Pract. doi:10.1155/2012/196037

  26. NHLBI National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576

    Google Scholar 

  27. Niwa K (2013) Aortopathy in congenital heart disease in adults: aortic dilatation with decreased aortic elasticity that impacts negatively on left ventricular function. Korean Circ J 43:215–220

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  28. Oulego-Erroz I, Alonso-Quintela P, Mora-Matilla M, Gautreaux Minaya S, Lapeña-López de Armentia S (2012) Ascending aorta elasticity in children with isolated bicuspid aortic valve. Int J Cardiol 168(2):1143–1146

    Article  PubMed  Google Scholar 

  29. Pees C, Michel-Behnke I (2012) Morphology of the bicuspid aortic valve and elasticity of the adjacent aorta in children. Am J Cardiol 110:1354–1360

    Article  PubMed  Google Scholar 

  30. Roberts CS, Roberts WC (1991) Dissection of the aorta associated with congenital malformations of the aortic valve. J Am Coll Cardiol 17:712–716

    Article  CAS  PubMed  Google Scholar 

  31. Roberts WC, Vowels TJ, Ko JM (2012) Natural history of adults with congenitally malformed aortic valves (unicuspid or bicuspid). Medicine 91:287–308

    Article  PubMed  Google Scholar 

  32. Sadron Blaye-Felice MA, Séguéla PE, Arnaudis B, Dulac Y, Lepage B, Acar P (2012) Usefulness of three-dimensional transthoracic echocardiography for the classification of congenital bicuspid aortic valve in children. Eur Heart J Cardiovasc Imaging 13:1047–1052

    Article  PubMed  Google Scholar 

  33. Sawaimoon SK, Tadhav MV, Rane SR, Sagale M, Khedhar B (2006) Aortic dissection and bicuspid aortic valve: an autopsy study. Indian J Pathol Microbiol 49:327–329

    PubMed  Google Scholar 

  34. Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH et al (2008) The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart 94:1634–1638

    Article  CAS  PubMed  Google Scholar 

  35. Spagnolo A, Giussani M, Ambruzzi AM, Bianchetti M, Maringhini S, Matteucci MC et al (2013) Focus on prevention, diagnosis, and treatment of hypertension in children and adolescents. Ital J Pediatr 39:20

    Article  PubMed Central  PubMed  Google Scholar 

  36. Steinberger J, Maller JH, Berry JM, Sinaiko AR (2000) Echocardiographic diagnosis of heart disease in apparently healthy adolescents. Pediatrics 105:815–818

    Article  CAS  PubMed  Google Scholar 

  37. Tadros TM, Klein MD, Shapira OM (2009) Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation 119:880–890

    Article  PubMed  Google Scholar 

  38. Teo LL, Cannell T, Babu-Narayan SV, Hughes M, Mohiaddin RH (2011) Prevalence of associated cardiovascular abnormalities in 500 patients with aortic coarctation referred for cardiovascular magnetic resonance imaging to a tertiary center. Pediatr Cardiol 32:1120–1127

    Article  PubMed  Google Scholar 

  39. Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S, Jamorski MT et al (2008) Outcomes in adults with bicuspid aortic valves. JAMA 300:1317–1325

    Article  CAS  PubMed  Google Scholar 

  40. Warren AE, Boyd ML, O’Connel C, Dodds L (2006) Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression, and risk factors. Heart 92:1496–1500

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  41. Zamorano JL, Badano LP, Bruce C, Chan KL, Gonçalves A, Hahn RT et al (2011) EAE/ASE recommendations for the use of echocardiography in new transcatheter interventions for valvular heart disease. Eur Heart J 32:2189–2214

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Pediatric Cardiology Unit, Meyer Hospital, Viale Pieraccini 24, Florence, Italy

    Gaia Spaziani, Silvia Favilli, Veronica Fibbi, Lorenzo Buonincontri, Iva Pollini & Enrico Chiappa

  2. Cardiology Unit, S. Maria Annunziata Hospital, Florence, Italy

    Piercarlo Ballo & Alfredo Zuppiroli

Authors
  1. Gaia Spaziani
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Piercarlo Ballo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Silvia Favilli
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Veronica Fibbi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Lorenzo Buonincontri
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Iva Pollini
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Alfredo Zuppiroli
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Enrico Chiappa
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gaia Spaziani.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Spaziani, G., Ballo, P., Favilli, S. et al. Clinical Outcome, Valve Dysfunction, and Progressive Aortic Dilation in a Pediatric Population With Isolated Bicuspid Aortic Valve. Pediatr Cardiol 35, 803–809 (2014). https://doi.org/10.1007/s00246-013-0856-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-013-0856-4

Keywords

Search

Navigation