When do you consider the use of PH specific therapies in patients with pre-capillary pulmonary hypertension associated with sickle cell disease?

Sickle cell-associated PH would be considered Group V PH. Evidence for treating sickle-associated pre-capillary PH with Pulmonary vasodilator therapies is poor. So, in a garden variety of sickle cell anemia associated with PH, I tend not to treat it with PV meds. If the PVR increases, I may sometime...

I agree with Dr. @Dr. First Last's comments above. This is a complex topic and patients with these problems should be evaluated at a center with expertise in sickle cell disease and PAH treatments. I would add two points:

First, screening for PH in patients with sickle cell disease should be with an...