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

Abstract

Cardiovascular involvement has a great impact on morbidity and mortality in sickle cell disease (SCD). Currently, few studies are available regarding the paediatric setting and, moreover, current guidelines for the echocardiogram screening program in the asymptomatic paediatric population are controversial. We performed a retrospective observational monocentric study on 64 SCD patients (37 male and 27 female, median age 10) at the Bambino Gesù Childrens’ Hospital, who had undergone a routine transthoracic echocardiogram. In total, 46 (72%) patients had at least one cardiac abnormality. Left atrial dilatation (LAD) was present in 41 (65%) patients and left ventricular hypertrophy (LVH) was found in 29 (45%) patients. Patients with LAD showed lower median haemoglobin levels (p = 0.009), and a higher absolute reticulocyte count (p = 0.04). LVH was negatively correlated with the median haemoglobin value (p = 0.006) and positively with the reticulocyte count (p = 0.03). Moreover, we found that patients with cardiac anomalies had higher transfusion needs and a lower frequency of pain crises. In our setting, cardiac involvement has a high prevalence in the paediatric cohort and seems to be associated with specific laboratory findings, and with a specific clinical phenotype characterized by complications related to high haemodynamic load.

Altered ventricular longitudinal strain in children with sickle cell disease: Role of TGF‐β and IL‐18

“…Surprisingly, the younger subgroup (<10.5 years) is significantly different in all these parameters when compared to healthy controls. This supports the concept of early subclinical cardiac involvement during childhood in SCD patients, which slowly progresses throughout life 3,20,21 …”

“…However, FWLS‐RV of patients was significantly lower when compared to control, reflecting significant deformed myocardial fibers of RV in absence of any evidence of PHT. Altered strain in SCD is multifactorial; however, chronic anemia and recurrent VOCs have been considered the main underlying factors that trigger cardiomyopathy 6,19–21 . Of note, 20% of children with SCD in the present study showed abnormally low GLS‐RV.…”

“…This supports the concept of early subclinical cardiac involvement during childhood in SCD patients, which slowly progresses throughout life. 3,20,21…”