Echocardiographic Evaluation in Paediatric Sickle Cell Disease Patients: A Pilot Study

Area of Science:

• Pediatric Cardiology
• Hematology
• Sickle Cell Disease Research

Background:

• Cardiovascular complications significantly impact morbidity and mortality in sickle cell disease (SCD).
• Limited research exists on cardiac involvement in pediatric SCD patients.
• Echocardiogram screening guidelines for asymptomatic children with SCD are controversial.

Purpose of the Study:

• To investigate the prevalence of cardiac abnormalities in asymptomatic pediatric SCD patients.
• To identify associations between cardiac findings and clinical/laboratory parameters in this cohort.

Main Methods:

• Retrospective observational monocentric study.
• 64 pediatric SCD patients underwent routine transthoracic echocardiography.
• Analysis of cardiac abnormalities, laboratory values (hemoglobin, reticulocytes), transfusion needs, and pain crisis frequency.

Main Results:

• 46 (72%) patients exhibited at least one cardiac abnormality.
• Left atrial dilatation (LAD) was present in 65% and left ventricular hypertrophy (LVH) in 45%.
• LAD correlated with lower hemoglobin and higher reticulocyte counts; LVH correlated with lower hemoglobin and higher reticulocytes. Cardiac anomalies were linked to higher transfusion needs and fewer pain crises.

Conclusions:

• Cardiac involvement is highly prevalent in pediatric SCD patients.
• Specific laboratory findings (hemoglobin, reticulocytes) are associated with cardiac anomalies.
• Cardiac involvement in pediatric SCD may present a phenotype of high hemodynamic load complications.