Yu Taniguchi1, Xavier Jaïs2, Mitja Jevnikar1, Athénaïs Boucly1, Jason Weatherald3, Philippe Brenot4, Olivier Planche5, Florence Parent1, Laurent Savale1, Elie Fadel6, David Montani1, Marc Humbert1, Olivier Sitbon1, Gérald Simonneau1. 1. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; Inserm UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France. 2. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; Inserm UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Electronic address: xavier.jais@aphp.fr. 3. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; University of Calgary, Department of Medicine, Division of Respirology, Calgary, Canada; Libin Cardiovascular Institute of Alberta, Calgary, Canada. 4. Inserm UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France; Service de Radiologie, Hôpital Marie Lannelongue, Le Plessis Robinson, France. 5. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; Inserm UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France; AP-HP, Service de Radiologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. 6. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; Inserm UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France; Service de Chirurgie Thoracique, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
Abstract
BACKGROUND: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) have recently evolved with the availability of balloon pulmonary angioplasty (BPA) and pulmonary vasodilators. Our aim was to analyze the prognostic variables associated with long-term outcome in a cohort of patients with not-operated CTEPH. METHODS: From January 2006 to December 2016, 343 newly diagnosed consecutive patients with not-operated CTEPH were diagnosed and followed up in the French Reference Center for Pulmonary Hypertension. Overall long-term survival and prognostic factors according to the diagnosis period (early period, 2006-2012, vs recent period, since 2013, i.e., one year before availability of BPA) were analyzed. RESULTS: In the overall population, baseline New York Heart Association functional class, right atrial pressure, 6-minute walk distance (6MWD), and diagnosis period were independent predictors of survival. The 1- and 3-year survival rates of patients diagnosed in the recent period (n = 170) were 91.6% and 85.0%, compared with 89.0% and 74.3% in patients diagnosed in the early period (n = 173), respectively (p = 0.030). Multivariate analysis from patients diagnosed in the recent period found that baseline 6MWD (per 20 m increase in distance) (hazard ratio [HR], 0.879; 95% confidence interval [CI], 0.832-0.928, p < 0.001) and BPA (HR, 0.307; 95% CI, 0.099-0.957; p = 0.042) were independently associated with survival. CONCLUSIONS: Survival of not-operated patients with CTEPH has significantly improved in the recent management era. New treatment options, including BPA, might have the potential to improve the prognosis of patients with inoperable CTEPH.
BACKGROUND: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) have recently evolved with the availability of balloon pulmonary angioplasty (BPA) and pulmonary vasodilators. Our aim was to analyze the prognostic variables associated with long-term outcome in a cohort of patients with not-operated CTEPH. METHODS: From January 2006 to December 2016, 343 newly diagnosed consecutive patients with not-operated CTEPH were diagnosed and followed up in the French Reference Center for Pulmonary Hypertension. Overall long-term survival and prognostic factors according to the diagnosis period (early period, 2006-2012, vs recent period, since 2013, i.e., one year before availability of BPA) were analyzed. RESULTS: In the overall population, baseline New York Heart Association functional class, right atrial pressure, 6-minute walk distance (6MWD), and diagnosis period were independent predictors of survival. The 1- and 3-year survival rates of patients diagnosed in the recent period (n = 170) were 91.6% and 85.0%, compared with 89.0% and 74.3% in patients diagnosed in the early period (n = 173), respectively (p = 0.030). Multivariate analysis from patients diagnosed in the recent period found that baseline 6MWD (per 20 m increase in distance) (hazard ratio [HR], 0.879; 95% confidence interval [CI], 0.832-0.928, p < 0.001) and BPA (HR, 0.307; 95% CI, 0.099-0.957; p = 0.042) were independently associated with survival. CONCLUSIONS: Survival of not-operated patients with CTEPH has significantly improved in the recent management era. New treatment options, including BPA, might have the potential to improve the prognosis of patients with inoperable CTEPH.
Authors: Gudula J A M Boon; Wilbert B van den Hout; Stefano Barco; Harm Jan Bogaard; Marion Delcroix; Menno V Huisman; Stavros V Konstantinides; Lilian J Meijboom; Esther J Nossent; Petr Symersky; Anton Vonk Noordegraaf; Frederikus A Klok Journal: ERJ Open Res Date: 2021-09-06
Authors: Christina A Eichstaedt; Jeremias Verweyen; Michael Halank; Nicola Benjamin; Christine Fischer; Eckhard Mayer; Stefan Guth; Christoph B Wiedenroth; Benjamin Egenlauf; Satenik Harutyunova; Panagiota Xanthouli; Alberto M Marra; Heinrike Wilkens; Ralf Ewert; Katrin Hinderhofer; Ekkehard Grünig Journal: Int J Mol Sci Date: 2020-05-08 Impact factor: 5.923
Authors: Szymon Darocha; Aleksander Araszkiewicz; Marcin Kurzyna; Marta Banaszkiewicz; Stanisław Jankiewicz; Anna Dobosiewicz; Sylwia Sławek-Szmyt; Magdalena Janus; Maciej Grymuza; Arkadiusz Pietrasik; Tatiana Mularek-Kubzdela; Piotr Kędzierski; Radosław Pietura; Dariusz Zieliński; Andrzej Biederman; Maciej Lesiak; Adam Torbicki Journal: J Clin Med Date: 2021-03-03 Impact factor: 4.241