Patient selection: child with sickle cell disease > 6 years of age
PAH was seen only in children who were SS (and not in those who had sickle-beta0 thalassemia).
Laboratory findings associated with PAH:
(1) high reticulocyte count
(2) low oxygen saturation
(3) high platelet count
The laboratory values overlapped those seen in children without PAH.
For the implementation the following cutoffs will be used:
(1) reticulocyte count > 11%
(2) oxygen saturation < 95%
(3) platelet count > 500,000 per µL
A child with risk factors present should undergo Doppler echocardiography with measurement of the tricuspid regurgitant jet velocity (TRJV). A TRJV >= 2.5 m/s indicates PAH. Children with a TRJV >= 3 m/s tended to have markedly abnormal laboratory values.
