A hemodynamic study of pulmonary hypertension in sickle cell disease.Reportar como inadecuado




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* Corresponding author 1 Service de pneumologie Béclère 2 Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique 3 Centre de référence des syndromes drépanocytaires majeurs 4 Service de cardiologie 5 Service d-hématologie, immunologie biologiques et cytogénétique 6 Centre intégré de drépanocytoses 7 Service de cardiologie Béclère 8 IMRB - Institut Mondor de Recherche Biomédicale 9 Service de cardiologie 10 Orgamétrie biostatistiques

Abstract : BACKGROUND: The prevalence and characteristics of pulmonary hypertension in adults with sickle cell disease have not been clearly established. METHODS: In this prospective study, we evaluated 398 outpatients with sickle cell disease mean age, 34 years at referral centers in France. All patients underwent Doppler echocardiography, with measurement of tricuspid-valve regurgitant jet velocity. Right heart catheterization was performed in 96 patients in whom pulmonary hypertension was suspected on the basis of a tricuspid regurgitant jet velocity of at least 2.5 m per second. Pulmonary hypertension was defined as a mean pulmonary arterial pressure of at least 25 mm Hg. RESULTS: The prevalence of a tricuspid regurgitant jet velocity of at least 2.5 m per second was 27%. In contrast, the prevalence of pulmonary hypertension as confirmed on catheterization was 6%. The positive predictive value of echocardiography for the detection of pulmonary hypertension was 25%. Among the 24 patients with confirmed pulmonary hypertension, the pulmonary-capillary wedge pressure was 15 mm Hg or less indicating precapillary pulmonary hypertension in 11 patients. Patients with confirmed pulmonary hypertension were older and had poorer functional capacity and higher levels of N-terminal pro-brain natriuretic peptide than other patients. In contrast, patients who had a tricuspid regurgitant jet velocity of at least 2.5 m per second without pulmonary hypertension and patients with a tricuspid regurgitant jet velocity of less than 2.5 m per second had similar clinical characteristics. CONCLUSIONS: In this study of adults with sickle cell disease, the prevalence of pulmonary hypertension as confirmed on right heart catheterization was 6%. Echocardiographic evaluation alone had a low positive predictive value for pulmonary hypertension. Funded by the French Ministry of Health and Assistance Publique-Hôpitaux de Paris; ClinicalTrials.gov number, NCT00434902







Autor: Florence Parent - Dora Bachir - Jocelyn Inamo - François Lionnet - Françoise Driss - Gylna Loko - Anoosha Habibi - Soumiya Benn

Fuente: https://hal.archives-ouvertes.fr/



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