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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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.
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.
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.
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.
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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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