This Article Full Text Full Text (PDF) Online Supplement All Versions of this Article: 200710-1606OCv1 200710-1606OCv2 177/6/646    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by Mekontso Dessap, A. Articles by Maitre, B. PubMed PubMed Citation Articles by Mekontso Dessap, A. Articles by Maitre, B.

Pulmonary Hypertension and Cor Pulmonale during Severe Acute Chest Syndrome in Sickle Cell Disease

¹ Medical Intensive Care Unit, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Henri Mondor–Albert Chenevier, Créteil, France; ² INSERM Unité 841, Institut Mondor de Recherches Biomédicales, Equipe 8, Faculté de Médecine, Université Paris XII, Créteil France; and ³ Sickle Cell Disease Center, ⁴ Public Health Unit, ⁵ Internal Medicine Unit, and ⁶ Pulmonary Disease Unit, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Henri Mondor–Albert Chenevier, Créteil, France

Correspondence and requests for reprints should be addressed to Armand Mekontso Dessap, M.D., Service de Réanimation Médicale, Centre Hospitalo–Universitaire Henri Mondor, 51 avenue du M^al de Lattre de Tassigny, 94010 Créteil Cedex, France. E-mail: armand.dessap{at}hmn.aphp.fr

Rationale: 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.

Objectives: 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.

Measurements and Main 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.

Key Words: hemoglobinopathies • lung injury • pulmonary pressure • cardiac biomarkers • echocardiography

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Moderate pulmonary hypertension has been reported in adult patients with steady-state sickle cell disease and is associated with a worse prognosis. Acute chest syndrome is a common complication of sickle cell disease that contributes substantially to mortality. What This Study Adds to the Field We report the first evidence that pulmonary pressure elevation is common during severe acute chest syndrome, and associated with a higher risk of death. These findings may suggest new therapeutic approaches to acute chest syndrome.