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scientific articles

Mortality in adults with sickle cell disease and pulmonary hypertension

key information

source: JAMA

year: 2012

authors: Mehari A, Gladwin MT, Tian X, Machado RF, Kato GJ


Noninvasive echocardiographic markers of pulmonary artery pressure have been associated with early mortality in some studies in adults with sickle cell disease (SCD),1,2 but considerable controversy remains regarding the prevalence of pulmonary hypertension and its contribution to mortality.3 We assessed survival in a cohort of patients with SCD with pulmonary hypertension documented by right heart catheterization (RHC).

Patients provided written informed consent to protocols approved by the institutional review board at the National Institutes of Health. All adults with stable SCD seen consecutively in the outpatient clinic at the National Institutes of Health were screened with no exclusion criterion applied. Those who underwent RHC between March 13, 2002, and June 8, 2010, with elevated tricuspid regurgitant velocity (TRV) on echocardiography (≥2.8 m/s) and clinical suspicion of pulmonary hypertension (6-minute walk distance <500 m, unexplained dyspnea or desaturation, or both) were included. Pulmonary hypertension was defined as a mean pulmonary artery pressure of 25 mm Hg or greater. Life status was ascertained from clinical records, the Social Security Death Index, state death certificates, and contact with the patient or family as of June 8, 2010. All causes of death were considered for survival analysis. Survival rates (estimated by the Kaplan-Meier method) were compared between (1) those with pulmonary hypertension documented by RHC, (2) those without pulmonary hypertension documented by RHC, and (3) those who did not undergo RHC. Hazard ratios (HRs) were calculated based on Cox proportional hazards regression. Statistical tests were 2-sided and performed using the statistical language R version 2.13.1. P < .05 was considered statistically significant.

organization: College of Medicine, Howard University, Washington DC

DOI: 10.1001/jama.2012.358

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