High blood pressure in the lungs a major risk for death in adults with sickle cell disease

gers high blood pressure in the lungs, doctors call it secondary pulmonary hypertension because it is secondary to another problem. This is the type of pulmonary hypertension evaluated in the NIH project.

"Secondary pulmonary hypertension develops in most types of hereditary and chronic anemias that are caused by hemolysis, the destruction of red blood cells. This suggests that there is a distinct syndrome of hemolysis-associated pulmonary hypertension, a complication that has been reported with increasing frequency in sickle cell patients," said Dr. Mark Gladwin, pulmonary specialist, NIH Clinical Center and lead investigator of this current research. He pointed out that retrospective studies show a prevalence of pulmonary hypertension ranging from 20-to-40 percent in patients with sickle cell disease.

The research project followed 195 patients, 82 men and 113 women, with an average age of 37, over two years. Doppler echocardiography, a test that uses sound waves to 'see' the heart, was performed on each person to assess their pulmonary-artery pressures. Doppler-defined pulmonary hypertension occurred in 32 percent of the patients. "The 'echo' is a test that is reasonably priced, non-invasive and should be recommended screening for adults with sickle cell disease just as the colonoscopy, cholesterol panel, mammogram, and other tests are. This would save lives and help to minimize a public health problem." said co-investigator Dr. Griffin Rodgers, chief, Clinical Hematology, National Institute of Diabetes and Digestive and Kidney Diseases.

"This is similar to what occurred in the mid-nineties. We saw that use of a transcranial Doppler exam changed the screening and management of pediatric patients with sickle cell disease in that it defined important clinical decision points that successfully led to preventing--or at least improving--the odds of reducing strokes in children and adolescents," added Dr. Rodgers.

Of the 195 individuals

Contact: Dianne Needham
NIH/National Institutes of Health

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