title: Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome.
abstract: 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 x min(-1) x m(-2) and pulmonary vascular resistance 521+/-205 dyn x s x 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.
authors: Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - Centre de référence des maladies pulmonaires rares, Université de Lyon, Université Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France.
authors: V
authors: V
authors: Cottin
authors: J
authors: J
authors: Le Pavec
authors: G
authors: G
authors: Prévot
authors: H
authors: H
authors: Mal
authors: M
authors: M
authors: Humbert
authors: G
authors: G
authors: Simonneau
authors: J-F
authors: JF
authors: Cordier
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000150:complications*; D011658:Pulmonary Fibrosis / Q000150:complications*; D012189:Retrospective Studies; D015996:Survival Rate; D013577:Syndrome