Guideline 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). 1 Guidelines 1. Medical management Hydroxyurea: initiate hydroxyurea therapy in patients with sickle cell disease who are at increased risk for mortality. B Phosphodiesterase inhibitors: avoid PDE5 inhibitor therapy as a first-line treatment for patients with symptomatic pulmonary hypertension of sickle cell disease. D Prostacyclin agonists and endothelin receptor antagonists: consider initiating either a prostacyclin agonist or an endothelin receptor antagonist for select patients with sickle cell disease who have marked elevation of pulmonary vascular resistance on right heart catheterization, normal pulmonary artery wedge pressure, and related symptoms. C PAH-targeted therapy: avoid targeted pulmonary arterial hypertension therapy in all patients with sickle cell disease who have elevated tricuspid regurgitant velocity alone, or elevated N- terminal pro-BNP alone, and for most patients with sickle cell disease who have right heart catheterization-confirmed pulmonary hypertension. D Anticoagulant therapy: consider administering indefinite anticoagulant therapy in patients with sickle cell disease who have right heart catheterization-confirmed pulmonary hypertension, VTE, and no additional risk factors for bleeding. C https://web.pathway.md/diseases/recnQQ5QSC58ctFw7 1/2 6/29/23, 11:35 PM Pulmonary hypertension of sickle cell disease Pathway 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. C Clinical findings Symptoms Ankle swelling Chest discomfort Chest pain Exercise intolerance Fainting Fatigue Shortness of breath References 1. Klings ES, Machado RF, Barst RJ et al. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 Mar 15;189 6 727 40. Open https://web.pathway.md/diseases/recnQQ5QSC58ctFw7 2/2