Skip to main content
SpringerLink
Log in

Echocardiographic findings in patients with sickle cell disease

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Pulmonary hypertension is a complication of sickle cell disease that is associated with increased mortality. Whether this complication is associated with hemolysis has been questioned. Systolic pulmonary artery blood pressure can be estimated from echocardiography-determined tricuspid regurgitation velocity (TRV). A velocity of 2.5 m/s or higher suggests possible pulmonary hypertension. A retrospective review of hospital records from adult patients with sickle cell disease undergoing echocardiography in 2006 and 2007 was performed at a tertiary level hospital. Echocardiographic, demographic, and clinical laboratory data were collected. Echocardiographic results were available for 105 adult sickle cell patients. Of these, 62 (59%) had a TRV ≥2.5 m/s and 24 (22.8%) had a TRV ≥3.0 m/s. Mitral valve regurgitation was observed in 44% and left ventricular abnormalities (defined by either hypertrophy or dilation) in 28% of cases. Elevated TRV had independent and significant associations with greater age, higher serum lactate dehydrogenase (LDH) concentration, and lower hemoglobin concentration. We confirmed that elevated TRV is common among hospital-based adults with sickle cell disease. Significant, independent associations were found with both elevated LDH concentration and degree of anemia, suggesting that hemolytic and other mechanisms contribute to pulmonary hypertension in patients with sickle cell disease.

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. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP (2004) Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 350:886–895

    Article  CAS  PubMed  Google Scholar 

  2. Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF (1994) Pulmonary hypertension in sickle cell disease. Am J Cardiol 74:626–628

    Article  CAS  PubMed  Google Scholar 

  3. Castro O (1996) Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 10:1289–1303

    Article  CAS  PubMed  Google Scholar 

  4. Ambrusko SJ, Gunawardena S, Sakara A, Windsor B, Lanford L, Michelson P, Krishnamurti L (2006) Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatr Blood Cancer 47:907–913

    Article  PubMed  Google Scholar 

  5. Ataga KI, Moore CG, Jones S, Olajide O, Strayhorn D, Hinderliter A, Orringer EP (2006) Pulmonary hypertension in patients with sickle cell disease: a longitudinal study. Br J Haematol 134:109–115

    Article  PubMed  Google Scholar 

  6. De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ (2008) Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Hematol 83:19–25

    Article  PubMed  Google Scholar 

  7. Powars D, Weidman JA, Odom-Maryon T, Niland JC, Johnson C (1988) Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 67:66–76

    CAS  Google Scholar 

  8. Qureshi N, Joyce JJ, Qi N, Chang RK (2006) Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance. J Pediatr 149:23–27

    Article  PubMed  Google Scholar 

  9. Akgul F, Yalcin F, Babayigit C, Seyfeli E, Seydaliyeva T, Gali E (2006) Right ventricular and pulmonary function in sickle cell disease patients with pulmonary hypertension. Pediatr Cardiol 27:440–446

    Article  PubMed  Google Scholar 

  10. Zilberman MV, Du W, Das S, Sarnaik SA (2007) Evaluation of left ventricular diastolic function in pediatric sickle cell disease patients. Am J Hematol 82:433–438

    Article  PubMed  Google Scholar 

  11. Kane A, Mbengue-Dieye A, Dieye O, Sylla A, Sall G, Diouf SM, Kuakuvi N (2001) Echocardiographic aspects in pediatric patients with sickle cell disease. Arch Pediatr 8:707–712

    Article  CAS  PubMed  Google Scholar 

  12. Ahmed S, Siddiqui AK, Sadiq A, Shahid RK, Patel DV, Russo LA (2004) Echocardiographic abnormalities in sickle cell disease. Am J Hematol 76:195–198

    Article  PubMed  Google Scholar 

  13. Hayag-Barin JE, Smith RE, Tucker FC Jr (1998) Hereditary spherocytosis, thrombocytosis, and chronic pulmonary emboli: a case report and review of the literature. Am J Hematol 57:82–84

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  15. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, Joussef J, Rombos J, Loukopoulos D (2001) Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 97:3411–3416

    Article  CAS  PubMed  Google Scholar 

  16. Heller PG, Grinberg AR, Lencioni M, Molina MM, Roncoroni AJ (1992) Pulmonary hypertension in paroxysmal nocturnal hemoglobinuria. Chest 102:642–643

    Article  CAS  PubMed  Google Scholar 

  17. Pashankar FD, Carbonella J, Bazzy-Asaad A, Friedman A (2008) Prevalence and risk factors of elevated pulmonary artery pressures in children with sickle cell disease. Pediatrics 121:777–782

