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Diagnosis of chronic thromboembolic pulmonary hypertension

Deepa Gopalan, Marion Delcroix, Matthias Held
European Respiratory Review 2017 26: 160108; DOI: 10.1183/16000617.0108-2016
Deepa Gopalan
1Imperial College Hospitals, London, UK
2Cambridge University Hospital, Cambridge, UK
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Marion Delcroix
3University Hospitals of Leuven, Leuven, Belgium
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Matthias Held
4Medical Mission Hospital, Dept of Internal Medicine, Center for Pulmonary Hypertension and Pulmonary Vascular Disease, Academic Teaching Hospital, Julius-Maximilian University of Würzburg, Würzburg, Germany
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  • FIGURE 1
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    FIGURE 1

    Cardiopulmonary exercise testing of a patient with chronic thromboembolic pulmonary hypertension showing fields 4, 6 and 9 of the Wasserman panel. a) Elevated slope of minute ventilation (V′E)/carbon dioxide output (V′CO2) ratio showing hyperventilation (field 4); b) elevated ventilator equivalents for oxygen (EQO2) and carbon dioxide (EQCO2) showing ineffective ventilation (field 6); c) low and decreasing end-tidal carbon dioxide tension (PETCO2), elevated alveolar–arterial oxygen tension gradient (PA–aO2) and elevated arterial–end-tidal carbon dioxide gradient (Pa–ETCO2) (field 9). PETO2: end-tidal oxygen tension; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; PO2: oxygen tension; PCO2: carbon dioxide tension.

  • FIGURE 2
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    FIGURE 2

    A 53-year-old patient with fibrosing mediastinitis. a) Anterior and b) posterior views from a perfusion scintigraphy scan show multiple segmental perfusion defects (arrows). c) Volume-rendered computed tomography angiography and d) magnetic resonance pulmonary angiography demonstrate attenuated pulmonary vasculature (arrows) with calcified mediastinal lymph nodes (arrowhead).

  • FIGURE 3
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    FIGURE 3

    A 45-year-old with pulmonary hypertension. a, b) Dual-energy computed tomography pulmonary angiography and c) corresponding magnetic resonance pulmonary angiography confirm chronic thromboembolic pulmonary hypertension. There is dilatation of the main pulmonary artery (PA), dilated right atrium (RA) and right ventricle (RV) with right ventricular hypertrophy, eccentric thrombus in right lower lobe and left main pulmonary artery (arrows), multiple segmental perfusion defects (*), proximal pulmonary arterial stenosis and webs (arrowheads).

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    FIGURE 4

    a) Magnetic resonance and b) catheter pulmonary angiography and c) computed tomography and d) catheter pulmonary angiography in two different cases of chronic thromboembolic pulmonary hypertension. There is good disease correlation on all three modalities, e.g. pouch defect on magnetic resonance image (arrows) and proximal stenosis in left lower lobe artery (arrowhead).

  • FIGURE 5
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    FIGURE 5

    A 50-year-old male with persistent abnormality on computed tomography (CT) despite anticoagulation for 1 year. a) CT scan showing an expansile low attenuation lesion in the right main pulmonary artery (arrow); b) 18F-fluorodeoxyglucose positron emission tomography with corresponding high uptake in keeping with pulmonary artery sarcoma. Note mediastinal soft tissue with increased uptake.

Supplementary Materials

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    Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.

    Figure S1. Still maximum-intensity projection image of a high-resolution magnetic resonance pulmonary angiogram demonstrating typical features of chronic thromboembolic pulmonary hypertension including pouch defect in right lower lobe (block arrow), tight trifurcation stenosis with post stenotic dilatation in left lower lobe (notched arrow). ERR-0108-2016_Suppl_Fig_S1

    Supplementary video. Rotating maximum-intensity projection of the magnetic resonance pulmonary angiographic dataset. ERR-0108-2016_Suppl_Video

  • Supplementary Material

    M. Delcroix ERR-0108-2016_Delcroix

    M. Held ERR-0108-2016_Held

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Vol 26 Issue 143 Table of Contents
European Respiratory Review: 26 (143)
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Diagnosis of chronic thromboembolic pulmonary hypertension
Deepa Gopalan, Marion Delcroix, Matthias Held
European Respiratory Review Mar 2017, 26 (143) 160108; DOI: 10.1183/16000617.0108-2016

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Diagnosis of chronic thromboembolic pulmonary hypertension
Deepa Gopalan, Marion Delcroix, Matthias Held
European Respiratory Review Mar 2017, 26 (143) 160108; DOI: 10.1183/16000617.0108-2016
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • The way into the diagnostic process: symptoms and clinical findings leading to the suspicion of CTEPH
    • Echocardiography
    • Cardiopulmonary exercise testing
    • Imaging
    • Conclusion
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  • Pulmonary vascular disease
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