An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease | oneSCDvoice
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guides & guidelines

An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease

key information

source: Agency for Healthcare Research & Quality

year: 2015

authors: Klings ES, Machado RF, Barst RJ, Morris CR, Mubarak KK, Gordeuk VR, Kato GJ, Ataga KI, Gibbs JS, Castro O, Rosenzweig EB, Sood N, Hsu L, Wilson KC, Telen MJ, DeCastro LM, Krishnamurti L, Steinberg MH, Badesch DB, Gladwin MT

summary/abstract:

Definitions for the levels of evidence (high, moderate, low, very low) and the strength of the recommendations (strong, weak) are provided at the end of the “Major Recommendations” field.

Estimating Mortality Risk in Sickle Cell Disease (SCD)

Conclusions from the Committee:

Risk stratification guides decision making.
Mortality risk can be determined by noninvasive measurement of the tricuspid regurgitant velocity (TRV) via Doppler echocardiography.
Serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) measurement is a reasonable noninvasive alternative if Doppler echocardiography is either unavailable or cannot obtain adequate images. (Note: Measurements may be misleading in patients with renal insufficiency.)
Mortality risk can also be determined invasively by direct hemodynamic measurements via right heart catheterization (RHC).

organisation: American Thoracic Society

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