Pulmonary hypertension in sickle cell disease

Kenneth Ataga, Namita Sood, Guy De Gent, Eileen Kelly, Ashley G. Henderson, Susan Jones, Dell Strayhorn, Alice Lail, Susan Lieff, Eugene P. Orringer

Research output: Contribution to journalArticle

117 Citations (Scopus)

Abstract

BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication. METHODS: Sixty patients (<18 years of age; mean [± SD] age, 37 ± 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (<75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.

Original languageEnglish (US)
Pages (from-to)665-669
Number of pages5
JournalAmerican Journal of Medicine
Volume117
Issue number9
DOIs
StatePublished - Nov 1 2004
Externally publishedYes

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Sickle Cell Anemia
Pulmonary Hypertension
Blood Pressure
Fetal Hemoglobin
Logistic Models
Nomograms
Doppler Echocardiography
Respiratory Function Tests
Hematologic Tests
Body Mass Index

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Ataga, K., Sood, N., De Gent, G., Kelly, E., Henderson, A. G., Jones, S., ... Orringer, E. P. (2004). Pulmonary hypertension in sickle cell disease. American Journal of Medicine, 117(9), 665-669. https://doi.org/10.1016/j.amjmed.2004.03.034

Pulmonary hypertension in sickle cell disease. / Ataga, Kenneth; Sood, Namita; De Gent, Guy; Kelly, Eileen; Henderson, Ashley G.; Jones, Susan; Strayhorn, Dell; Lail, Alice; Lieff, Susan; Orringer, Eugene P.

In: American Journal of Medicine, Vol. 117, No. 9, 01.11.2004, p. 665-669.

Research output: Contribution to journalArticle

Ataga, K, Sood, N, De Gent, G, Kelly, E, Henderson, AG, Jones, S, Strayhorn, D, Lail, A, Lieff, S & Orringer, EP 2004, 'Pulmonary hypertension in sickle cell disease', American Journal of Medicine, vol. 117, no. 9, pp. 665-669. https://doi.org/10.1016/j.amjmed.2004.03.034
Ataga K, Sood N, De Gent G, Kelly E, Henderson AG, Jones S et al. Pulmonary hypertension in sickle cell disease. American Journal of Medicine. 2004 Nov 1;117(9):665-669. https://doi.org/10.1016/j.amjmed.2004.03.034
Ataga, Kenneth ; Sood, Namita ; De Gent, Guy ; Kelly, Eileen ; Henderson, Ashley G. ; Jones, Susan ; Strayhorn, Dell ; Lail, Alice ; Lieff, Susan ; Orringer, Eugene P. / Pulmonary hypertension in sickle cell disease. In: American Journal of Medicine. 2004 ; Vol. 117, No. 9. pp. 665-669.
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