Echocardiographic Screening of Cardiovascular Status in Pediatric Sickle Cell Disease

Area of Science:

• Pediatric Cardiology
• Hematology
• Cardiovascular Disease

Background:

• Echocardiography is vital for assessing cardiac function in sickle cell disease (SCD).
• Elevated right ventricular pressure and left ventricular diastolic dysfunction predict mortality in adult SCD patients.
• The role of routine echocardiographic screening in pediatric SCD is debated.

Purpose of the Study:

• To evaluate the utility of routine echocardiographic screening in children with SCD aged ≥10 years.
• To determine associations between echocardiographic findings and markers of disease severity.
• To assess the relationship between echocardiographic findings and biventricular systolic/diastolic function.

Main Methods:

• Retrospective review of 172 children (≥10 years) with SCD undergoing outpatient echocardiography.
• Analysis of tricuspid regurgitation jet velocity (TRV) for right ventricular (RV) pressure estimation.
• Assessment of left ventricle (LV) dilation and biventricular function indices.

Main Results:

• 61% had measurable TRV; 30% showed elevated RV pressure (TRV ≥ 2.5 m/s).
• LV dilation occurred in 25% of patients.
• Elevated RV pressure associated with chronic transfusion and high LDH; LV dilation associated with lower hemoglobin and higher reticulocyte/LDH/bilirubin.
• No association found between RV pressure/LV dilation and biventricular dysfunction.
• The single death in the cohort had normal echocardiographic findings.

Conclusions:

• Mild RV pressure elevation and LV dilation in pediatric SCD correlate with laboratory markers of disease severity.
• These echocardiographic findings were not associated with ventricular dysfunction in this cohort over 3 years.
• Routine echocardiography may help identify children with SCD at higher risk based on disease markers.