title: Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease.
abstract: BACKGROUND
  Steady-state mild pulmonary hypertension is a risk factor for death in adults with sickle cell disease. Acute pulmonary hypertension has been reported during exercise and vasoocclusive pain crisis in these patients.
  
  
  OBJECTIVE
  The aim of the present study was to evaluate changes in pulmonary pressures and cardiac biomarkers during severe acute chest syndrome and their associations with mortality.
  
  
  METHODS
  We prospectively evaluated 70 consecutive adults who received standardized treatment in our intensive care unit for a total of 84 episodes. At admission, cardiac biomarkers were measured. Transthoracic echocardiography was performed for pulmonary hypertension assessment via measurement of tricuspid regurgitant jet velocity and was repeated when possible after resolution.
  
  
  RESULTS
  Tricuspid jet velocity was less than 2.5 m/second in 34 (40%) of the 84 episodes, 2.5 to 2.9 m/second in 19 (23%), and 3 m/second or greater in 31 episodes (37%). Cor pulmonale occurred in 11 (13%) episodes. Tricuspid jet velocity showed significant positive correlations with B-type natriuretic peptide (rho = 0.54, P < 0.01) and cardiac troponin I (rho = 0.42, P < 0.01). Pulmonary pressures increased compared with steady state then decreased after resolution. All five patients who required invasive ventilation and all four patients who died during the immediate hospital course had jet velocity values of 3 m/second or greater. Overall mortality was 12.9% (9 patients) and survival was significantly lower in patients whose jet velocity was 3 m/second or greater during at least one episode, compared with the other patients (P < 0.01).
  
  
  CONCLUSIONS
  Pulmonary pressures increase during severe acute chest syndrome, and pulmonary hypertension is associated with cardiac biomarker elevation and a higher risk of death.
authors.affiliation: Service de Réanimation Médicale, Centre Hospitalo-Universitaire Henri Mondor, 51 avenue du M de Lattre de Tassigny, 94010 Créteil Cedex, France. armand.dessap@hmn.aphp.fr
authors.forename: Armand
authors.initials: A
authors.lastname: Mekontso Dessap
authors.forename: Rusel
authors.initials: R
authors.lastname: Leon
authors.forename: Anoosha
authors.initials: A
authors.lastname: Habibi
authors.forename: Ruben
authors.initials: R
authors.lastname: Nzouakou
authors.forename: Françoise
authors.initials: F
authors.lastname: Roudot-Thoraval
authors.forename: Serge
authors.initials: S
authors.lastname: Adnot
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Frederic
authors.initials: F
authors.lastname: Galacteros
authors.forename: Christian
authors.initials: C
authors.lastname: Brun-Buisson
authors.forename: Laurent
authors.initials: L
authors.lastname: Brochard
authors.forename: Bernard
authors.initials: B
authors.lastname: Maitre
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000401:mortality / Q000503:physiopathology; D015415:Biomarkers; D001794:Blood Pressure; D004452:Echocardiography; D005260:Female; D005602:France; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality / Q000503:physiopathology*; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011446:Prospective Studies; D011651:Pulmonary Artery; D011660:Pulmonary Heart Disease / Q000209:etiology / Q000401:mortality / Q000503:physiopathology*; D012128:Respiratory Distress Syndrome / Q000209:etiology / Q000503:physiopathology*
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain