Document Detail

Pulmonary arterial hypertension and sickle cell disease.
MedLine Citation:
PMID:  22743045     Owner:  NLM     Status:  Publisher    
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.
Laurent Savale; Bernard Maitre; Dora Bachir; Frédéric Galactéros; Gerald Simonneau; Florence Parent
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-26
Journal Detail:
Title:  Presse medicale (Paris, France : 1983)     Volume:  -     ISSN:  0755-4982     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8302490     Medline TA:  Presse Med     Country:  -    
Other Details:
Languages:  FRE     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Masson SAS. All rights reserved.
Université Paris-Sud, faculté de médecine, 94276 Kremlin-Bicêtre, France; AP-HP, hôpital Bicêtre, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et réanimation respiratoire, 94275 Le Kremlin-Bicêtre, France; Inserm U999, hypertension artérielle pulmonaire : physiopathologie et innovation thérapeutique, 92350 Le Plessis-Robinson, France.
Vernacular Title:
Hypertension artérielle pulmonaire et drépanocytose.
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