Journal of pediatric hematology/oncology
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J. Pediatr. Hematol. Oncol. · Feb 2009
A prospective appraisal of pulmonary hypertension in children with sickle cell disease.
Pulmonary hypertension (PHT) is a life-threatening complication of sickle cell disease that occurs in 20% to 40% of adults. Measurement of maximal tricuspid regurgitant jet velocity (TRV) by echocardiography provides a noninvasive screening tool; TRV values > or =2.5 m/s are correlated with PHT and increased mortality. Our objective was to estimate the prevalence of PHT in our pediatric sickle cell population and its possible association with various clinical and laboratory findings, including obstructive sleep apnea and/or pulmonary dysfunction. ⋯ Elevation of TRV was relatively uncommon in our pediatric patients as compared with prevalence reported in adults and may be associated with increased hemolysis. There was no association with obstructive sleep apnea or abnormal pulmonary function.