Athénaïs Boucly1,2,3, Laurent Savale1,2,3, Xavier Jaïs1,2,3, Fabrice Bauer4, Emmanuel Bergot5, Laurent Bertoletti6, Antoine Beurnier1,2,3, Arnaud Bourdin7,8, Hélène Bouvaist9, Sophie Bulifon1,2,3, Céline Chabanne10, Ari Chaouat11, Vincent Cottin12, Claire Dauphin13, Bruno Degano14, Pascal De Groote15, Nicolas Favrolt16, Yuanchao Feng17, Delphine Horeau-Langlard18, Mitja Jevnikar1,2,3, Etienne-Marie Jutant1,2,3, Zhiying Liang17, Pascal Magro19, Pierre Mauran20, Pamela Moceri21, Jean-François Mornex12, Sylvain Palat22, Florence Parent1,2,3, François Picard23,24, Jérémie Pichon1,2,3, Patrice Poubeau25, Grégoire Prévot26, Sébastien Renard27, Martine Reynaud-Gaubert28, Marianne Riou29, Pascal Roblot30, Olivier Sanchez31, Andrei Seferian1,2,3, Cécile Tromeur32, Jason Weatherald17,33, Gérald Simonneau1,2,3, David Montani1,2,3, Marc Humbert1,2,3, Olivier Sitbon1,2,3. 1. Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France. 2. Service de Pneumologie et Soins Intensifs, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France. 3. Unité Mixte de Recherche S999, Hôpital Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale, Le Plessis-Robinson, France. 4. Unité 1096, Département de Chirurgie Cardiaque, Centre Hospitalier Universitaire de Rouen, Université de Rouen-Institut National de la Santé et de la Recherche Médicale, Rouen, France. 5. Unité de Formation et de Recherche en Santé, Service de Pneumologie et Oncologie Thoracique, Centre Hospitalier Universitaire de Caen, Université De Caen, Caen, France. 6. Unité Mixte de Recherche 1059, Service de Médecine Vasculaire et Thérapeutique, Centre Hospitalier Universitaire de Saint-Étienne, Université Jean-Monnet-Institut National de la Santé et de la Recherche Médicale, Saint-Étienne, France. 7. Service de Pneumologie, Centre Hospitalier Universitaire de Montpellier, Université de Montpellier, Montpellier, France. 8. Laboratoire de Physiologie et Médecine Expérimentale, Centre Hospitalier Universitaire de Montpellier, Université de Montpellier-Centre National de la Recherche Scientifique-Institut National de la Santé et de la Recherche Médicale, Montpellier, France. 9. Service de Cardiologie and. 10. Unité Mixte de Recherche 1099: Laboratoire Traitement du Signal et de l'Image, Service de Cardiologie et Maladies Vasculaires, Centre Hospitalier Universitaire de Rennes, Université de Rennes-Institut National de la Santé et de la Recherche Médicale, Rennes, France. 11. Unité Mixte de Recherche S1116, Département de Pneumologie, Faculté de Médecine de Nancy, Centre Hospitalier Régional Universitaire de Nancy, Université de Lorraine-Institut National de la Santé et de la Recherche Médicale, Vandœuvre-lès-Nancy, France. 12. Unité Mixte de Recherche 754: Infections Virales et Pathologie Comparée, Hospices Civils de Lyon, Université Lyon 1-Institut National de la Recherche Agronomique-Centre National de Référence des Maladies Pulmonaires Rares, Lyon, France. 13. Service de Cardiologie et Maladies Vasculaires, Hôpital Gabriel Montpied, Centre Hospitalier Universitaire de Clermont-Ferrand, Clermont-Ferrand, France. 14. Service de Pneumologie, Hôpital Universitaire Grenoble-Alpes, Grenoble, France. 15. Unité 1167, Service de Cardiologie, Centre Hospitalier Universitaire de Lille, Université de Lille-Institut Pasteur de Lille-Institut National de la Santé et de la Recherche Médicale, Lille, France. 16. Service de Pneumologie et Soins Intensifs Respiratoires, Centre Hospitalier Universitaire de François Mitterrand, Dijon, France. 