Sickle cell disease (SCD) is characterized by chronic hemolytic anemia and intermittent vaso-occlusive events associated with cardiac abnormalities.

To assess 3 dimensional (3D) echocardiographic of right ventricle (RV) volumes and function in a pediatric SCD population.

Eighteen patients with SCD aged 4 to 17 years old (mean age: 8.0±4 years, 56% male, body surface area (BSA) 1.0±0.35) and 18 healthy controls matched for age, gender and BSA were prospectively included and compared. Echocardiograms were performed using a commercially available ultrasound Philipps EPIQ 7C system using matrix X5-1 transducer. 3D indexed RV volumes and ejection fraction (3D-RVEF) were obtained using full volume acquisitions. RV free wall strain, tricuspid S-wave, tricuspid annular plane systolic excursion (TAPSE), indexed cardiac output, systolic pulmonary pressure (sPAP) and hemoglobin were assessed. Data were analyzed with TomtecArena© software (v2.3, Germany).

Cardiac output was significantly higher in SCD children (4.5 vs. 3.6 l/min/m², P=0.025), as sPAP (24.9 vs. 21.9mmHg, P=0.015), 3D-RV diastolic volume (58.1 vs. 47.5ml/m², P=0.025) and 3D-RV systolic volume (28.8 vs. 21.4mL/m², P=0.005). 3D-RVEF and RV free wall strain were significantly lower in SCD compared to control population (respectively 51.9 vs. 56.3%, P=0.018; −28.6 vs. −32, P=0.017). There were no difference regarding TAPSE and doppler S-wave. Mean hemoglobin in SCD population was 9.6±1.7g/dl (Fig. 1).

Despite normal RV systolic function parameters, 3D-RVEF and RV free wall strain are lower in children with SCD. Chronic anemia generating volume overload and vaso-occlusive events could explain these findings. This data must be confirmed by cardiac magnetic resonance imaging.