Pediatric Cardiology

, Volume 29, Issue 2, pp 309–312 | Cite as

Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease

  • O. C. Onyekwere
  • A. Campbell
  • M. Teshome
  • S. Onyeagoro
  • C. Sylvan
  • A. Akintilo
  • S. Hutchinson
  • G. Ensing
  • P. Gaskin
  • G. Kato
  • S. Rana
  • J. Kwagyan
  • V. Gordeuk
  • J. Williams
  • O. Castro
Article

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.

Keywords

Hemolysis Pulmonary disease Sickle cell disease Tricuspid valve regurgitation 

References

  1. 1.
    Castro O (1996) Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 10(6):1289–1230CPubMedCrossRefGoogle Scholar
  2. 2.
    Collins FS, Orringer EP (1982) Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies. Am J Med 73(6):814–821PubMedCrossRefGoogle Scholar
  3. 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 108:1214–1219PubMedCrossRefGoogle Scholar
  4. 4.
    Dantzker DR (1994) Primary pulmonary hypertension. The American experience. Chest 105(Supple 2):26S–28SPubMedGoogle Scholar
  5. 5.
    Denbow CE, Chung EE, Serjeant GR (1993) Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease. Br Heart J 69:536–538PubMedCrossRefGoogle Scholar
  6. 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–194SPubMedCrossRefGoogle Scholar
  7. 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):886–895PubMedCrossRefGoogle Scholar
  8. 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 129:449–464PubMedCrossRefGoogle Scholar
  9. 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 169Google Scholar
  10. 10.
    Norris SL, Johnson C, et al. (1992) Left ventricular filling pressure in sickle cell anemia. J Assoc Acad Minor Phys 3(1):20–3PubMedGoogle Scholar
  11. 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):1639–1644PubMedCrossRefGoogle Scholar
  12. 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):66–76Google Scholar
  13. 13.
    Rich S, Dantzker DR, et al. (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107(2):216–223PubMedGoogle Scholar
  14. 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):1526–1528PubMedCrossRefGoogle Scholar
  15. 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):654–658PubMedCrossRefGoogle Scholar
  16. 16.
    Sutton LL, Castro O, et al. (1994) Pulmonary hypertension in sickle cell disease. Am J Cardiol 74(6):626–628PubMedCrossRefGoogle Scholar
  17. 17.
    Van Enk A, Visschers G, et al. (1992). Maternal death due to sickle cell chronic lung disease. Br J Obstet Gynaecol 99(2):162–163PubMedGoogle Scholar
  18. 18.
    Verresen D, De Backer W, et al. (1990) Pulmonary hypertension and sickle hemoglobinopathy [letter; comment]. Chest 98(4):1042PubMedCrossRefGoogle Scholar
  19. 19.
    Weitzenblum E (2003) Chronic cor pulmonale. Heart 89:225–230PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • O. C. Onyekwere
    • 1
  • A. Campbell
    • 3
  • M. Teshome
    • 3
  • S. Onyeagoro
    • 1
  • C. Sylvan
    • 1
  • A. Akintilo
    • 1
  • S. Hutchinson
    • 3
  • G. Ensing
    • 3
  • P. Gaskin
    • 4
    • 5
  • G. Kato
    • 6
  • S. Rana
    • 4
  • J. Kwagyan
    • 2
  • V. Gordeuk
    • 1
    • 2
  • J. Williams
    • 3
  • O. Castro
    • 1
  1. 1.Center for Sickle Cell DiseaseHoward UniversityWashingtonUSA
  2. 2.General Clinical Research CenterHoward UniversityWashingtonUSA
  3. 3.Center for Sickle Cell DiseaseUniversity of MichiganAnn ArborUSA
  4. 4.Department of Pediatrics and Child HealthHoward UniversityWashingtonUSA
  5. 5.Pediatric CardiologyUniversity of MarylandBaltimoreUSA
  6. 6.Vascular Therapeutics Section, National Heart, Lung and Blood Institute, and the Critical Care Medicine DepartmentClinical Center, National Institutes of HealthBethesdaUSA

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