Laurent Godinas1, Caroline Sattler2, Edmund M Lau3, Xavier Jaïs2, Yu Taniguchi2, Mitja Jevnikar2, Jason Weatherald4, Olivier Sitbon2, Laurent Savale2, David Montani2, Gérald Simonneau2, Marc Humbert2, Pierantonio Laveneziana5, Gilles Garcia2. 1. Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l׳Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France; CHU UCL Namur, Université Catholique de Louvain, Département de Pneumologie, Yvoir, Belgium. Electronic address: laurent.godinas@uclouvain.be. 2. Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l׳Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 3. Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l׳Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia. 4. Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l׳Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France; Department of Medicine, Division of Respirology University of Calgary, Calgary, Alberta, Canada. 5. AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service des Explorations Fonctionnelles de la Respiration, de l׳Exercice et de la Dyspnée, Paris, France; Sorbonne Universités, UPMC Université Paris 06, UMR_S 1158, Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France.
Abstract
BACKGROUND: Cardiopulmonary exercise testing (CPET) is frequently used for the evaluation of patients with pulmonary hypertension (PH). Non-operable distal chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subgroup of PH where microvascular disease resembling pulmonary arterial hypertension (PAH) may predominate and efficacious medical therapy is now available. However, little is known regarding the detailed CPET profile of patients with distal CTEPH, and whether ventilation and gas exchange responses are different from PAH. METHODS: Forty-nine consecutive patients with non-operable distal CTEPH according to multidisciplinary team assessment and 45 PAH patients underwent CPET and right heart catheterization. Patients were followed up for a median of 3.2 years (interquartile range: 1.8 to 4.4). RESULTS: Pulmonary hemodynamics were similar in distal CTEPH and PAH groups, but patients with distal CTEPH achieved a lower percent predicted peak oxygen consumption (59 ± 13% vs 66 ± 14%, p < 0.05). At peak exercise, higher physiologic dead-space fraction (VD/VT) (0.45 ± 0.07 vs 0.35 ± 0.07, p < 0.0001) and higher arterial-to-end-tidal carbon dioxide gradient (9 ± 3 vs 5 ± 3 mm Hg, p < 0.0001) were observed in distal CTEPH compared with PAH. Ventilatory efficiency, expressed as VE/VCO2 slope, was also more impaired in distal CTEPH (52.2 ± 10.1 vs 43.8 ± 8.4 liters/min, p < 0.0001). In the distal CTEPH group only, higher VD/VT was associated with lower peak oxygen consumption (r = -0.46, p = 0.003) and worse survival. CONCLUSIONS: Compared with PAH, a distinct pattern of response to exercise was observed in distal CTEPH, characterized by increased dead-space ventilation that resulted in worse ventilatory efficiency and greater impairment of exercise capacity. In distal CTEPH, dead-space ventilation correlated with exercise capacity and was associated with survival.
BACKGROUND: Cardiopulmonary exercise testing (CPET) is frequently used for the evaluation of patients with pulmonary hypertension (PH). Non-operable distal chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subgroup of PH where microvascular disease resembling pulmonary arterial hypertension (PAH) may predominate and efficacious medical therapy is now available. However, little is known regarding the detailed CPET profile of patients with distal CTEPH, and whether ventilation and gas exchange responses are different from PAH. METHODS: Forty-nine consecutive patients with non-operable distal CTEPH according to multidisciplinary team assessment and 45 PAH patients underwent CPET and right heart catheterization. Patients were followed up for a median of 3.2 years (interquartile range: 1.8 to 4.4). RESULTS: Pulmonary hemodynamics were similar in distal CTEPH and PAH groups, but patients with distal CTEPH achieved a lower percent predicted peak oxygen consumption (59 ± 13% vs 66 ± 14%, p < 0.05). At peak exercise, higher physiologic dead-space fraction (VD/VT) (0.45 ± 0.07 vs 0.35 ± 0.07, p < 0.0001) and higher arterial-to-end-tidal carbon dioxide gradient (9 ± 3 vs 5 ± 3 mm Hg, p < 0.0001) were observed in distal CTEPH compared with PAH. Ventilatory efficiency, expressed as VE/VCO2 slope, was also more impaired in distal CTEPH (52.2 ± 10.1 vs 43.8 ± 8.4 liters/min, p < 0.0001). In the distal CTEPH group only, higher VD/VT was associated with lower peak oxygen consumption (r = -0.46, p = 0.003) and worse survival. CONCLUSIONS: Compared with PAH, a distinct pattern of response to exercise was observed in distal CTEPH, characterized by increased dead-space ventilation that resulted in worse ventilatory efficiency and greater impairment of exercise capacity. In distal CTEPH, dead-space ventilation correlated with exercise capacity and was associated with survival.
Authors: Jens Spiesshoefer; Britta Bannwitz; Michael Mohr; Simon Herkenrath; Winfried Randerath; Paolo Sciarrone; Christian Thiedemann; Hartmut Schneider; Andrew T Braun; Michele Emdin; Claudio Passino; Michael Dreher; Matthias Boentert; Alberto Giannoni Journal: Sleep Breath Date: 2020-08-22 Impact factor: 2.816