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Haemodynamic definitions and updated clinical classification of pulmonary hypertension

Gérald Simonneau, David Montani, David S. Celermajer, Christopher P. Denton, Michael A. Gatzoulis, Michael Krowka, Paul G. Williams, Rogerio Souza
European Respiratory Journal 2019 53: 1801913; DOI: 10.1183/13993003.01913-2018
Gérald Simonneau
1Université Paris-Sud, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Service de Pneumologie, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Hôpital de Bicêtre, Le Kremlin-Bicêtre, France
2INSERM UMR_S999, LabEx LERMIT, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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David Montani
1Université Paris-Sud, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Service de Pneumologie, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Hôpital de Bicêtre, Le Kremlin-Bicêtre, France
2INSERM UMR_S999, LabEx LERMIT, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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  • ORCID record for David Montani
David S. Celermajer
3Faculty of Medicine and Health, The University of Sydney, Sydney, Australia
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Christopher P. Denton
4Centre for Rheumatology, Royal Free Campus, University College London, London, UK
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Michael A. Gatzoulis
5Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Trust, and the National Heart and Lung Institute, Imperial College London, London, UK
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Michael Krowka
6Transplant Center, Mayo Clinic, Rochester, MN, USA
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Paul G. Williams
7Center of Chest Disease and Critical Care, Milpark Hospital, Johannesburg, South Africa
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Rogerio Souza
8Pulmonary Circulation Unit, Pulmonary Division, Heart Institute (InCor), Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil
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Figures

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  • FIGURE 1
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    FIGURE 1

    The slope of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship is different in normal control versus pulmonary arterial hypertension (PAH) subjects. Reproduced and modified from [26] with permission.

  • FIGURE 2
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    FIGURE 2

    Knowledge of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship does not allow distinction between left heart disease (LHD) and pulmonary vascular disease (PVD) patients; knowledge of exercise pulmonary arterial wedge pressure is also required. TPR: total pulmonary resistance; WU: Wood Units. Reproduced from [29] with permission.

Tables

  • Figures
  • TABLE 1

    Haemodynamic definitions of pulmonary hypertension (PH)

    DefinitionsCharacteristicsClinical groups#
    Pre-capillary PHmPAP >20 mmHg1, 3, 4 and 5
    PAWP ≤15 mmHg
    PVR ≥3 WU
    Isolated post-capillary PH (IpcPH)mPAP >20 mmHg2 and 5
    PAWP >15 mmHg
    PVR <3 WU
    Combined pre- and post-capillary PH (CpcPH)mPAP >20 mmHg2 and 5
    PAWP >15 mmHg
    PVR ≥3 WU

    mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; WU: Wood Units. #: group 1: PAH; group 2: PH due to left heart disease; group 3: PH due to lung diseases and/or hypoxia; group 4: PH due to pulmonary artery obstructions; group 5: PH with unclear and/or multifactorial mechanisms.

    • TABLE 2 Updated clinical classification of pulmonary hypertension (PH)
      1 PAH
       1.1 Idiopathic PAH
       1.2 Heritable PAH
       1.3 Drug- and toxin-induced PAH (table 3)
       1.4 PAH associated with:
        1.4.1 Connective tissue disease
        1.4.2 HIV infection
        1.4.3 Portal hypertension
        1.4.4 Congenital heart disease
        1.4.5 Schistosomiasis
       1.5 PAH long-term responders to calcium channel blockers (table 4)
       1.6 PAH with overt features of venous/capillaries (PVOD/PCH) involvement (table 5)
       1.7 Persistent PH of the newborn syndrome
      2 PH due to left heart disease
       2.1 PH due to heart failure with preserved LVEF
       2.2 PH due to heart failure with reduced LVEF
       2.3 Valvular heart disease
       2.4 Congenital/acquired cardiovascular conditions leading to post-capillary PH
      3 PH due to lung diseases and/or hypoxia
       3.1 Obstructive lung disease
       3.2 Restrictive lung disease
       3.3 Other lung disease with mixed restrictive/obstructive pattern
       3.4 Hypoxia without lung disease
       3.5 Developmental lung disorders
      4 PH due to pulmonary artery obstructions (table 6)
       4.1 Chronic thromboembolic PH
       4.2 Other pulmonary artery obstructions
      5 PH with unclear and/or multifactorial mechanisms (table 7)
       5.1 Haematological disorders
       5.2 Systemic and metabolic disorders
       5.3 Others
       5.4 Complex congenital heart disease

      PAH: pulmonary arterial hypertension; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; LVEF: left ventricular ejection fraction.

