Treatment
Î For patients with SCD who have an increased risk for mortality, the ATS
recommends hydroxyurea (strong recommendation, moderate-quality
evidence).
Î For patients with SCD who have an increased risk for mortality and who
either are not responsive to or not candidates for hydroxyurea, the ATS
suggests chronic transfusion therapy (weak recommendation, low-quality
evidence).
Î For patients with SCD who have RHC-confirmed PH, venous
thromboembolism, and no additional risk factors for hemorrhage, the ATS
suggests indefinite anticoagulant therapy rather than a limited duration
of therapy (weak recommendation, low-quality evidence).
Î For all patients with SCD who have elevated TRV alone or elevated NT-
pro- BNP alone, the ATS recommends against targeted PAH therapy
(strong recommendation, moderate-quality evidence).
Targeted PAH therapy currently includes prostacyclin agonist, endothelin receptor
antagonist, and phosphodiesterase-5 inhibitor therapy.
Î For most patients with SCD who have RHC-confirmed PH, the ATS
recommends against targeted PAH therapy (strong recommendation,
moderate-quality evidence).
Î For select patients with SCD who have RHC-confirmed marked elevation
of their pulmonary vascular resistance, normal pulmonary artery wedge
pressure, and related symptoms, the ATS suggests a trial of either
a prostacyclin agonist or an endothelin receptor antagonist (weak
recommendation, very low-quality evidence).
Î For patients with SCD who have RHC-confirmed marked elevation of their
pulmonary vascular resistance, normal pulmonary artery wedge pressure,
and related symptoms the ATS recommends against phosphodiesterase-5
inhibitor therapy as a first-line agent (strong recommendation, moderate-
quality evidence).
