Skip to main content

Advertisement

SpringerLink
Log in

Evaluation of Cardiac Functions of Patients with Benign Joint Hypermobility Syndrome

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

Abstract

We sought to investigate whether echocardiography with tissue Doppler imaging identifies myocardial dysfunction in children with benign joint hypermobility syndrome (BJHS). This cross-sectional study enrolled 75 children with BJHS and 70 healthy children. We performed detailed echocardiography in individuals with BJHS without inherited connective tissue disorders. Any congenital or acquired cardiac disease was excluded by clinical and echocardiographic examination. Both groups were similar in terms of age, sex, and body mass index. The diameter of the aortic annulus and sinus valsalva were wider in patients with BJHS. There was no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the two groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Deceleration time of early mitral inflow was prolonged in patients with BJHS. Mitral and tricuspid annulus Ea velocity were significantly lower in children with BJHS. Ea, Aa, and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall, and right ventricle free wall were lower in patients with BJHS than in the control group. The E/Ea ratio was greater in patients with BJHS than in the control group. Isovolumic relaxation time and right-ventricular (RV) and left-ventricular (LV) myocardial performance indices (MPIs) were greater in patients with BJHS. This study showed the diastolic dysfunction in patients with BJHS. In addition, we detected increased LV and RV MPI. We believe that BJHS may affect proteins of the myocardial cytoskeleton and extracellular matrix.

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

Similar content being viewed by others

References

  1. Aessopos A, Farmakis D, Taktikou H, Loukopoulos D (2000) Doppler-determined peak systolic tricuspid pressure gradient in persons with normal pulmonary function and tricuspid regurgitation. J Am Soc Echocardiogr 13:645–649

    Article  CAS  PubMed  Google Scholar 

  2. Biro F, Gewanter HL, Baum J (1983) The hypermobility syndrome. Pediatrics 72:701–706

    CAS  PubMed  Google Scholar 

  3. Child AH (1986) Joint hypermobility syndrome. Inherited disorder of collagen synthesis. J Rheumatol 13:239–243

    CAS  PubMed  Google Scholar 

  4. Fikree A, Aziz Q, Grahame R (2013) Joint hypermobility syndrome. Rheum Dis Clin North Am 39(2):419–430

    Article  PubMed  Google Scholar 

  5. Grahame R (2000) The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 27:1777–1779

    CAS  PubMed  Google Scholar 

  6. Hakim AJ, Cherkas LF, Grahame R, Spector TD, MacGregor AJ (2004) The genetic epidemiology of joint hypermobility: a population study of female twins. Arthritis Rheum 50:2640–2644

    Article  PubMed  Google Scholar 

  7. Karatasakis GT, Karagounis LA, Kalyvas PA, Manginas A, Athanassopoulos GD, Aggelakas S et al (1998) Prognostic significance of echocardiography estimated right ventricular shortening in advanced heart failure. Am J Cardiol 82:329–334

    Article  CAS  PubMed  Google Scholar 

  8. 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 

  9. 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 

  10. Magnusson SP (2001) Viscoelastic properties and flexibility of the human muscle-tendon unit in benign joint hypermobility syndrome. J Rheumatol 28:2720–2725

    CAS  PubMed  Google Scholar 

  11. McDonnell NB, Gorman BL, Mandel KW, Schurman SH, Assanah-Carroll A, Mayer SA et al (2002) Diastolic subclinical primary alterations in Marfan syndrome and Marfan-related disorders. Clin Cardiol 25:416–420

    Article  Google Scholar 

  12. McDonnell NB, Gorman BL, Mandel KW, Schurman SH, Assanah-Carroll A, Mayer SA et al (2006) Echocardiographic findings in classical and hypermobile Ehlers-Danlos syndromes. Am J Med Genet A 140(2):129–136

    Article  PubMed  Google Scholar 

  13. Migliaccio S, Barbaro G, Fornari R, Di Lorenzo G, Celli M, Lubrano C et al (2009) Impairment of diastolic function in adult patients affected by osteogenesis imperfecta clinically asymptomatic for cardiac disease: casuality or causality? Int J Cardiol 131:200–203

    Article  PubMed  Google Scholar 

  14. Pai RG, Bodenheimer MM, Pai SM, Koss JH, Adamick RD (1991) Usefulness of systolic excursion of the mitral annulus as an index of left ventricular systolic function. Am J Cardiol 67:222–224

    Article  CAS  PubMed  Google Scholar 

  15. Pearlman AS, Gardin JM, Martin RP, Parisi AF, Popp RL, Quinones MA et al (1987) Guidelines for optimal physician training in echocardiography. Recommendations of the American Society of Echocardiography Committee for Physician Training in Echocardiography. Am J Cardiol 60:158–163

    Article  CAS  PubMed  Google Scholar 

  16. Rybczynski M, Koschyk DH, Aydin MA, Robinson PN, Brinken T, Franzen O et al (2007) Tissue Doppler imaging identifies myocardial dysfunction in adults with Marfan syndrome. Clin Cardiol 30:19–24

    Article  PubMed  Google Scholar 

  17. Savolainen S, Nisula L, Keto P, Hekali P, Viitasalo M et al (1994) Left ventricular function in children with the Marfan syndrome. Eur Heart J 15:625–630

    CAS  PubMed  Google Scholar 

  18. Simpson MR (2006) Benign joint hypermobility syndrome: evaluation, diagnosis, and management. J Am Osteopath Assoc 106:531–536

    PubMed  Google Scholar 

  19. Tei C, Dujardin K, Hodge D, Kyle R, Tajik A, Seward J (1996) Doppler index combining systolic and diastolic myocardial performance: clinical value in cardiac amyloidosis. J Am Coll Cardiol 28:658–664

    Article  CAS  PubMed  Google Scholar 

  20. Tei C, Dujardin KS, Hodge DO, Bailey KR, McGoon MD, Tajik AJ et al (1996) Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr 9:838–847

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Balikesir Ataturk Hospital, Yıldız Mahallesi, Soma Caddesi, Balikesir, Turkey

    Sevket Balli

  2. Department of Cardiology, Balikesir Ataturk Hospital, Balikesir, Turkey

    Mehmet Zafer Aydın

  3. Department of Rheumatology, Balikesir Ataturk Hospital, Balikesir, Turkey

    Vedat Gerdan

  4. Department of Pediatric Cardiology, Faculty of Medicine, Yuzuncu Yil University, Van, Turkey

    İbrahim Ece

  5. Department of Pediatric Cardiology, Faculty of Medicine, Cumhuriyet University, Sivas, Turkey

    Mehmet Burhan Oflaz

  6. Department of Pediatric Cardiology, Mersin Children’s Hospital, Mersin, Turkey

    Ayse Esin Kibar

  7. Department of Pediatrics, Balikesir Ataturk Hospital, Balikesir, Turkey

    Eylem Sen Dalkiran

Authors
  1. Sevket Balli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mehmet Zafer Aydın
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vedat Gerdan
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. İbrahim Ece
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mehmet Burhan Oflaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ayse Esin Kibar
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Eylem Sen Dalkiran
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sevket Balli.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Balli, S., Aydın, M.Z., Gerdan, V. et al. Evaluation of Cardiac Functions of Patients with Benign Joint Hypermobility Syndrome. Pediatr Cardiol 35, 374–379 (2014). https://doi.org/10.1007/s00246-013-0785-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-013-0785-2

Keywords

Search

Navigation