title: An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.
abstract: 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 clinician-investigators 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 evidence-based recommendations. Target audiences include all clinicians who take care of patients with SCD.
  
  
  RESULTS
  Mortality risk stratification guides decision making. An increased risk for mortality is defined as a TRV equal to or greater than 2.5 m/second, an NT-pro-BNP level 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 a weak 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 capillary wedge 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.
authors.forename: Elizabeth S
authors.initials: ES
authors.lastname: Klings
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.forename: Claudia R
authors.initials: CR
authors.lastname: Morris
authors.forename: Kamal K
authors.initials: KK
authors.lastname: Mubarak
authors.forename: Victor R
authors.initials: VR
authors.lastname: Gordeuk
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.forename: Kenneth I
authors.initials: KI
authors.lastname: Ataga
authors.forename: J Simon
authors.initials: JS
authors.lastname: Gibbs
authors.forename: Oswaldo
authors.initials: O
authors.lastname: Castro
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
authors.forename: Namita
authors.initials: N
authors.lastname: Sood
authors.forename: Lewis
authors.initials: L
authors.lastname: Hsu
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Wilson
authors.forename: Marilyn J
authors.initials: MJ
authors.lastname: Telen
authors.forename: Laura M
authors.initials: LM
authors.lastname: Decastro
authors.forename: Lakshmanan
authors.initials: L
authors.lastname: Krishnamurti
authors.forename: Martin H
authors.initials: MH
authors.lastname: Steinberg
authors.forename: David B
authors.initials: DB
authors.lastname: Badesch
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000986:Antisickling Agents / Q000627:therapeutic use; D006328:Cardiac Catheterization; D003661:Decision Support Techniques; D015150:Echocardiography, Doppler; D017707:Erythrocyte Transfusion; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D018570:Risk Assessment; D012720:Severity of Illness Index
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D000986:Antisickling Agents; D058986:Phosphodiesterase 5 Inhibitors; D006918:Hydroxyurea