title: A hemodynamic study of pulmonary hypertension in sickle cell disease.
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.).
authors: Université Paris-Sud, Kremlin-Bicêtre, Hôpital Antoine Béclère, Clamart, France. gerald.simonneau@abc.aphp.fr
authors: Florence
authors: F
authors: Parent
authors: Dora
authors: D
authors: Bachir
authors: Jocelyn
authors: J
authors: Inamo
authors: François
authors: F
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authors: Hajji
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authors: DS
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authors: Clerson
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authors: R
authors: Girot
authors: Frederic
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authors: Galacteros
authors: Gerald
authors: G
authors: Simonneau
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D006328:Cardiac Catheterization / Q000009:adverse effects; D015150:Echocardiography, Doppler; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D015995:Prevalence; D011446:Prospective Studies