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Previous Volume 350:886-895 February 26, 2004 Number 9 Next

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ABSTRACT

Background The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown.

Methods We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [±SD] age, 36±12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up.

Results Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P<0.001) and remained so after adjustment for other possible risk factors in a proportional-hazards regression model.

Conclusions Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Therapeutic trials targeting this population of patients are indicated.

Source Information

From the Critical Care Medicine Department, Clinical Center (M.T.G., M.L.J., K.M., W.A.C., J.S.N., I.E., L.A.H., W.C.B., F.P.O.), the Cardiovascular Branch, National Heart, Lung, and Blood Institute (M.T.G., V.S., Y.S., J.F.P., I.E.), and the Laboratory of Chemical Biology (M.T.G., A.N.S.) and the Molecular and Clinical Hematology Branch (G.P.R.), National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Md.; and the Cardiology Division, Department of Medicine (B.B.), and the Center for Sickle Cell Disease (O.C.), Howard University College of Medicine, Washington, D.C.

Address reprint requests to Dr. Gladwin at the National Institutes of Health, Bldg. 10, Rm. 7D-43, 10 Center Dr., Bethesda, MD 20892-1662, or at mgladwin{at}nih.gov.

Full Text of this Article

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Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease
Klings E. S., Farber H. W., Hassoun P. M., Krishnan J. A., Gladwin M. T., Sachdev V., Ognibene F. P.
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N Engl J Med 2004; 350:2521-2522, Jun 10, 2004. Correspondence

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