Abstract 2303: Prospective Correlation of Echocardiography Findings with Hematologic and Pulmonary Function Parameters in Children with Sickle Cell Disease

Abstract

Background Adults with sickle cell disease (SCD) have an increased prevalence of pulmonary hypertension (PAH), associated with significant morbidity and mortality. This finding has not been validated in children. We carried out a prospective comparison of echocardiography (echo) data between SCD and control patients and a correlation between echo findings and hematologic and pulmonary function testing (PFT) in SCD patients. Methods Children with SCD and age and gender matched controls were prospectively enrolled during well visits. Each subject underwent a history and examination, echo, hematologic testing, and PFT. Echo data was compared between SCD patients and controls, and regression analysis was performed to assess for correlation between echo parameters and anemia, markers of hemolysis, and PFT in SCD patients. Results Of the 194 SCD patients and 29 controls enrolled, 180 SCD patients and 26 controls had measurable tricuspid valve regurgitation (TR) to estimate systolic pulmonary pressure. TR, left ventricle (LV) size and LV mass were significantly higher in children with SCD (Table 1 ). Linear regression analysis in SCD patients showed that TR, LV size, LV mass, and diastolic function (E/E TDI ) correlated significantly with hemoglobin, markers of hemolysis, and obstructive pulmonary disease (Table 2 ). There was no correlation between echo parameters and age, blood pressure, or measures of restrictive lung disease Conclusion Children with SCD have higher estimated pulmonary pressure, LV size and mass and a trend toward worse diastolic function when compared to controls. TR and LV size and mass correlate with anemia, hemolysis, and obstructive patterns on PFT. Table 1 - Comparative Data Table 2 - Regression Analysis