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The pathophysiology of chronic thromboembolic pulmonary hypertension

Gérald Simonneau, Adam Torbicki, Peter Dorfmüller, Nick Kim
European Respiratory Review 2017 26: 160112; DOI: 10.1183/16000617.0112-2016
Gérald Simonneau
1Assistance Publique–Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, Hôpital Marie Lannelongue, Paris Sud University, Paris, France
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  • For correspondence: gerald.simonneau@gmail.com
Adam Torbicki
2Dept of Pulmonary Circulation and Thromboembolic Diseases, Centre of Postgraduate Medical Education, Europejskie Centrum Zdrowia Otwock, Otwock, Poland
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Peter Dorfmüller
3Dept of Pathology, Paris Sud University, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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Nick Kim
4Division of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, CA, USA
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  • FIGURE 1
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    FIGURE 1

    Pathophysiology of chronic thromboembolic pulmonary hypertension (CTEPH). PH: pulmonary hypertension. Reproduced and modified from [4] with permission.

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    FIGURE 2

    Natural history of chronic thromboembolic pulmonary hypertension (CTEPH). PE: pulmonary embolism. Reproduced and modified from [2] with permission.

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    FIGURE 3

    Microvasculopathy in chronic thromboembolic pulmonary hypertension involving pulmonary arterioles, venules and capillaries. Schematic representation of anastomosis between systemic and pulmonary circulation through hypertrophic bronchial arteries and vasa vasorum. PA: pulmonary artery; PVOD: pulmonary veno-occlusive disease; PAH: pulmonary arterial hypertension. Reproduced and modified from [67] with permission.

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    FIGURE 4

    Patient with inoperable chronic thromboembolic pulmonary hypertension showing a typical aspect of subpleural hypoperfusion at the capillary phase of pulmonary angiography. Image kindly provided by P. Dartevelle, French National Reference Centre of Pulmonary Hypertension, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.

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    FIGURE 5

    Relationship between pulmonary vascular obstruction (PVO) and pulmonary vascular resistance in chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (PE). a) Patient AL: 24-year-old female with CTEPH. Percentage of PVO estimated on perfusion lung scan at 75%. Total occlusion of left lung and occlusion of right middle and lower lobes. Haemodynamics: mean pulmonary arterial pressure (PAP) 32 mmHg; cardiac index 1.7 L·min-1·m-2; total pulmonary vascular resistance (TPR) 18.8 mmHg·L-1·min·m2. Despite 75% PVO, the TPR was only 18.8. b) Patient BJ: 54-year-old female with CTEPH. Percentage of PVO estimated on perfusion lung scan at 35%; multiple bilateral segmental and subsegmental perfusion defects. Haemodynamics: mean PAP 45 mmHg; cardiac index 1.4 L·min-1·m-2; TPR 32.1 mmHg·L-1·min·m2. c) Relationship between percentage of PVO assessed by perfusion lung scan and TPR in patients with acute PE (n=31). A strong hyperbolic correlation was found. d) For a given degree of PVO, most patients with CTEPH (n=45) have higher TPR values than patients with acute PE (n=31), suggesting that, in addition to mechanical obstruction by organised clots, they have small-vessel disease. Patient AL is located on the hyperbolic correlation (no microvasculopathy), whereas patient BJ has a disproportionate and very high level of TPR compared to mild PVO (severe microvasculopathy). Reproduced and modified from [86] with permission.

Supplementary Materials

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    Table S1. Incidence of CTEPH confirmed by right heart catheterisation after an acute PE ERR-0112-2016_Supplementary_information

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    N. Kim ERR-0112-2016_Kim

    G. Simonneau ERR-0112-2016_Simonneau

    A. Torbicki ERR-0112-2016_Torbicki

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Vol 26 Issue 143 Table of Contents
European Respiratory Review: 26 (143)
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The pathophysiology of chronic thromboembolic pulmonary hypertension
Gérald Simonneau, Adam Torbicki, Peter Dorfmüller, Nick Kim
European Respiratory Review Mar 2017, 26 (143) 160112; DOI: 10.1183/16000617.0112-2016

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The pathophysiology of chronic thromboembolic pulmonary hypertension
Gérald Simonneau, Adam Torbicki, Peter Dorfmüller, Nick Kim
European Respiratory Review Mar 2017, 26 (143) 160112; DOI: 10.1183/16000617.0112-2016
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Relationship between PE and CTEPH
    • Thrombus nonresolution in CTEPH
    • Small-vessel disease in CTEPH
    • Molecular mechanisms of small-vessel disease
    • Summary and conclusions
    • Supplementary material
    • Disclosures
    • Acknowledgements
    • Footnotes
    • References
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Subjects

  • Pulmonary vascular disease
  • Mechanisms of lung disease
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More in this TOC Section

  • Medications for the treatment of pulmonary arterial hypertension
  • Air pollution, metabolites and respiratory health across the life-course
  • Air pollution as an early determinant of COPD
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