Diastolic dysfunction in sickle cell

In adults with sickle cell disease (SCD), echocardiographic evidence of diastolic dysfunction is an independent risk factor for death that is additive to pulmonary hypertension. In this issue of Blood, Johnson and colleagues describe echocardiography and polysomnography results from 44 children with SCD. Because their echocardiograms were ordered for clinical indications, potentially biasing toward more symptomatic patients, the reported prevalence of increased left ventricular mass should be interpreted with caution. Nevertheless, the results demonstrate left ventricular hypertrophy and diastolic dysfunction significantly correlating with low TcO₂, both asleep and awake, and with systolic blood pressure. This confirms the association of left ventricular hypertrophy, diastolic dysfunction, and low waking TcO₂ in 310 children with SCD reported by Dham et al.