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

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

Correspondence and requests for reprints should be addressed to Roberto F. Machado, M.D., Vascular Medicine Branch, NHLBI Critical Care Medicine Department, Clinical Center, National Institutes of Health, Building 10-CRC, Room 5-5140, 10 Center Drive, MSC 1454, Bethesda MD, 20892–1454. E-mail: robertom{at}nhlbi.nih.gov

Rationale: 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.

Objectives: To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH.

Methods: 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.

Measurements and Main Results: 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, supporting an independent contribution of increasing pulmonary artery pressures to loss of exercise capacity.

Conclusions: Patients with SCD-associated PH 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 SCD.

Key Words: sickle cell disease • pulmonary hypertension • six-minute walk • hemodynamics • echocardiogram

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Pulmonary hypertension is an emerging complication of sickle cell disease with high mortality. There are few data characterizing hemodynamics and cardiopulmonary function in this population. What This Study Adds to the Field Patients with sickle cell disease–associated pulmonary hypertension have both pulmonary arterial and venous pulmonary hypertension associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia.

 

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