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. 2022 Dec 20;12(1):7.
doi: 10.3390/jcm12010007.

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

Letizia Sabatini  1   2 , Marcello Chinali  3 , Alessio Franceschini  3 , Margherita Di Mauro  1   2 , Silvio Marchesani  1   2 , Francesca Fini  1   2 , Giorgia Arcuri  2 , Mariachiara Lodi  4 , Giuseppe Palumbo  2   4 , Giulia Ceglie  2   4
Affiliations

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

Letizia Sabatini et al. J Clin Med. .

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.

Keywords: cardiovascular complications; echocardiography; paediatric population; sickle cell disease.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Correlation between LAD and markers of haemolytic anaemia. Box plot showing lower median Hb level (A), increased absolute reticulocyte count (B) in patients with left atrial dilatation compared to patients without this echocardiography alteration.
Figure 2
Figure 2
Correlation between LVH and markers of haemolytic anaemia. Box plot showing lower median Hb level (A), and increased absolute reticulocyte count (B) in patients with left ventricular Hypertrophy compared to patients without this echocardiography alteration.
See this image and copyright information in PMC

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