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Pulmonary hypertension of sickle cell disease

Key sources
The following summarized guidelines for the management of pulmonary hypertension of sickle cell disease are prepared by our editorial team based on guidelines from the American Thoracic Society (ATS 2014).
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Guidelines

1.Medical management

Hydroxyurea: initiate hydroxyurea therapy in patients with sickle cell disease who are at increased risk for mortality.
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More topics in this section

  • Phosphodiesterase inhibitors

  • Prostacyclin agonists and endothelin receptor antagonists

  • PAH-targeted therapy

  • Anticoagulant therapy

2.Therapeutic procedures

Chronic transfusion: consider initiating chronic transfusion therapy in patients with sickle cell disease who have an increased risk for mortality and who do not respond to hydroxyurea or not candidates for hydroxyurea.
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