Increased pulmonary artery pressures among adolescents with sickle cell disease

The prevalence of pulmonary hypertension (PHT) among adolescents with sickle cell disease (SCD) is unknown. A tricuspid regurgitant (TR) jet peak velocity of 2.5 m/s or more is a screening test for PHT. The authors retrospectively reviewed echocardiograms and clinical data of adolescents followed at the Texas Children's Sickle Cell Center. Of 80 evaluable adolescents with SCD and echocardiogram data, 21 (26%) had a TR jet velocity of 2.5 m/s or more. Of these 21 patients with PHT, 12 (57%) had an echocardiogram performed during an inpatient stay for vaso-occlusive crisis (n = 6), acute chest syndrome (n = 4), fever (n = 1), or seizures (n = 1), and 9 (43%) had an echocardiogram performed as an outpatient in a baseline state of health. Elevation of pulmonary artery pressures was common in this adolescent cohort, but clinical symptoms were rare. Prospective study is warranted to determine the prevalence and course of elevated pulmonary artery pressures in this age group.