Key result
Children with sickle cell disease had higher right ventricular global longitudinal strain compared to age- and sex-matched controls (-25.72% vs. -24.54%, P=0.005).
Why the study?
Cardiac disease is the primary cause of death in sickle cell disease, but right and left ventricular global longitudinal strain as early markers of systolic dysfunction are not well investigated in children with the disease.
Does sickle cell disease alter ventricular global longitudinal strain in children compared to healthy controls?
Case-Control (n=214)
Does sickle cell disease alter ventricular global longitudinal strain in children compared to healthy controls?
Elevated right ventricular global longitudinal strain is present in children with sickle cell disease and may serve as an early marker of altered myocardial function and compensatory response.
Answers are grounded in this paper and cite their sources.
RV strain elevation may mark early myocardial changes in pediatric sickle cell disease; leaves open prognostic value and need for prospective validation.
Summary Cardiac disease is the primary cause of death in sickle cell disease (SCD). Right and left ventricular global longitudinal strain (RVGLS, LVGLS) are early markers of systolic dysfunction but are not well investigated among children with SCD. One hundred and forty‐three patients with HbSS or HbSβ0‐thalassaemia (median age 11 years, range 5–19 years) and 71 controls matched for age and sex were compared. RVGLS and LVGLS were measured and compared with conventional measures of echocardiography and markers of haemolysis and inflammation. RVGLS was higher in children with SCD than in controls (−25·72% ± 3·45% vs. −24·54% ± 2·41%, P = 0·005); LVGLS was not different. RVGLS decreased with older age in children with SCD (ρ = 0·338, P < 0·001) but not among controls. Decreased RVGLS was associated with increased left atrial end diastolic volume (ρ = 0·181, P = 0·04); RVGLS increased with cardiac output (r = −0·279, P = 0·01). RVGLS and LVGLS were not associated with disease‐modifying therapies, degree of anaemia or haemolysis markers. Elevated RVGLS may indicate an early RV compensatory mechanism in response to upstream myocardial insults and elevated cardiac output. Global longitudinal strain may serve as an early marker of altered myocardial function in children with SCD.
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Whipple et al. (2018) conducted a case-control in Sickle cell disease (n=214). Sickle cell disease vs. Age- and sex-matched controls was evaluated on Right ventricular global longitudinal strain (RVGLS) (p=0.005). Children with sickle cell disease had higher right ventricular global longitudinal strain compared to age- and sex-matched controls (-25.72% vs. -24.54%, P=0.005).
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