17. Libin Cardiovascular Institute, University of Calgary, Calgary, Alberta, Canada. 18. Service de Pneumologie, Hôpital Laënnec, Centre Hospitalier Universitaire de Nantes, Nantes, France. 19. Service de Pneumologie, Centre Hospitalier Universitaire de Tours, Tours, France. 20. Unité de Cardiologie Pédiatrique et Congénitale, CHU de Reims, American Memorial Hospital, Reims, France. 21. Unité de Recherche Clinique Côte d'Azur, Service de Cardiologie, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, Nice, France. 22. Département de Médecine Interne, Centre Hospitalier Universitaire de Dupuytren, Limoges, France. 23. Unité de Traitement de l'Insuffisance Cardiaque and. 24. Centre de Compétences de l'Hypertension Pulmonaire, Hôpital Cardiologique Haut-Lévêque, Centre Hospitalier Universitaire de Bordeaux, Université de Bordeaux, Bordeaux, France. 25. Service de Médecine Interne, Centre Hospitalier Universitaire de la Réunion, Saint-Pierre de la Réunion, France. 26. Service de Pneumologie, Hôpital Larrey, Centre Hospitalier Universitaire de Toulouse, Toulouse, France. 27. Service de Cardiologie, Centre Régional de Compétences de l'Hypertension Pulmonaire, Hôpital La Timone, Marseille, France. 28. Service de Pneumologie, Équipe de Transplantation Pulmonaire, Centre de Compétences des Maladies Pulmonaires Rares, Aix-Marseille Université-Hôpital Nord, Assistance Publique-Hôpitaux de Marseille, Marseille, France. 29. Département de Pneumologie, Nouvel Hôpital Civil, Strasbourg, France. 30. Service de Médecine Interne, Maladies Infectieuses et Tropicales, Hôpital Universitaire de Poitiers, Poitiers, France. 31. Service de Pneumologie, Université de Paris-Hôpital Européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France. 32. Unité 1412: Centre d'Investigation Clinique de Brest, Département de Médecine Interne et Pneumologie, Centre Hospitalier Universitaire de Brest-Université de Bretagne Occidentale-Institut National de la Santé et de la Recherche Médicale, Brest, France; and. 33. Division of Respirology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
Abstract
Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Measurements and Main Results: Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.
Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Measurements and Main Results: Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.
Authors: Kevin Y Chang; Sue Duval; David B Badesch; Todd M Bull; Murali M Chakinala; Teresa De Marco; Robert P Frantz; Anna Hemnes; Stephen C Mathai; Erika Berman Rosenzweig; John J Ryan; Thenappan Thenappan Journal: J Am Heart Assoc Date: 2022-04-27 Impact factor: 6.106
Authors: Athénaïs Boucly; Jason Weatherald; Laurent Savale; Xavier Jaïs; David Montani; Marc Humbert; Olivier Sitbon Journal: Am J Respir Crit Care Med Date: 2021-12-15 Impact factor: 21.405
Authors: Marius M Hoeper; Christine Pausch; Ekkehard Grünig; Gerd Staehler; Doerte Huscher; David Pittrow; Karen M Olsson; Carmine Dario Vizza; Henning Gall; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Stephan Rosenkranz; Da-Hee Park; Ralf Ewert; Harald Kaemmerer; Tobias J Lange; Hans-Joachim Kabitz; Dirk Skowasch; Andris Skride; Martin Claussen; Juergen Behr; Katrin Milger; Michael Halank; Heinrike Wilkens; Hans-Jürgen Seyfarth; Matthias Held; Daniel Dumitrescu; Iraklis Tsangaris; Anton Vonk-Noordegraaf; Silvia Ulrich; Hans Klose Journal: Eur Respir J Date: 2022-06-02 Impact factor: 33.795