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Eur Respir J. 2019 Mar 28. pii: 1802004. doi: 10.1183/13993003.02004-2018. [Epub ahead of print]

Risk Assessment in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Author information

1
University Paris-Sud, Faculté de Médecine, Université Paris Saclay, Le Kremlin-Bicêtre, France marc.humbert@aphp.fr.
2
Assistance-Publique-Hôpitaux de Paris, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
3
INSERM Unité 999, Le Kremlin-Bicêtre, France.
4
Division of Pulmonary, Critical Care and Sleep Medicine, Tufts Medical Center, Boston, Massachusetts, United States of America.
5
University of Giessen and Marburg Lung Center, Giessen, Germany, member of the German Center for Lung Research (DZL).
6
Department of Medicine, Imperial College London, London, United Kingdom.
7
Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, Pennsylvania.
8
Chrestos Concept GmbH & Co. KG, Essen, Germany.
9
Bayer AG, Berlin, Germany.
10
Clinic for Respiratory Medicine, Hannover Medical School, Hannover, Germany, member of the German Center for Lung Research (DZL).

Abstract

RATIONALE:

Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate, or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension in the French, Swedish, and COMPERA registries.

OBJECTIVE:

To investigate 3 abbreviated risk stratification methods for patients with mostly prevalent pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, in patients from the PATENT-1/-2 and CHEST-1/-2 studies of riociguat.

METHODS:

Risk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.

MEASUREMENTS AND MAIN RESULTS:

With all 3 methods, riociguat improved risk group/strata in patients with pulmonary arterial hypertension after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving ≥1 low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening, compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension.

CONCLUSIONS:

This analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in pulmonary arterial hypertension. Further assessment of these methods in patients with chronic thromboembolic pulmonary hypertension is warranted.

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