What is the prognosis for adult patients with sickle cell disease who develop pulmonary hypertension?

Gladwin MT, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004 Feb 26;350(9):886-95.

Objective: To study prevalence of pulmonary hypertension and its affect on prognosis in patients with sickle cell disease.

Methods: Performed doppler echocardiography in 195 consecutive patients with sickle cell disease (mean age 36 years.)

Results: 32% had pulmonary hypertension defined as tricuspid regurgitant jet velocity at least 2.5 meters/second (compared to 5% of 41 controls). Pulmonary hypertension associated with 10 times risk of mortality over follow-up of median 18 months.

Powars DR, et al. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005 Nov;84(6):363-76.

Objective: To study factors associated with the development of irreversible organ damage and survival in patients with sickle cell disease.

Methods: Prospective cohort study of 1,056 patients, looking at age, sex and prior medical conditions.

Results: Figure 3 compares survival curves for patients born before 1975 and those born on or after 1975. Multiple tables summarize findings for type of organ failure.


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Clinical Informationist at EUH Branch Library
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