articleNew England Journal of MedicineFeb 25, 2004Closed access

Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease

National Institutes of Health Clinical Center · Center for Clinical Research (United States) · +3 more institutions

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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.

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