Severity of pulmonary hypertension during vaso‐occlusive pain crisis and exercise in patients with sickle cell disease

Summary Pulmonary hypertension is associated with sudden death and is a risk factor for mortality in adult patients with sickle cell disease. The high mortality despite only mild‐to‐moderate increases in pulmonary vascular resistance remains an unresolved paradox. Accordingly, little is known about...

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Bibliographic Details
Published in:British journal of haematology 2007-01, Vol.136 (2), p.319-325
Main Authors: Machado, Roberto F., Kyle Mack, A., Martyr, Sabrina, Barnett, Christopher, MacArthur, Peter, Sachdev, Vandana, Ernst, Inez, Hunter, Lori A., Coles, Wynona A., Nichols, James P., Kato, Gregory J., Gladwin, Mark T.
Format: Article
Language:English
Subjects:
Adult
Analysis of Variance
Anemia, Sickle Cell - complications
Anemia, Sickle Cell - diagnostic imaging
Anemias. Hemoglobinopathies
Biological and medical sciences
Blood Pressure
Cardiac Catheterization
Diseases of red blood cells
Echocardiography
Exercise
Female
haemolysis
Hematologic and hematopoietic diseases
Hemoglobins - analysis
Humans
Hypertension, Pulmonary - diagnostic imaging
Hypertension, Pulmonary - etiology
L-Lactate Dehydrogenase - blood
Male
Medical sciences
Middle Aged
nitric oxide
Pain - diagnostic imaging
Pain - etiology
Pneumology
pulmonary hypertension
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
sickle cell disease
Vascular Diseases - complications
Vascular Diseases - diagnostic imaging
Vascular Resistance
vaso‐occlusive crisis
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Summary:Summary Pulmonary hypertension is associated with sudden death and is a risk factor for mortality in adult patients with sickle cell disease. The high mortality despite only mild‐to‐moderate increases in pulmonary vascular resistance remains an unresolved paradox. Accordingly, little is known about the cardiovascular effects of stressors, such as vaso‐occlusive pain crisis (VOC) and exercise, which may acutely increase pulmonary pressures and impair right heart function. We therefore evaluated pulmonary artery pressures by echocardiogram in 25 patients with sickle cell disease in steady‐state and during VOC, and by right heart catheterisation with exercise in a second cohort of 21 patients to determine whether pulmonary hypertension worsens during acute cardiopulmonary stress. TRV increased during VOC (P 
ISSN:0007-1048
1365-2141
DOI:10.1111/j.1365-2141.2006.06417.x