1. by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado
Pathophysiology and treatment of pulmonary hypertension in sickle cell disease
by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado
Blood
Volume 127(7):
February 18, 2016
©2016 by American Society of Hematology

2. Schematic representations of pulmonary hypertension.
Schematic representations of pulmonary hypertension. (A) Schematic cross-sectional representation of a normal pulmonary arteriole and a pulmonary arteriole affected by pulmonary hypertension. (Adapted from Pugliese et al.7) (B) Schematic representation of site of initiation of elevated pulmonary arterial pressure of precapillary pulmonary hypertension, postcapillary pulmonary hypertension, and CTEPH. L.V., left ventricle; PH, pulmonary hypertension; R.A., right atrium; R.V., right ventricle.
Victor R. Gordeuk et al. Blood 2016;127:
©2016 by American Society of Hematology

3. 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;...
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 regurgitation velocity. Note: Echocardiography should be performed while patients are clinically stable. PAH therapy is to be considered on the basis of a weak recommendation and very low-quality evidence. Reprinted with permission of the American Thoracic Society.84 The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.
Victor R. Gordeuk et al. Blood 2016;127:
©2016 by American Society of Hematology