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Pulmonary hypertension and chronic lung disease: where are we headed?

Davide Elia, Antonella Caminati, Maurizio Zompatori, Roberto Cassandro, Chiara Lonati, Francesca Luisi, Giuseppe Pelosi, Steeve Provencher, Sergio Harari
European Respiratory Review 2019 28: 190065; DOI: 10.1183/16000617.0065-2019
Davide Elia
1U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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Antonella Caminati
1U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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  • For correspondence: lafitta@libero.it
Maurizio Zompatori
2U.O. di Radiologia Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
3Radiologia, Università di Bologna. Ospedale O. Malpighi, Bologna, Italy
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Roberto Cassandro
1U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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Chiara Lonati
4U.O. di Medicina Generale, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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Francesca Luisi
1U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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Giuseppe Pelosi
5Servizio di Anatomia Patologica, Polo Scientifico e Tecnologico, Multimedica Srl, IRCCS, Milan, Italy
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Steeve Provencher
6Institut de Cardiologie et de Pneumologie de Québec, Quebec, Canada
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Sergio Harari
1U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
4U.O. di Medicina Generale, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
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  • ORCID record for Sergio Harari
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    FIGURE 1

    Measures obtained from the computed tomography scan that are useful for the radiological suspicion of pulmonary hypertension (PH). Main pulmonary artery (MPA) dilatation values >29 mm in men and >27 mm in women are strongly related to PH (sensitivity 87%, specificity 89%), when its value is >35 mm the specificity reaches 100%. Right and left pulmonary artery (RPA and LPA, respectively) diameter>18 mm is abnormal. MPA dilatation and an ascending aorta (AAO) diameter ratio >1, in individuals <50 years old and with diffuse pulmonary fibrosis, is highly specific for hypertension (specificity >90%, sensitivity 70%). In patients with severe COPD this ratio is more accurate than echocardiography.

Tables

  • Figures
  • TABLE 1

    Updated clinical classification of pulmonary hypertension (PH)

    1 PAH
    • 1.1 Idiopathic PAH

    • 1.2 Heritable PAH

    • 1.3 Drug- and toxin-induced PAH

    • 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

    • 1.6 PAH with overt features of venous/capillaries (PVOD/PCH) involvement

    • 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
    • 4.1 Chronic thromboembolic PH

    • 4.2 Other pulmonary artery obstructions

    5 PH with unclear and/or multifactorial mechanisms
    • 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. Reproduced from [8] with permission from the publisher.

    • TABLE 2

      Principle variables in 6-min walk related to a poor prognosis in patients affected by idiopathic pulmonary fibrosis

      Value predictors of a poor prognosis
      Distance m<250
      SpO2 nadir %<88
      HRR bpm<13
      DSP %·m<200

      SpO2: arterial oxygen saturation measured by pulse oximetry; HRR: heart rate recovery after 1 min following the test; DSP: distance saturation product, the product of the 6-min walk distance and the lowest registered SpO2.

      • TABLE 3

        Principle variables in 6-min walk test related to a poor prognosis in patients affected by COPD

        Value predictors of a poor prognosis
        Distance m≤334
        6MWspeed m·s−1≤0.8
        6MWW m·kg−1≤20 000
        DSP %·m≤290

        6MWspeed: calculated by dividing the 6-min walk distance by the total walking time; 6MWW: 6-min walking work, the product of the 6-min walking distance and the patient weight; DSP: distance saturation product, the product of the 6-min walk distance and the lowest registered arterial oxygen saturation measured by pulse oximetry.

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        Pulmonary hypertension and chronic lung disease: where are we headed?
        Davide Elia, Antonella Caminati, Maurizio Zompatori, Roberto Cassandro, Chiara Lonati, Francesca Luisi, Giuseppe Pelosi, Steeve Provencher, Sergio Harari
        European Respiratory Review Sep 2019, 28 (153) 190065; DOI: 10.1183/16000617.0065-2019

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        Pulmonary hypertension and chronic lung disease: where are we headed?
        Davide Elia, Antonella Caminati, Maurizio Zompatori, Roberto Cassandro, Chiara Lonati, Francesca Luisi, Giuseppe Pelosi, Steeve Provencher, Sergio Harari
        European Respiratory Review Sep 2019, 28 (153) 190065; DOI: 10.1183/16000617.0065-2019
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        • Article
          • Abstract
          • Abstract
          • Introduction
          • Biomarkers research for early diagnosis and prognosis
          • The 6-min walk test and cardiopulmonary exercise test
          • Echocardiography
          • Imaging
          • Treatment
          • Pulmonary rehabilitation
          • Conclusion
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