Early detection of pulmonary vasculopathy in children with sickle cell disease by new echocardiography-based blood speckle technology

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BACKGROUND: Right ventricle (RV) dysfunction because of pulmonary vasculopathy is the most common cause of death in sickle cell disease (SCD). This study aimed to explore the diagnostic accuracy of blood-speckle tracking and vortex detection in the early detection of pulmonary vasculopathy in patients with SCD. METHODS: This study was conducted as a cross-sectional study including thirty patients and thirty controls. Patients with SCD were examined using 3D echocardiography to determine the presence of RV dysfunction, as a surrogate of pulmonary vasculopathy: in addition to blood speckle tracking echocardiography to determine the vortex timing in the RV and its presence or absence in the pulmonary artery. Patients' demographic and hematologic data were also retrieved from patients' files. RESULTS: Pulmonary vortex formation was 100% sensitive in the detection of RV dysfunction. LDH (Lactate Dehydrogenase) was the only variable significantly different between cases with pulmonary vortex formation and those without (520 vs. 257, P < 0.001), LDH > 400 was 72% sensitive and 100% specific in the detection of pulmonary vortex formation. CONCLUSIONS: Pulmonary vortex formation was a sensitive indicator of RV dysfunction, thus suggesting its accuracy in the early detection of early pulmonary vascular changes in SCD. LDH as a marker of intravascular hemolysis, is a sensitive marker that can be used for risk stratification of SCD patients.

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