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Connective tissue disease associated with pulmonary arterial hypertension: management of a patient with severe haemodynamic impairment

Olivier Sitbon, Laurent Bertoletti
European Respiratory Review 2014 23: 505-509; DOI: 10.1183/09059180.00009214
Olivier Sitbon
1Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. 2AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. 3INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 4Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord, CHU de Saint-Etienne, Saint-Etienne, France. 5Université Jean-Monnet, Groupe de Recherche sur la Thrombose (EA 3065), Inserm, CIC1408, Saint-Etienne, France
1Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. 2AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. 3INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 4Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord, CHU de Saint-Etienne, Saint-Etienne, France. 5Université Jean-Monnet, Groupe de Recherche sur la Thrombose (EA 3065), Inserm, CIC1408, Saint-Etienne, France
1Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. 2AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. 3INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 4Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord, CHU de Saint-Etienne, Saint-Etienne, France. 5Université Jean-Monnet, Groupe de Recherche sur la Thrombose (EA 3065), Inserm, CIC1408, Saint-Etienne, France
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  • For correspondence: olivier.sitbon@bct.aphp.fr
Laurent Bertoletti
1Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. 2AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. 3INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 4Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord, CHU de Saint-Etienne, Saint-Etienne, France. 5Université Jean-Monnet, Groupe de Recherche sur la Thrombose (EA 3065), Inserm, CIC1408, Saint-Etienne, France
1Faculté de Médecine, Université Paris-Sud, Le Kremlin-Bicêtre, France. 2AP–HP, Centre de Référence de l’hypertension pulmonaire sévère, Service de pneumologie et soins intensifs, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. 3INSERM U-999, LabEx LERMIT, Centre chirurgical Marie Lannelongue, Le Plessis-Robinson, France. 4Service de Médecine Vasculaire et Thérapeutique, Hôpital Nord, CHU de Saint-Etienne, Saint-Etienne, France. 5Université Jean-Monnet, Groupe de Recherche sur la Thrombose (EA 3065), Inserm, CIC1408, Saint-Etienne, France
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  • Table 1. Patient risk assessment
    ParameterStable and satisfactoryStable and not satisfactoryUnstable and deteriorating
    ESC/ERS guidelinesCurrent caseESC/ERS guidelinesCurrent caseESC/ERS guidelinesCurrent case
    Clinical RV failureNoYesYes
    Rate of progressionSlowRapidRapid
    SyncopeNoNoYes
    NYHA FCI, IIIIIIVIV
    6MWD m>500#300–500<300220
    V′O2peak mL·min−1·kg−1>1512–15<12Not determined
    BNP/NT-proBNPNormal/near normalElevatedVery elevated/increasingVery elevated
    Echocardiography findingsNo PE and TAPSE >2.0 cmTAPSE 1.5–2.0 cmPE and/or TAPSE <1.5 cmPE and TAPSE 1.2 cm
    HaemodynamicsRAP <8 mmHg and CI
    ≥2.5–3.0 L·min−1·m−2
    RAP 8–15 mmHg or CI
    2.0–2.5 L·min−1·m−2
    RAP 12 mmHgRAP >15 mmHg and/or CI ≤2.0 L·min−1·m−2CI 1.6 L·min−1·m−2
    • ESC: European Society of Cardiology; ERS: European Respiratory Society; RV: right ventricular; NYHA: New York Heart Association; FC: functional class; 6MWD: 6-min walking distance; V′O2peak: peak oxygen uptake; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-BNP; PE: pericardial effusion; TAPSE: tricuspid annular plane systolic excursion; RAP: right atrial pressure; CI: cardiac index. #: depending on age, height, weight, aetiology of pulmonary arterial hypertension and presence of comorbidities. Adapted from [3] with permission from the publisher.

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Connective tissue disease associated with pulmonary arterial hypertension: management of a patient with severe haemodynamic impairment
Olivier Sitbon, Laurent Bertoletti
European Respiratory Review Dec 2014, 23 (134) 505-509; DOI: 10.1183/09059180.00009214

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Connective tissue disease associated with pulmonary arterial hypertension: management of a patient with severe haemodynamic impairment
Olivier Sitbon, Laurent Bertoletti
European Respiratory Review Dec 2014, 23 (134) 505-509; DOI: 10.1183/09059180.00009214
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