Disclaimer
is Guideline attempts to define principles of practice that should produce high-quality patient care.
It is applicable to specialists, primary care, and providers at all levels. is Guideline should not be
considered exclusive of other methods of care reasonably directed at obtaining the same results. e
ultimate judgment concerning the propriety of any course of conduct must be made by the clinician aer
consideration of each individual patient situation.
Neither IGC, the medical associations, nor the authors endorse any product or service associated with
the distributor of this clinical reference tool.
Abbreviations
6MWD, 6-minute walk distance; ANA, anti-nuclear antibody; CXR, chest X-ray; EKG,
electrocardiogram; HR, heart rate; LFTs, liver function tests; mPAP, mean pulmonary artery
pressure; NT-pro-BNP, N-terminal pro–brain natriuretic peptide; p, pressure; PAH, pulmonary
artery hypertension; PAWP, pulmonary artery wedge pressure; PH, pulmonary hypertension;
PVR, pulmonary vascular resistance; RHC, right heart catheterization; SCD, sickle cell disease;
TR maxPG, peak gradient of tricuspid regurgitation; TR Vmax, tricuspid regurgitation peak
velocity; TRV, tricuspid regurgitant jet velocity; v, velocity
Source
Klings ES, Machado RF, Barst RJ, et al; American oracic Society Ad Hoc Committee on
Pulmonary Hypertension of Sickle Cell Disease. An official American oracic Society clinical
practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of
sickle cell disease. Am J Respir Crit Care Med. 2014;189(6):727-40.
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