Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome

Vincent Cottin, J. Le Pavec, G. Prévot, H. Mal, M. Humbert, G. Simonneau, Jean François Cordier, A. Berezné, D. Coëtmeur, I. Danner-Boucher, D. Funke, D. Israel-Biet, E. Marchand, L. Mouthon

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Résumé

This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68±9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation. Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244±126 m. Forced vital capacity was 86±18%, forced expiratory volume in 1 s 78±19%, and carbon monoxide diffusion transfer coefficient 28±16% of predicted. Room air arterial oxygen tension was 7.5±1.6 kPa (56±12 mmHg). Mean pulmonary artery pressure was 40±9 mmHg, cardiac index 2.5±0.7 L·min -1·m -2 and pulmonary vascular resistance 521±205 dyn·s·cm -5. 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival. Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters. Copyright

langue originaleAnglais
Pages (de - à)105-111
Nombre de pages7
journalEuropean respiratory journal
Volume35
Numéro de publication1
Les DOIs
Etat de la publicationPublié - janv. 2010
Modification externeOui

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    Cottin, V., Le Pavec, J., Prévot, G., Mal, H., Humbert, M., Simonneau, G., Cordier, J. F., Berezné, A., Coëtmeur, D., Danner-Boucher, I., Funke, D., Israel-Biet, D., Marchand, E., & Mouthon, L. (2010). Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. European respiratory journal, 35(1), 105-111. https://doi.org/10.1183/09031936.00038709