Algorithm for evaluation of pulmonary hypertension related to sickle cell disease

TRV: tricuspid regurgitant jet velocity; SCD: sickle cell disease; PH: pulmonary hypertension; 6MWD: 6-minute walk distance; NT-pro-BNP: N-terminal pro-brain natriuretic peptide; mPAP: mean pulmonary artery pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; PAH: pulmonary artery hypertension; ANA: anti-nuclear antibody; LFTs: liver function tests; EKG: electrocardiogram.
* The use of the term screening refers to mortality risk assessment. Echocardiography should be performed while patients are clinically stable. Patients with an mPAP between 20 and 25 mmHg need further study as they may be at increased mortality risk. NOTE: PAH therapy is to be considered on the basis of a weak recommendation and very low-quality evidence.