A prospective appraisal of pulmonary hypertension in children with sickle cell disease.
OBJECTIVE
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.


METHODS
Eligible children had measurement of the TRV. Clinical data were collected, including detailed history with a standardized sleep apnea questionnaire; those with suggestive histories had polysomonography. Pulmonary function was assessed using whole body plethysmography.


RESULTS
Of 48 subjects (79% homozygous sickle cell anemia; median age 12 y; 11 receiving chronic transfusion) enrolled in the study, 4 (8.3%) had TRV >2.5 m/s; all had homozygous sickle cell anemia and 1 was receiving hydroxyurea after 3 years of transfusion for secondary stroke prevention. Subjects with elevated TRV had higher indirect bilirubin levels; we found no association between elevated TRV and obstructive apnea or pulmonary function abnormalities.


CONCLUSIONS
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.
Sedrak
Aziza
A
Department of Pediatrics, Divisions of Pediatric Hematology/Oncology, Pulmonology and Cardiology, SUNY Downstate Medical Center, Brooklyn, NY 11203, USA.
Rao
Sreedhar P
SP
Miller
Scott T
ST
Hekmat
Vahid
V
Rao
Madu
M
D000755:Anemia, Sickle Cell / Q000150:complications*; D001663:Bilirubin / Q000097:blood; D002648:Child; D003625:Data Collection; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D015995:Prevalence; D012129:Respiratory Function Tests; D020181:Sleep Apnea, Obstructive; D014262:Tricuspid Valve Insufficiency
D001663:Bilirubin