Proposed algorithm for evaluation of PH related to SCD. 6MWD, 6-minute walk distance; ANA, anti-nuclear antibody; CXR, chest radiograph; 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; PVR, pulmonary vascular resistance. 1The 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. PH therapy is to be considered on the basis of a weak recommendation and very-low-quality evidence. Reprinted from Klings et al,59 with permission.