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Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study

Olivier Sitbon, Xavier Jaїs, Laurent Savale, Vincent Cottin, Emmanuel Bergot, Elise Artaud Macari, Hélène Bouvaist, Claire Dauphin, François Picard, Sophie Bulifon, David Montani, Marc Humbert, Gérald Simonneau
European Respiratory Journal 2014; DOI: 10.1183/09031936.00116313
Olivier Sitbon
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Xavier Jaїs
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Laurent Savale
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Vincent Cottin
4Univ. Lyon-1, Hospices Civils de Lyon, Centre de Référence des maladies pulmonaires rares, Centre de Compétences de l'Hypertension Pulmonaire, Hôpital Louis Pradel, Lyon,
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Emmanuel Bergot
5Service de Pneumologie et d'oncologie thoracique, Hôpital Universitaire de Caen, Caen,
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Elise Artaud Macari
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Hélène Bouvaist
6Service de Cardiologie, CHU, Grenoble,
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Claire Dauphin
7Service de Cardiologie, CHU Gabriel-Montpied, Clermont-Ferrand,
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François Picard
8Service de Cardiologie, Hôpital Haut-Lévêque, Pessac, France
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Sophie Bulifon
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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David Montani
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Marc Humbert
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Gérald Simonneau
1Univ. Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre,
2APHP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hôpital de Bicêtre, Le Kremlin-Bicêtre,
3INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson,
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Abstract

Patients with severe pulmonary arterial hypertension (PAH) in New York Heart Association (NYHA) functional class (FC) III/IV have a poor prognosis, despite survival benefits being demonstrated with intravenous epoprostenol. In this pilot study, the efficacy and safety of a triple combination therapy regimen in patients with severe PAH was investigated.

Data from newly diagnosed NYHA FC III/IV PAH patients (n = 19) initiated on upfront triple combination therapy (intravenous epoprostenol, bosentan and sildenafil) were collected retrospectively from a prospective registry.

Significant improvements in 6-min walk distance and haemodynamics were observed after 4 months’ triple combination therapy in 18 patients (p<0.01); 17 patients had improved to NYHA FC I or II. One patient was not included in the month 4 assessment (due to an emergency lung transplant in month 3). At the final evaluation (mean±sd 32±19 months), all 18 patients had sustained clinical and haemodynamic improvement. Overall survival estimates for the triple combination cohort were 100% at 1, 2 and 3 years. Expected survival calculated from the French equation was 75% (95% CI 68–82%), 60% (95% CI 50–70%) and 49% (95% CI 38–60%) at 1, 2 and 3 years, respectively.

This pilot study provides preliminary evidence of the long-term benefits of upfront triple combination therapy in patients with severe PAH.

Abstract

Upfront triple combination therapy shows promising long-term efficacy in severe pulmonary arterial hypertension http://ow.ly/tKgHX

  • Received July 9, 2013.
  • Accepted February 11, 2014.
  • © ERS
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Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study
Olivier Sitbon, Xavier Jaїs, Laurent Savale, Vincent Cottin, Emmanuel Bergot, Elise Artaud Macari, Hélène Bouvaist, Claire Dauphin, François Picard, Sophie Bulifon, David Montani, Marc Humbert, Gérald Simonneau
European Respiratory Journal Jan 2014, erj01163-2013; DOI: 10.1183/09031936.00116313

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Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study
Olivier Sitbon, Xavier Jaїs, Laurent Savale, Vincent Cottin, Emmanuel Bergot, Elise Artaud Macari, Hélène Bouvaist, Claire Dauphin, François Picard, Sophie Bulifon, David Montani, Marc Humbert, Gérald Simonneau
European Respiratory Journal Jan 2014, erj01163-2013; DOI: 10.1183/09031936.00116313
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