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Pulmonary HTN Prevalence Studied in Sickle Cell Cases

Six percent of patients with sickle cell disease have pulmonary hypertension confirmed by catheterization

WEDNESDAY, July 6 (HealthDay News) -- The prevalence of pulmonary hypertension, as confirmed by right heart catheterization, in adult patients with sickle cell disease is about six percent, according to a study published in the July 7 issue of the New England Journal of Medicine.

Florence Parent, M.D., from the Université Paris-Sud, and colleagues assessed the prevalence and characteristics of pulmonary hypertension in 398 patients (average age, 34 years) with sickle cell disease. Doppler echocardiography with measurement of tricuspid valve regurgitant jet velocity was performed in all patients. On the basis of a velocity of at least 2.5 m per second, 96 patients with suspected hypertension underwent right heart catheterization.

The investigators found that 27 percent of the patients had a tricuspid valve regurgitant jet velocity of at least 2.5 m per second. Pulmonary hypertension was confirmed by catheterization for 6 percent of the patients. Echocardiography had a 25 percent positive predictive value for detecting hypertension. Of the 24 patients with confirmed pulmonary hypertension, 11 had a pulmonary-capillary wedge pressure of 15 mm Hg or less, indicative of precapillary pulmonary hypertension. Confirmed pulmonary hypertension was seen in older patients with poorer functional capacity and higher levels of N-terminal pro-brain natriuretic peptides. Similar clinical characteristics were seen in patients with a tricuspid valve regurgitant jet velocity of at least 2.5 m per second without hypertension and those with a tricuspid jet velocity of less than 2.5 m per second.

"In this study of adults with sickle cell disease, the prevalence of pulmonary hypertension as confirmed on right heart catheterization was 6 percent," the authors write. "Echocardiographic evaluation alone had a low positive predictive value for pulmonary hypertension."

One author disclosed financial relationships with Ikaria, GlaxoSmithKline, and Actelion.

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