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Published ahead of print on March 22, 2007, doi:10.1164/rccm.200610-1498OC

Am. J. Respir. Crit. Care Med., Volume 175, Number 12, June 2007, 1272-1279

A more recent version of this article appeared on June 15, 2007
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Submitted on October 18, 2006
Accepted on March 19, 2007

Hemodynamic and Functional Assessment of Sickle Cell Disease Patients with Pulmonary Hypertension

Anastasia Anthi1, Roberto F Machado1*, Maria L Jison2, Angelo M Taveira-DaSilva3, Lewis J Rubin4, Lori Hunter5, Christian J Hunter1, Wynona Coles1, James Nichols1, Nilo A Avila6, Vandana Sachdev7, Clara C Chen8, and Mark T Gladwin1

1 Vascular Medicine Branch, National Heart Lung and Blood Institute, Bethesda, MD, USA; Critical Care Medicine Department, Clinical Center, Bethesda, MD, USA, 2 Critical Care Medicine Department, Clinical Center, Bethesda, MD, USA, 3 Pulmonary - Critical Care Medicine Branch, National Heart Lung and Blood Institute, Bethesda, MD, USA, 4 Department of Medicine, School of Medicine, University of California, San Diego, La Jolla, CA, USA, 5 Vascular Medicine Branch, National Heart Lung and Blood Institute, Bethesda, MD, USA; Critical Care Medicine Department, Clinical Center, Bethesda, MD, USA; Department of Nursing and Patient Care Services, National Institutes of Health, Bethesda, MD, USA, 6 Department of Diagnostic Radiology, Clinical Center, Bethesda, MD, USA, 7 Vascular Medicine Branch, National Heart Lung and Blood Institute, Bethesda, MD, USA, 8 Department of Nuclear Medicine, Clinical Center, Bethesda, MD, USA

* To whom correspondence should be addressed. E-mail: robertom{at}nhlbi.nih.gov.

Rationale- While pulmonary hypertension is a common complication of sickle cell disease (SCD) associated with high mortality, there exists little data characterizing hemodynamics and cardiopulmonary function in this population. Objective- To characterize hemodynamics and cardiopulmonary function in patients with sickle cell disease with and without pulmonary hypertension (PH). Methods- SCD patients with PH (n=26) were compared with control SCD subjects, matched for age, hemoglobin and fetal hemoglobin levels, without PH (n=17) were evaluated. Measurements and Main Results- Upon catheterization 54% of the patients with PH had pulmonary arterial hypertension, and 46 % had pulmonary venous hypertension. When compared to controls, patients with PH exhibited lower six-minute walk distance (435 ± 31 versus 320 ± 20 meters; p=0.002) and oxygen consumption (50 ± 3% versus 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, supporting an independent contribution of increasing pulmonary artery pressures to loss of exercise capacity. Conclusions- Patients with sickle cell disease associated pulmonary hypertension have both pulmonary arterial and venous PH associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia. These data support the use of the six-minute walk distance as an index of PH and cardiopulmonary function in patients with sickle cell disease.
Key words: sickle cell disease, pulmonary hypertension, six-minute walk, hemodynamics, echocardiogram




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