TY - JOUR
T1 - An official american thoracic society clinical practice guideline
T2 - Diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease
AU - Klings, Elizabeth S.
AU - Machado, Roberto F.
AU - Barst, Robyn J.
AU - Morris, Claudia R.
AU - Mubarak, Kamal K.
AU - Gordeuk, Victor R.
AU - Kato, Gregory J.
AU - Ataga, Kenneth I.
AU - Gibbs, J. Simon
AU - Castro, Oswaldo
AU - Rosenzweig, Erika B.
AU - Sood, Namita
AU - Hsu, Lewis
AU - Wilson, Kevin C.
AU - Telen, Marilyn J.
AU - DeCastro, Laura M.
AU - Krishnamurti, Lakshmanan
AU - Steinberg, Martin H.
AU - Badesch, David B.
AU - Gladwin, Mark T.
PY - 2014/3/15
Y1 - 2014/3/15
N2 - Background: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) diagnosed by right heart catheterization (RHC) are independent risk factors for mortality. Methods: A multidisciplinary committee was formed by clinicianinvestigators experienced in the management of patients with PH and/or SCD. Clinically important questions were posed, related evidence was appraised, and questions were answered with evidencebased recommendations. Target audiences include all clinicians who take care of patients with SCD. Results: Mortality risk stratification guides decision making. An increased risk formortality is defined as a TRVequal to or greater than 2.5m/second, anNT-pro-BNPlevel equal to or greater than 160 pg/ml, or RHC-confirmed PH. For patients identified as having increased mortality risk, we make a strong recommendation for hydroxyurea as first-line therapy and aweak recommendation for chronic transfusions as an alternative therapy. For all patients with SCD with elevated TRV alone or elevated NT-pro-BNP alone, and for patients with SCD with RHC-confirmed PH with elevated pulmonary artery wedge pressure and low pulmonary vascular resistance, we make a strong recommendation against PAH-specific therapy. However, for select patients with SCD with RHC-confirmed PH who have elevated pulmonary vascular resistance and normal pulmonary capillarywedge pressure, we make a weak recommendation for either prostacyclin agonist or endothelin receptor antagonist therapy and a strong recommendation against phosphodiesterase-5 inhibitor therapy. Conclusions: Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.
AB - Background: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) diagnosed by right heart catheterization (RHC) are independent risk factors for mortality. Methods: A multidisciplinary committee was formed by clinicianinvestigators experienced in the management of patients with PH and/or SCD. Clinically important questions were posed, related evidence was appraised, and questions were answered with evidencebased recommendations. Target audiences include all clinicians who take care of patients with SCD. Results: Mortality risk stratification guides decision making. An increased risk formortality is defined as a TRVequal to or greater than 2.5m/second, anNT-pro-BNPlevel equal to or greater than 160 pg/ml, or RHC-confirmed PH. For patients identified as having increased mortality risk, we make a strong recommendation for hydroxyurea as first-line therapy and aweak recommendation for chronic transfusions as an alternative therapy. For all patients with SCD with elevated TRV alone or elevated NT-pro-BNP alone, and for patients with SCD with RHC-confirmed PH with elevated pulmonary artery wedge pressure and low pulmonary vascular resistance, we make a strong recommendation against PAH-specific therapy. However, for select patients with SCD with RHC-confirmed PH who have elevated pulmonary vascular resistance and normal pulmonary capillarywedge pressure, we make a weak recommendation for either prostacyclin agonist or endothelin receptor antagonist therapy and a strong recommendation against phosphodiesterase-5 inhibitor therapy. Conclusions: Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.
KW - Dyspnea
KW - Hemolysis
KW - Mortality
KW - Pulmonary hypertension
KW - Sickle cell disease
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U2 - 10.1164/rccm.201401-0065ST
DO - 10.1164/rccm.201401-0065ST
M3 - Article
C2 - 24628312
AN - SCOPUS:84896501856
VL - 189
SP - 727
EP - 740
JO - American Review of Respiratory Disease
JF - American Review of Respiratory Disease
SN - 1073-449X
IS - 6
ER -