Gaëlle Dauriat1, Martine Reynaud-Gaubert2, Vincent Cottin3, Bouchra Lamia4, David Montani5, Mathieu Canuet6, Clement Boissin7, Cecile Tromeur8, Ari Chaouat9, Bruno Degano10, Emmanuel Bergot11, Olivier Sanchez12, Gregoire Prevot13, Olivier Sitbon5, Gabriel Thabut1, Drifa Belhadi14, Yolande Costa de Beauregard15, Amina Bencherif15, Marc Humbert5, Gerald Simonneau5, Cedric Laouenan14, Hervé Mal16. 1. Service de pneumologie B, hôpital Bichat, Paris, France, Université Paris 7, Inserm UMR1152. 2. Service de pneumologie, hôpital Nord, Marseille, France. 3. Service de pneumologie hôpital Louis Pradel, Lyon, France. 4. Service de pneumologie, Normandie Université, UNIROUEN, EA 3830. CHU de Rouen et Groupe Hospitalier du Havre, France. 5. Service de pneumologie, hôpital Bicêtre; Le Kremlin Bicêtre, France; Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. 6. Service de pneumologie, Nouvel Hôpital Civil, Strasbourg, France. 7. Service de pneumologie, hôpital Arnaud de Villeneuve, Montpellier, France. 8. Service de pneumologie, hôpital de la cavale blanche, Brest, France. 9. Service de pneumologie, hôpital Brabois, Nancy, France. 10. Service de pneumologie, hôpital Albert Michalon, Grenoble, France. 11. Service de pneumologie, hôpital côte de nacre, Caen France. 12. Service de pneumologie, hôpital européen Georges Pompidou, Paris, France. 13. Service de pneumologie, hôpital Larrey, Toulouse, France. 14. Unité de recherche clinique, hôpital Bichat, Paris, France. 15. Centre d'investigation clinique, hôpital Bichat, Paris, France. 16. Service de pneumologie B, hôpital Bichat, Paris, France, Université Paris 7, Inserm UMR1152. Electronic address: herve.mal@bch.aphp.fr.
Abstract
BACKGROUND: A small proportion of patients with chronic obstructive pulmonary disease (COPD) patients present severe pulmonary hypertension (PH), defined by mean pulmonary artery pressure (mPAP) ≥35 mm Hg measured by right heart catheterization. Little is known about the characteristics of severe PH-COPD. The aim of the study based on a national registry was to describe this phenotype. METHODS: We prospectively included and followed patients with incident PH-COPD. Clinical, functional, hemodynamic data at inclusion and follow-up were retrieved. Survival assessed by Kaplan-Meier analysis was the primary end-point. RESULTS: From 2012 to 2016, 99 patients from 13 French centers were included in the study (82 males; median age 66.0 years [interquartile range 62.0-72.0]). At inclusion, most patients had marked dyspnea (55.6% and 22.2% New York Heart Association class III and IV, respectively). During 12 months before inclusion, 42.9% had an exacerbation requiring a hospitalization. Pulmonary function tests showed a moderate obstructive pattern with median (interquartile range) FEV1 50.0 [35.0-63.0] % predicted and low diffusing capacity for carbon monoxide, median 20.0 [16.5-30.6] % predicted. The median values for PaO2 and PaCO2 on room air were 50.0 [44.8-62.0] and 36.0 [31.1-43.0] mm Hg. Median values of mPAP, pulmonary artery occlusion pressure, cardiac index and pulmonary vascular resistance were 42.0 [37.0-48.0] mm Hg, 11.0 [9.0-14.0] mm Hg, 3.0 [2.4-3.6] L/min/m2, and 6.3 [4.2-7.9] WU, respectively. Mean restricted survival was 15.0 [13.9-16.0] months. CONCLUSIONS: Severe PH-COPD is characterized by moderate airway obstruction but marked dyspnea and marked hypoxemia, low DLCO and high mPAP. This phenotype is associated with poor prognosis.
BACKGROUND: A small proportion of patients with chronic obstructive pulmonary disease (COPD) patients present severe pulmonary hypertension (PH), defined by mean pulmonary artery pressure (mPAP) ≥35 mm Hg measured by right heart catheterization. Little is known about the characteristics of severe PH-COPD. The aim of the study based on a national registry was to describe this phenotype. METHODS: We prospectively included and followed patients with incident PH-COPD. Clinical, functional, hemodynamic data at inclusion and follow-up were retrieved. Survival assessed by Kaplan-Meier analysis was the primary end-point. RESULTS: From 2012 to 2016, 99 patients from 13 French centers were included in the study (82 males; median age 66.0 years [interquartile range 62.0-72.0]). At inclusion, most patients had marked dyspnea (55.6% and 22.2% New York Heart Association class III and IV, respectively). During 12 months before inclusion, 42.9% had an exacerbation requiring a hospitalization. Pulmonary function tests showed a moderate obstructive pattern with median (interquartile range) FEV1 50.0 [35.0-63.0] % predicted and low diffusing capacity for carbon monoxide, median 20.0 [16.5-30.6] % predicted. The median values for PaO2 and PaCO2 on room air were 50.0 [44.8-62.0] and 36.0 [31.1-43.0] mm Hg. Median values of mPAP, pulmonary artery occlusion pressure, cardiac index and pulmonary vascular resistance were 42.0 [37.0-48.0] mm Hg, 11.0 [9.0-14.0] mm Hg, 3.0 [2.4-3.6] L/min/m2, and 6.3 [4.2-7.9] WU, respectively. Mean restricted survival was 15.0 [13.9-16.0] months. CONCLUSIONS: Severe PH-COPD is characterized by moderate airway obstruction but marked dyspnea and marked hypoxemia, low DLCO and high mPAP. This phenotype is associated with poor prognosis.