title: [Pulmonary arterial hypertension and sickle cell disease].
abstract: Recent hemodynamic studies performed in large cohorts of adult patients with sickle cell disease have established the prevalence of pulmonary hypertension in this disease about 6 to 10%. Over half of these correspond to postcapillary pulmonary hypertension. Precapilliary arterial pulmonary hypertension seems to be a relatively infrequent complication of the disease. It is characterized by a different hemodynamic profile of idiopathic PAH with lower levels of pulmonary pressures and pulmonary vascular resistance. However, pulmonary vascular disease appears to have a significant impact on the functional status and vital prognosis of patients with sickle cell disease. The predictive value of echocardiography to detect pulmonary hypertension in this population is low (25-32%) when the threshold of tricuspid regurgitation velocity of 2.5m/s is used. At present, no specific treatments for pulmonary arterial hypertension is currently approved for the treatment of PAH associated with sickle cell disease due to lack of data in this specific population.
authors: Université Paris-Sud, faculté de médecine, 94276 Kremlin-Bicêtre, France. laurent.savale@abc.aphp.fr
authors: Laurent
authors: L
authors: Savale
authors: Bernard
authors: B
authors: Maitre
authors: Dora
authors: D
authors: Bachir
authors: Frédéric
authors: F
authors: Galactéros
authors: Gerald
authors: G
authors: Simonneau
authors: Florence
authors: F
authors: Parent
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy