Evaluation of cardiac function in patients with sickle cell disease with left ventricular global longitudinal strain

Abstract

Background and ObjectivesThe importance of myocardial dysfunction in sickle cell disease (SCD) is currently debated. It is difficult to find a reliable index of function in patients with chronic overload as in SCD. Speckle tracking echocardiography, a new mean of evaluating cardiac function, might be a useful tool in SCD. It has been applied in many fields to detect early cardiac function deterioration, and it is less load dependent compared with other function parameters. Studies in patients with SCD are rare, and the results are conflicting. The present study aimed to determine whether left ventricular global longitudinal strain (LV-GLS) was abnormal in a population of adults with SCD and whether it was correlated with clinical or biological parameters.MethodsWe prospectively enrolled 37 patients and 34 age- and sex-matched healthy controls. Echocardiography was performed in patients and controls.ResultsWe found that the left ventricular diameter and mass were higher and the ejection fraction and longitudinal strain were lower in patients compared with controls. Diastolic dysfunction was uncommon. LV-GLS was abnormal in 21% of the patients. No correlation was observed between strain and clinical or biological parameters.ConclusionsWe concluded that LV-GLS could be a useful tool for evaluating these patients. However, the clinical impact of reduced LV-GLS remains to be determined.

Sickle cell disease and ventricular myocardial strain: A systematic review

“…was retrospective, and one (Mah et al.) was experimental 15,31–43,45,47 . Most studies obtained written consent and approval through an institutional review board or ethics committee.…”

“…Most studies obtained written consent and approval through an institutional review board or ethics committee. Eleven studies reported that patients were in steady state at the time of myocardial strain evaluation, defined as not being in any state of crisis or experiencing an acute event, generally for a minimum of 1 month 15,32,34–37,39–42,44,47 . The remaining six studies did not indicate timing of the patients’ last crisis in relation to myocardial strain evaluation (Table 3).…”

“…All remaining subjects were classified as having SCD ( n = 136) (Table 1). 15,31–47 Youssry et al. classified patients by subphenotype, not genotype, as follows: viscosity vaso‐occlusion cohort (lactate dehydrogenase <270 U/L) and hemolysis endothelial dysfunction cohort (lactate dehydrogenase ≥270 U/L) 46 .…”

“…Thirteen studies included controls as a comparison group for a combined total of 421 controls (Table 1). 15,31–34,36,37,39–41,44–47 Nine studies reported that controls were matched for age and gender, while four studies reported no significant difference in these variables. Eight studies evaluated children (age ≤17 years), and 12 evaluated adults.…”

“…A growing number of studies have evaluated myocardial strain in individuals with SCD, but collective results have not yet been summarized. This systematic review is warranted to synthesize reported findings and remaining gaps in knowledge 15,31–47 …”