What are the ASH guidelines on treatment of pulmonary hypertenstion (PH) in patients with sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Treatment of PAH is as follows:

  • For children and adults with SCD who do not have PAH confirmed by right-heart catheterization, the ASH guideline panel strongly recommends against the use of PAH-specific therapies. 
  • For children and adults with SCD and a diagnosis of PAH confirmed by right-heart catheterization, the ASH guideline panel recommends considering initiation and/or optimization of disease-modifying therapy such as hydroxyurea or chronic transfusions.
  • For children and adults with SCD and a diagnosis of PAH confirmed by right-heart catheterization, the ASH guideline panel suggests the use of PAH-specific therapies under the care of a PH specialist, given the lack of alternative treatment options, associated high morbidity and mortality, and the possibility of increased adverse effects (eg, pain) with PAH-specific therapy such as sildenafil.
  • Consider potential differences in the pathophysiologic basis of PAH (eg, contribution of chronic anemia and high-output cardiac states) and differences in adverse-effect profiles (eg, pain) when determining treatment options for PAH confirmed by right-heart catheterization in SCD.
  • The recommendation for PAH-specific therapy in SCD applies to patients with SCD who have no other clear reason for their PAH confirmed by right-heart catheterization (eg, obstructive sleep apnea, significant lung disease, left-heart failure).

End points for monitoring the benefits of PAH-specific therapy in these patients include the following:

  • Improvements in cardiopulmonary hemodynamics, as determined by right-heart catheterization
  • Improvements in clinical status, such as a change in PAH symptoms or functional status, initiation of other PAH drugs, or diuretic requirements (eg, in the setting of right-heart failure)

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