Pulmonary hypertension in sickle cell disease.
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 (> or =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 (> or =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.


CONCLUSIONS
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.
Ataga
Kenneth I
KI
Department of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA. kataga@med.unc.edu
Sood
Namita
N
De Gent
Guy
G
Kelly
Eileen
E
Henderson
Ashley G
AG
Jones
Susan
S
Strayhorn
Dell
D
Lail
Alice
A
Lieff
Susan
S
Orringer
Eugene P
EP
D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D003430:Cross-Sectional Studies; D015150:Echocardiography, Doppler; D005260:Female; D006403:Hematologic Tests; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D016015:Logistic Models; D008297:Male; D015995:Prevalence; D012129:Respiratory Function Tests