Figure 2.
Proposed algorithm for evaluation of pulmonary hypertension related to sickle cell disease. 6MWD = 6-minute walk distance; ANA = anti-nuclear antibody; CXR = chest X-ray; EKG = electrocardiogram; LFTs = liver function tests; mPAP = mean pulmonary artery pressure; NT-pro-BNP = N-terminal pro–brain natriuretic peptide; PAWP = pulmonary artery wedge pressure; PH = pulmonary hypertension; PVR = pulmonary vascular resistance; SCD = sickle cell disease; TRV = tricuspid regurgitant jet velocity. 1Note: 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 mm Hg 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.