• Presse Med · Mar 2013

    [Pulmonary arterial hypertension and sickle cell disease].

    • Laurent Savale, Bernard Maitre, Dora Bachir, Frédéric Galactéros, Gerald Simonneau, and Florence Parent.
    • Université Paris-Sud, faculté de médecine, 94276 Kremlin-Bicêtre, France. laurent.savale@abc.aphp.fr
    • Presse Med. 2013 Mar 1;42(3):338-46.

    AbstractRecent 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.Copyright © 2012 Elsevier Masson SAS. All rights reserved.

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