Pulmonary Hypertension in Sickle Cell Disease

Pulmonary hypertension (PH) is common in sickle cell disease and is known to be a risk factor for death.To improve the care of patients with sickle cell disease, the American Thoracic Society has developed a clinical practice guideline on the treatment of PH in sickle cell disease for pulmonologists, hematologists, cardiologists, pediatricians, and internists.The guideline was created by assembling a panel of experts, reviewing the literature, synthesizing the evidence, and then generating recommendations.This summary for practicing clinicians is prepared from the official guideline document (1).