Ultrasound screening helps adults with sickle cell disease
Doppler echocardiography screening in adults with sickle cell disease may help detect pulmonary hypertension before it becomes fatal, according to a study published in the Feb. 26 issue New England Journal of Medicine.
Ninety percent of sickle cell disease patients assessed in primary-care centers go unchecked for pulmonary disease. Doppler echocardiographic screening in this population could identify a high-risk group that may benefit from intervention, said lead author Dr. Mark T. Gladwin, a senior investigator at the National Institutes of Health's Critical Care Medicine Department.
Gladwin and colleagues from the NIH and Howard University College of Medicine assessed 195 consecutive patients with sickle cell disease. Patients underwent systolic pressure evaluation of their pulmonary arteries with Doppler echocardiography.
Pulmonary hypertension occurred in 32% of patients. Hypertense patients -- those defined as having a tricuspid regurgitant jet velocity of 2.5 m/sec or more -- bore a higher death risk than patients with normal blood pressure (p<0.001). Patients remained high risk even after researchers accounted for confounding factors.
The prevalence of pulmonary hypertension in this population and the significance of its prospective prognosis were previously unknown, Gladwin said. Retrospective studies had shown that sickle cell patients had lower pulmonary pressure and higher cardiac output than patients with primary pulmonary hypertension. Yet the two-year mortality rates for both groups reached 50%. This anomaly led Gladwin and colleagues to undertake the present prospective study.
Therapeutic trials of oxygen, warfarin, transfusion, and pulmonary vasodilator and remodeling medications should be performed to evaluate their potential in lowering the morbidity and mortality associated with pulmonary hypertension in these patients, according to the study.
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