Proposed algorithm for evaluation of PH related to SCD. Echocardiography should be performed while patients are clinically stable. Patients with an mPAP between 20 and 25 mmHg need further study because they may be at increased mortality risk. PAH therapy is to be considered on the basis of a weak recommendation and very low-quality evidence. 6MWD indicates 6-minute walk distance; ANA, antinuclear antibody; CXR, chest X-ray; EKG, electrocardiogram; LFTs, liver function tests; PAWP, pulmonary artery wedge pressure. (Reprinted with permission from Klings et al.¹⁵ )