Diastolic dysfunction in sickle cell

Abstract

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.

Representative echocardiographic images from a patient with sickle cell disease. (A) Apical 4-chamber view showing concentric left ventricular hypertrophy and a dilated left atrium. (B) Doppler of the mitral inflow used to obtain a peak E velocity for early ventricular filling. (C) Tissue Doppler of the lateral mitral annulus used to obtain a peak e′ velocity. The E/e′ ratio is an indicator of left ventricular filling pressures and is used in the assessment of diastolic dysfunction.