Quantitative assessment of ventricular function in sickle cell disease: effect of long-term erythrocytapheresis

Area of Science:

• Cardiology
• Hematology
• Pediatrics

Background:

• Previous studies indicate cardiac dysfunction in sickle cell disease (SCD) patients on chronic transfusion protocols, with elevated left ventricular myocardial performance index (LVMPI).
• LVMPI is a validated, non-invasive measure of overall cardiac function.
• No prior studies have assessed cardiac function in SCD patients undergoing long-term erythrocytapheresis (LTE).

Purpose of the Study:

• To evaluate cardiac function in pediatric and young adult patients with SCD.
• To compare cardiac function, measured by LVMPI, between non-transfused SCD patients (NT-SCD) and those on LTE (T-SCD).

Main Methods:

• Recorded LVMPI in 22 patients with SCD aged 3-20 years.
• Compared LVMPI between NT-SCD and T-SCD groups.
• Analyzed differences in hemoglobin levels and TR velocity among T-SCD, severe NT-SCD, and mild NT-SCD patients.

Main Results:

• Males with SCD exhibited higher mean LVMPI than females.
• Significant differences in hemoglobin and TR velocity were observed among T-SCD, severe NT-SCD, and mild NT-SCD groups.
• T-SCD patients demonstrated significantly elevated LVMPI compared to both severe and mild NT-SCD patients.

Conclusions:

• SCD patients on LTE exhibit cardiac dysfunction, evidenced by elevated LVMPI.
• Elevated LVMPI in T-SCD patients may reflect the overall disease severity.
• Further research with serial LVMPI monitoring in a larger SCD cohort is warranted.