Pulmonary hypertension in sickle cell disease

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. Sixty pa...

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Veröffentlicht in:The American journal of medicine 2004-11, Vol.117 (9), p.665-669
Hauptverfasser: Ataga, Kenneth I., Sood, Namita, De Gent, Guy, Kelly, Eileen, Henderson, Ashley G., Jones, Susan, Strayhorn, Dell, Lail, Alice, Lieff, Susan, Orringer, Eugene P.
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container_end_page 669
container_issue 9
container_start_page 665
container_title The American journal of medicine
container_volume 117
creator Ataga, Kenneth I.
Sood, Namita
De Gent, Guy
Kelly, Eileen
Henderson, Ashley G.
Jones, Susan
Strayhorn, Dell
Lail, Alice
Lieff, Susan
Orringer, Eugene P.
description 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. 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. 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. 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.
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subjects Adult
Anemia, Sickle Cell - complications
Anemia, Sickle Cell - diagnosis
Anemia, Sickle Cell - epidemiology
Biological and medical sciences
Blood pressure
Cross-Sectional Studies
Echocardiography, Doppler
Female
General aspects
Health risk assessment
Hematologic Tests
Humans
Hypertension
Hypertension, Pulmonary - diagnosis
Hypertension, Pulmonary - epidemiology
Hypertension, Pulmonary - etiology
Logistic Models
Male
Medical sciences
Pneumology
Prevalence
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
Respiratory Function Tests
Sickle cell anemia
title Pulmonary hypertension in sickle cell disease
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