High prevalence of pulmonary hypertension in children with sickle cell disease

Pulmonary hypertension (PHT) affects approximately 30% of adults with sickle cell disease. Adults with PHT have a significantly higher mortality rate. We report the results of a prospective study of the prevalence of PHT among children with sickle cell disease. In our cohort, 31% of children ! 10 years of age have evidence of PHT by Doppler echocardiography. Factors associated with the presence of PHT are male sex and elevated reticulocyte count. We recommend screening all children for PHT starting at the age of 10 years.