Abstract

Background. Echocardiography-determined tricuspid regurgitant jet velocity >2.5 m/sec predicts elevated systolic pulmonary artery pressure and early mortality in adults with sickle cell disease (SCD). The definition, prevalence and clinical correlates of elevated jet velocity have not been established in pediatric patients.

Methods. A prospective, case controlled, multi-center study of 310 children and adolescents with SCD under basal conditions was conducted to test the hypotheses that elevated jet velocity affects 10–20% of pediatric patients, is associated with both hemolysis and hypoxia, and has clinical correlates of acute chest syndrome, stroke, high transfusion requirement and abnormal six-minute-walk test.

Results. Elevated jet velocity (defined as ≥2.60 m/sec based on the mean ±2 SD in 54 controls) occurred in 32 patients (11%; 95% confidence interval 8–15%). A hemolytic index (generated by principal component analysis of lactate-dehydrogenase, aspartate-aminotransferase, bilirubin and reticulocytes), and oxygen saturation ≤98% had independent associations with elevated jet velocity (P=0.009 and P=0.028, respectively) that persisted after adjustment for hemoglobin, systolic blood pressure and left ventricular diastolic function. Significant clinical correlates of elevated jet velocity included histories of acute chest syndrome and multiple blood transfusions and degree of oxygen desaturation during the six-minute-walk, but not history of stroke or six-minute-walk distance.

Conclusion. This prospective study indicates an 11% prevalence of elevated jet velocity according to a pediatric-specific definition and provides evidence for independent associations with hemolysis and oxygen desaturation. Given these associations and the correlation with acute chest syndrome, elevated jet velocity may predict severe SCD complications, and future investigations should address whether early intervention is beneficial.

 Tricuspid regurgitant jet velocity<2.6 m/sec Tricuspid regurgitant jet velocity≥2.6 m/sec P value 
*number (%) 
** Derived from reticulocyte count, lactate dehydrogenase, total bilirubin, asparate aminotransferase 
1Adjusted for age and study site 
Marker of systemic blood pressure      
Systolic blood pressure (mmHg) 258 112 (103–120) 32 118 (109–128) 0.0301 
Marker of anemia      
Hemoglobin (g/dL) 251 9.3 (8.2–10.6) 30 9.1 (7.6–10.0) 0.0411 
Markers of hemolysis      
Reticulocytes (%) 244 6.8 (3.9–11.5) 30 9.6 (4.4–14.60 0.0061 
Reticulocyte count (K/uL) 245 210 (148–309) 30 248 (120–352) 0.0501 
Lactate dehydrogenase(U/L) 247 356 (270–496) 29 457 (342–570) 0.0091 
Total bilirubin (mg/dL) 253 2.1 (1.3–3.3) 30 2.8 (2.3–5.3) 0.0051 
Asparate aminotransferase (U/L) 253 38 (29–52) 30 47 (35–64) 0.0011 
Hemolytic index (relative unit)** 237 −0.08 (−1.22–0.91) 28 0.91 (−0.10–2.50) 0.00021 
Marker of hypoxia      
Oxygen saturation (%) 250 99 (97–99) 30 97 (96–98) 0.0051 
 Tricuspid regurgitant jet velocity<2.6 m/sec Tricuspid regurgitant jet velocity≥2.6 m/sec P value 
*number (%) 
** Derived from reticulocyte count, lactate dehydrogenase, total bilirubin, asparate aminotransferase 
1Adjusted for age and study site 
Marker of systemic blood pressure      
Systolic blood pressure (mmHg) 258 112 (103–120) 32 118 (109–128) 0.0301 
Marker of anemia      
Hemoglobin (g/dL) 251 9.3 (8.2–10.6) 30 9.1 (7.6–10.0) 0.0411 
Markers of hemolysis      
Reticulocytes (%) 244 6.8 (3.9–11.5) 30 9.6 (4.4–14.60 0.0061 
Reticulocyte count (K/uL) 245 210 (148–309) 30 248 (120–352) 0.0501 
Lactate dehydrogenase(U/L) 247 356 (270–496) 29 457 (342–570) 0.0091 
Total bilirubin (mg/dL) 253 2.1 (1.3–3.3) 30 2.8 (2.3–5.3) 0.0051 
Asparate aminotransferase (U/L) 253 38 (29–52) 30 47 (35–64) 0.0011 
Hemolytic index (relative unit)** 237 −0.08 (−1.22–0.91) 28 0.91 (−0.10–2.50) 0.00021 
Marker of hypoxia      
Oxygen saturation (%) 250 99 (97–99) 30 97 (96–98) 0.0051 

Distribution of markers prospectively hypothesized to be associated with pulmonary hypertension according to tricuspid regurgitant jet velocity categories. Results are in median and interquartile range unless otherwise indicated.

Disclosures: No relevant conflicts of interest to declare.