SpringerLink
Log in

Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease

  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV ≥2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV ≥3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD.

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

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

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. Castro O (1996) Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 10(6):1289–1230C

    Article  PubMed  CAS  Google Scholar 

  2. Collins FS, Orringer EP (1982) Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies. Am J Med 73(6):814–821

    Article  PubMed  CAS  Google Scholar 

  3. Covitz W, Espeland M, Gallagher D, et al. (1995) The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest 108:1214–1219

    Article  PubMed  CAS  Google Scholar 

  4. Dantzker DR (1994) Primary pulmonary hypertension. The American experience. Chest 105(Supple 2):26S–28S

    PubMed  CAS  Google Scholar 

  5. Denbow CE, Chung EE, Serjeant GR (1993) Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease. Br Heart J 69:536–538

    Article  PubMed  CAS  Google Scholar 

  6. Galie N, Manes A, Uguccioni L, Serafini F, De Rosa M, Branzi A, et al. (1998) Primary pulmonary hypertension: Insights into pathogenesis from epidemiology. Chest 114(Suppl 3):184S–194S

    Article  PubMed  CAS  Google Scholar 

  7. Gladwin MT, Sachdev V, Rodgers GR, Castro O, Ognibene FP, et al. (2004) Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 350(9):886–895

    Article  PubMed  CAS  Google Scholar 

  8. Machado RF, Gladwin MT (2005) Chronic sickle cell lung disease: New insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension. Br J Haematol 129:449–464

    Article  PubMed  CAS  Google Scholar 

  9. Morris CR, Gardner J, Hagar W, Vichinsky EP (2004) Pulmonary hypertension in sickle cell disease: A common complication for both adults and children (abstr). In: The 27th Annual Meeting of the National Sickle Cell Disease Program, Los Angeles, CA, p 169

  10. Norris SL, Johnson C, et al. (1992) Left ventricular filling pressure in sickle cell anemia. J Assoc Acad Minor Phys 3(1):20–3

    PubMed  CAS  Google Scholar 

  11. Platt O S, Brambilla DJ, et al. (1994) Mortality in sickle cell disease. Life expectancy and risk factors for early death [see comments]. N Engl J Med 330(23):1639–1644

    Article  PubMed  CAS  Google Scholar 

  12. Powars D, Weidman JA, et al. (1988). Sickle cell chronic lung disease: Prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 67(1):66–76

    CAS  Google Scholar 

  13. Rich S, Dantzker DR, et al. (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107(2):216–223

    PubMed  CAS  Google Scholar 

  14. Simmons BE, Santhanam V, et al. (1988) Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Arch Intern Med 148(7):1526–1528

    Article  PubMed  CAS  Google Scholar 

  15. Suell MN, Bezold LI, Okcu MF, et al. (2005). Increased pulmonary artery pressures among adolescents with sickle cell disease. J Pediatr Hematol Oncol 27(12):654–658

    Article  PubMed  Google Scholar 

  16. Sutton LL, Castro O, et al. (1994) Pulmonary hypertension in sickle cell disease. Am J Cardiol 74(6):626–628

    Article  PubMed  CAS  Google Scholar 

  17. Van Enk A, Visschers G, et al. (1992). Maternal death due to sickle cell chronic lung disease. Br J Obstet Gynaecol 99(2):162–163

    PubMed  Google Scholar 

  18. Verresen D, De Backer W, et al. (1990) Pulmonary hypertension and sickle hemoglobinopathy [letter; comment]. Chest 98(4):1042

    Article  PubMed  CAS  Google Scholar 

  19. Weitzenblum E (2003) Chronic cor pulmonale. Heart 89:225–230

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We thank the staff members, patients, and parents who took part in this study.

Author information

Authors and Affiliations

  1. Center for Sickle Cell Disease, Howard University, Washington, DC, USA

    O. C. Onyekwere, S. Onyeagoro, C. Sylvan, A. Akintilo, V. Gordeuk & O. Castro

  2. General Clinical Research Center, Howard University, Washington, DC, USA

    J. Kwagyan & V. Gordeuk

  3. Center for Sickle Cell Disease, University of Michigan, Ann Arbor, MI, USA

    A. Campbell, M. Teshome, S. Hutchinson, G. Ensing & J. Williams

  4. Department of Pediatrics and Child Health, Howard University, Washington, DC, USA

    P. Gaskin & S. Rana

  5. Pediatric Cardiology, University of Maryland, Baltimore, MD, USA

    P. Gaskin

  6. Vascular Therapeutics Section, National Heart, Lung and Blood Institute, and the Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD, USA

    G. Kato

Authors
  1. O. C. Onyekwere
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Campbell
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Teshome
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. S. Onyeagoro
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. C. Sylvan
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A. Akintilo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. S. Hutchinson
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. G. Ensing
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. P. Gaskin
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. G. Kato
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. S. Rana
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. J. Kwagyan
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. V. Gordeuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. J. Williams
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. O. Castro
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to O. C. Onyekwere.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Onyekwere, O.C., Campbell, A., Teshome, M. et al. Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease. Pediatr Cardiol 29, 309–312 (2008). https://doi.org/10.1007/s00246-007-9018-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-007-9018-x

Keywords

Search

Navigation