3-Dimensional echocardiographic evaluation of right ventricular function in pediatric sickle cell disease population

Abstract

Abstract Introduction Sickle cell disease (SCD) is characterized by chronic hemolytic anemia and intermittent vaso-occlusive events associated with cardiac abnormalities. Aim To assess 3 dimensional (3D) echocardiographic of right ventricle (RV) volumes and function in a pediatric SCD population. Methods 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 9 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). Results Cardiac output was significantly higher in SCD children (4.5 vs 3.6 l/min/m2, p=0.025), as sPAP (24.9 vs 21.9 mmHg, p=0.015), 3D-RV diastolic volume (58.1 vs 47.5 ml/m2, p=0.025) and 3D-RV systolic volume (28.8 vs 21.4 ml/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±1.7 g/dl. Conclusions 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. 3D sickle cell Funding Acknowledgement Type of funding source: None