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Updated: May 21, 2026

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
[Pulmonary arterial hypertension and sickle cell disease]
Laurent Savale1, Bernard Maitre, Dora Bachir
1Université Paris-Sud, faculté de médecine, 94276 Kremlin-Bicêtre, France. laurent.savale@abc.aphp.fr
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.
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