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Clinical Cases in Chronic Thromboembolic Pulmonary Hypertension pp 109–119Cite as

Case 12: Pulmonary Veno-Occlusive Disease …but It Started Out as CTEPH

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Part of the book series: Clinical Cases in Cardiology ((CCC))

Abstract

The diagnosis of pulmonary veno-occlusive disease can be very challenging. Although lung ventilation-perfusion scintigraphy is typically normal in this disease, an abnormal scan can be observed in patients with PVOD. This case presentation illustrates this point, and how the abnormal lung scan and pulmonary angiogram was initially interpreted as CTEPH in this 23 year old male.

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Further Reading

  • Bailey CL, Channick RM, Auger WR, Fedullo PF, Kerr KM, Yung GL, Rubin LJ. “High probability” perfusion lung scans in pulmonary veno-occlusive disease. Am J Resp Crit Care Med. 2000;162:1974–8.

    Article  CAS  Google Scholar 

  • Balke L, Both M, Winkler C, Schreiber T, Röcken C, Koch K, Bewig B. Scintigraphy leading to the misdiagnosis of chronic thromboembolic disease in a patient with pulmonary veno-occlusive disease. Circulation. 2016;133(16):1627–8.

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  • Montani D, Girerd B, Jais X, Levy M, Amar D, Savale L, Dorfmuller P, Seferian A, Lau EM, Eyries M, Le Pavec J, Parent F, Bonnet D, Soubrier F, Fadel E, Sitbon O, Simonneau G, Humbert M. Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study. Lancet Respir Med. 2017;5(2):125–34.

    Article  CAS  Google Scholar 

  • Seferian A, Helal B, Jaïs X, Girerd B, Price LC, Günther S, Savale L, Dorfmüller P, Parent F, Sitbon O, Humbert M, Simonneau G, Montani D. Ventilation/perfusion lung scan in pulmonary veno-occlusive disease. Eur Respir J. 2012;40:75–83.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Pulmonary Hypertension Program, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital, Detroit, MI, USA

    Rana Awdish & Sara Hegab

  2. Pulmonary Hypertension Program, Heart and Vascular Institute, St. John Providence Ascension Health, Southfield, MI, USA

    Rana Awdish & Sara Hegab

  3. School of Medicine, Wayne State University, Detroit, MI, USA

    Rana Awdish & Sara Hegab

  4. Department of Radiology, Imperial College Hospitals, London, UK

    Deepa Gopalan

  5. Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA

    William R. Auger

Authors
  1. Rana Awdish
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  2. Sara Hegab
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  3. Deepa Gopalan
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  4. William R. Auger
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Corresponding author

Correspondence to Rana Awdish .

Editor information

Editors and Affiliations

  1. Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA

    William R. Auger

  2. Department of Radiology, Imperial College Hospitals, London, UK

    Deepa Gopalan

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Awdish, R., Hegab, S., Gopalan, D., Auger, W.R. (2020). Case 12: Pulmonary Veno-Occlusive Disease …but It Started Out as CTEPH. In: Auger, W., Gopalan, D. (eds) Clinical Cases in Chronic Thromboembolic Pulmonary Hypertension. Clinical Cases in Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-030-17366-1_12

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  • DOI: https://doi.org/10.1007/978-3-030-17366-1_12

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-17365-4

  • Online ISBN: 978-3-030-17366-1

  • eBook Packages: MedicineMedicine (R0)

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