Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension

American Journal of Respiratory and Critical Care Medicine
Anastasia AnthiMark T Gladwin

Abstract

Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population. To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH. Patients with SCD with PH (n = 26) were compared with control subjects with SCD but without PH (n = 17), matched for age, hemoglobin levels, and fetal hemoglobin levels. Upon catheterization, 54% of the patients with PH had pulmonary arterial hypertension, and 46% had pulmonary venous hypertension. When compared with control subjects, patients with PH exhibited lower six-minute-walk distance (435 +/- 31 vs. 320 +/- 20 m, p = 0.002) and oxygen consumption (50 +/- 3% vs. 41 +/- 2% of predicted, p = 0.02), and also had mild restrictive lung disease and more perfusion abnormalities on radionuclide lung scans. The six-minute-walk distance in this population inversely correlated with tricuspid regurgitant jet velocity (r = -0.55, p < 0.001), and mean pulmonary artery pressure (r = -0.57, p < 0.001), and directly correlated with maximal oxygen consumption (r = 0.49, p = 0.004), even after adjustment for hemoglobin, supp...Continue Reading

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Related Concepts

Anemia, Sickle Cell
Cardiac Output
Cardiopulmonary Exercise Test
Cirrhosis
Cardiac Catheterization Procedures
Pulmonary Hypertension
Lung
Oxygen Consumption
Respiratory Function Tests
Tomography, X-Ray Computerized Axial

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