      • TABLE 3

        Updated classification of drugs and toxins associated with PAH

        DefinitePossible
        AminorexCocaine
        FenfluraminePhenylpropanolamine
        Dexfenfluraminel-tryptophan
        BenfluorexSt John's wort
        MethamphetaminesAmphetamines
        DasatinibInterferon-α and -β
        Toxic rapeseed oilAlkylating agents
        Bosutinib
        Direct-acting antiviral agents against hepatitis C virus
        Leflunomide
        Indirubin (Chinese herb Qing-Dai)
      • TABLE 4

        Definitions of acute and long-term response

        Acute pulmonary vasoreactivity# for patients with idiopathic, hereditable or drug-induced PAHReduction of mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg
        Increased or unchanged cardiac output
        Long-term response to CCBsNew York Heart Association Functional Class I/II
        With sustained haemodynamic improvement (same or better than achieved in the acute test) after at least 1 year on CCBs only

        PAH: pulmonary arterial hypertension; mPAP: mean pulmonary arterial pressure; CCB: calcium channel blocker. #: nitric oxide (10–20 ppm) is recommended for performing vasoreactivity testing, but i.v. epoprostenol, i.v. adenosine or inhaled iloprost can be used as alternatives.

        • TABLE 5

          Signs evocative of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement

          Pulmonary function testsDecreased DLCO (frequently <50%)
          Severe hypoxaemia
          Chest HRCTSeptal lines
          Centrilobular ground-glass opacities/nodules
          Mediastinal lymph node enlargement
          Response to PAH therapyPossible pulmonary oedema
          Genetic backgroundBiallelic EIF2AK4 mutations
          Occupational exposureOrganic solvent (trichloroethylene)

          DLCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; PAH: pulmonary arterial hypertension.

          • TABLE 6

            Pulmonary hypertension (PH) due to pulmonary artery obstructions

            4.1 Chronic thromboembolic PH
            4.2 Other pulmonary artery obstructions
             4.2.1 Sarcoma (high or intermediate grade) or angiosarcoma
             4.2.2 Other malignant tumours
             Renal carcinoma
             Uterine carcinoma
             Germ cell tumours of the testis
             Other tumours
             4.2.3 Non-malignant tumours
             Uterine leiomyoma
             4.2.4 Arteritis without connective tissue disease
             4.2.5 Congenital pulmonary artery stenoses
             4.2.6 Parasites  Hydatidosis
          • TABLE 7

            Pulmonary hypertension with unclear and/or multifactorial mechanisms

            5.1 Haematological disordersChronic haemolytic anaemia
            Myeloproliferative disorders
            5.2 Systemic and metabolic disordersPulmonary Langerhans cell histiocytosis
            Gaucher disease
            Glycogen storage disease
            Neurofibromatosis
            Sarcoidosis
            5.3 OthersChronic renal failure with or without haemodialysis
            Fibrosing mediastinitis
            5.4 Complex congenital heart diseaseSee the Task Force article by Rosenzweig et al. [31] in this issue of the European Respiratory Journal
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          Haemodynamic definitions and updated clinical classification of pulmonary hypertension
          Gérald Simonneau, David Montani, David S. Celermajer, Christopher P. Denton, Michael A. Gatzoulis, Michael Krowka, Paul G. Williams, Rogerio Souza
          European Respiratory Journal Jan 2019, 53 (1) 1801913; DOI: 10.1183/13993003.01913-2018

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          Haemodynamic definitions and updated clinical classification of pulmonary hypertension
          Gérald Simonneau, David Montani, David S. Celermajer, Christopher P. Denton, Michael A. Gatzoulis, Michael Krowka, Paul G. Williams, Rogerio Souza
          European Respiratory Journal Jan 2019, 53 (1) 1801913; DOI: 10.1183/13993003.01913-2018
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