Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease.
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.
Mekontso Dessap
Armand
A
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
Leon
Rusel
R
Habibi
Anoosha
A
Nzouakou
Ruben
R
Roudot-Thoraval
Françoise
F
Adnot
Serge
S
Godeau
Bertrand
B
Galacteros
Frederic
F
Brun-Buisson
Christian
C
Brochard
Laurent
L
Maitre
Bernard
B
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*
D015415:Biomarkers; D020097:Natriuretic Peptide, Brain