    Article  PubMed  Google Scholar 

  18. Castro O, Hoque M, Brown BD (2003) Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood 101:1257–1261

    Article  CAS  PubMed  Google Scholar 

  19. Machado RF, Mack AK, Martyr S, Barnett C, Macarthur P, Sachdev V, Ernst I, Hunter LA, Coles WA, Nichols JP, Kato GJ, Gladwin MT (2007) Severity of pulmonary hypertension during vaso-occlusive pain crisis and exercise in patients with sickle cell disease. Br J Haematol 136:319–325

    Article  PubMed  Google Scholar 

  20. Simmons BE, Santhanam V, Castaner A, Rao KR, Sachdev N, Cooper R (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 148:1526–1528

    Article  CAS  PubMed  Google Scholar 

  21. Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Cannon RO 3rd, Schechter AN, Gladwin MT (2002) Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med 8:1383–1389

    Article  CAS  PubMed  Google Scholar 

  22. Liem RI, Young LT, Thompson AA (2007) Tricuspid regurgitant jet velocity is associated with hemolysis in children and young adults with sickle cell disease evaluated for pulmonary hypertension. Haematologica 92:1549–1552

    Article  PubMed  Google Scholar 

  23. Gladwin MT, Shelhamer JH, Ognibene FP, Pease-Fye ME, Nichols JS, Link B, Patel DB, Jankowski MA, Pannell LK, Schechter AN, Rodgers GP (2002) Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease. Br J Haematol 116:436–444

    Article  CAS  PubMed  Google Scholar 

  24. Kato GJ, McGowan V, Machado RF, Little JA, Taylor JT, Morris CR, Nichols JS, Wang X, Poljakovic M, Morris SM Jr, Gladwin MT (2006) Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 107:2279–2285

    Article  CAS  PubMed  Google Scholar 

  25. Olson JS, Foley EW, Rogge C, Tsai AL, Doyle MP, Lemon DD (2004) No scavenging and the hypertensive effect of hemoglobin-based blood substitutes. Free Radic Biol Med 36:685–697

    Article  CAS  PubMed  Google Scholar 

  26. Gerry JL, Bulkley BH, Hutchins GM (1978) Clinicopathologic analysis of cardiac dysfunction in 52 patients with sickle cell anemia. Am J Cardiol 42:211–216

    Article  CAS  PubMed  Google Scholar 

  27. Fox ER, Wilson RS, Penman AD, King JJ, Towery JG, Butler KR, McMullan MR, Skelton TN, Mosley TH, Taylor HA (2007) Epidemiology of pure valvular regurgitation in the large middle-aged African American cohort of the atherosclerosis risk in communities study. Am Heart J 154:1229–1234

    Article  PubMed  Google Scholar 

  28. Nkomo VT, Arnett DK, Benjamin EJ, Liebson PR, Hutchinson RG, Skelton TN (2004) Left ventricular structure and systolic function in African Americans: the Atherosclerosis Risk in Communities (ARIC) study. Ethn Dis 14:483–488

    PubMed  Google Scholar 

Download references

Acknowledgments

This study was supported in part by grant nos. 2 R25 HL003679-08 and 1 R01 HL079912-02 from NHLBI and by Howard University GCRC grant no 2MOI RR10284-10 from NCRR, NIH, Bethesda, MD.

Author information

Authors and Affiliations

  1. Center for Sickle Cell Disease and Department of Medicine, Howard University, 1840 7th Street, NW, Room # 201, Washington, DC, 20001, USA

    Shahla G. Naoman, Mehdi Nouraie, Oswaldo L. Castro, Catherine Nwokolo, Margaret Fadojutimi-Akinsiku, Sharmin Diaz & Victor R. Gordeuk

  2. Division of Cardiology, Howard University, 2041 Georgia Avenue, NW, Suite 6C03, Washington, DC, 20060, USA

    Georgia Willie-Carnegie & Newton Andrews

Authors
  1. Shahla G. Naoman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mehdi Nouraie
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Oswaldo L. Castro
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Catherine Nwokolo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Margaret Fadojutimi-Akinsiku
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Sharmin Diaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Georgia Willie-Carnegie
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Newton Andrews
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Victor R. Gordeuk
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mehdi Nouraie.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Naoman, S.G., Nouraie, M., Castro, O.L. et al. Echocardiographic findings in patients with sickle cell disease. Ann Hematol 89, 61–66 (2010). https://doi.org/10.1007/s00277-009-0764-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-009-0764-0

Keywords

Search

Navigation