Skip to main content

Advertisement

Log in

Pulmonary veno-occlusive disease: the role of CT

  • Chest Radiology
  • Published:
La radiologia medica Aims and scope Submit manuscript

Abstract

Objective

The authors sought to evaluate the diagnostic accuracy of high-resolution computed tomography (HRCT) in the detection of pulmonary veno-occlusive disease (PVOD) in patients with pre-capillary pulmonary arterial hypertension (PAH) of unknown aetiology, and to identify the role of CT in diagnosis and therapy.

Materials and methods

The CT scans of 96 patients were retrospectively reviewed and assessed for specific HRCT findings: ground-glass opacities, septal lines and mediastinal lymph nodal enlargement (short diameter ≥1 cm). According to the HRCT findings, patients were divided into PVOD-suspicious and not PVOD-suspicious. Subsequently, a clinical-instrumental evaluation was performed, and the response to therapy and histopathological reports were evaluated.

Results

Radiological evaluation based on HRCT findings revealed 29 patients as PVOD-suspicious and 67 as not PVOD-suspicious. The final diagnosis was PVOD in 22 patients and idiopathic PAH in 74 patients. The CT scan showed 95.5 % sensitivity, 89 % specificity, 72.5 % positive predictive value, and 98.5 % negative predictive value, with a diagnostic accuracy of 90.5 % in identifying patients with PVOD.

Conclusions

Chest CT can be considered a screening test in the assessment of patients with PAH of unknown aetiology, and the radiologist can help the clinician to identify patients with CT findings that make PVOD highly probable.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Holcomb BW Jr, Loyd JE, Ely Ew et al (2000) Pulmonary veno-occlusive disease: a case series and new observations. Chest 118:1671–1679

    Article  PubMed  Google Scholar 

  2. Takeda Y, Yamamoto K, Tomimoto S et al (2011) Pulmonary venous occlusion and death in pulmonary arterial hypertension: survival analyses using radiographic surrogates. BMC Pulm Med 11:47. doi:10.1186/1471-2466-11-47

    Article  PubMed Central  PubMed  Google Scholar 

  3. Mandel J, Mark EJ, Hales CA (2000) Pulmonary venoocclusive disease. Am J Respir Crit Care Med 162:1964–1973

    Article  CAS  PubMed  Google Scholar 

  4. Valmary S, Dorfmüller P, Montani D (2010) Human gamma-herpesviruses EBV and HHV-8 are not detected in the lungs of patients with severe pulmonary arterial hypertension. Chest 139:1310–1316

    Article  PubMed  Google Scholar 

  5. Swift GL, Gibbs A, Campbell IA et al (1993) Pulmonary veno-occlusive disease and Hodgkin’s lymphoma. Eur Respir J 6:596–598

    CAS  PubMed  Google Scholar 

  6. Ibrahim NB, Burnley H, Gaber KA et al (2005) Segmental pulmonary veno-occlusive disease secondary to lung cancer. J Clin Pathol 58:434–436

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  7. Knight BK, Rose AG (1985) Pulmonary veno-occlusive disease after chemotherapy. Thorax 40:874–875

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Ozsoyoglu A, Swartz J, Farver CF et al (2006) High-resolution computed tomographic imaging and pathologic features of pulmonary veno-occlusive disease: a review of three patients. Curr Probl Diagn Radiol 35:219–223

    Article  PubMed  Google Scholar 

  9. Eltorky MA, Headley AS, Winer-Muram H et al (1994) Pulmonary capillary hemangiomatosis: a clinicopathologic review. Ann Thorac Surg 57:772–776

    Article  CAS  PubMed  Google Scholar 

  10. Carrington CB, Liebow AA (1970) Pulmonary venoocclusive disease. Hum Pathol 1:322–324

    Article  CAS  PubMed  Google Scholar 

  11. Wagenvoort CA, Wagenvoort N, Takahashi T (1985) Pulmonary veno-occlusive disease: involvement of pulmonary arteries and review of the literature. Hum Pathol 16:1033–1041

    Article  CAS  PubMed  Google Scholar 

  12. Frazier AA, Franks TJ, Mohammed TL et al (2007) From the archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics 27:867–882

    Article  PubMed  Google Scholar 

  13. Galiè N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537

    Article  PubMed  Google Scholar 

  14. Nawaz S, Dobersen MJ, Blount SG Jr et al (1990) Florid pulmonary veno-occlusive disease. Chest 98:1037–1039

    Article  CAS  PubMed  Google Scholar 

  15. Pietra GG, Capron F, Stewart S et al (2004) Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol 43:25–32

    Article  Google Scholar 

  16. Grunig E, Barner A, Bell M et al (2011) Non-invasive diagnosis of pulmonary hypertension: ESC/ERS Guidelines with updated commentary of the Cologne consensus conference 2011. Int J Cardiol 154S:S3–S12

    Article  Google Scholar 

  17. Montani D, Kemp K, Dorfmuller P et al (2009) Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and difference. Semin Respir Crit Care Med 30:411–412

    Article  PubMed  Google Scholar 

  18. Resten A, Maitre S, Humbert M et al (2004) Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol 183:65–70

    Article  PubMed  Google Scholar 

  19. Resten A, Maître S, Capron F et al (2003) Pulmonary hypertension: CT findings in pulmonary veno-occlusive disease. J Radiol 84:1739–1745

    CAS  PubMed  Google Scholar 

  20. Humbert M, Maître S, Capron F et al (1998) Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 157:1681–1685

    Article  CAS  PubMed  Google Scholar 

  21. Masters K, Bennett S (2013) Pulmonary veno-occlusive disease: an uncommon cause of pulmonary hypertension. BMJ Case Rep. doi:10.1136/bcr-2012-007752

    PubMed  Google Scholar 

  22. Montani D, Jaıs X, Price LC et al (2009) Cautious epoprostenol therapy is a safe bridge to lung transplantation in pulmonary veno-occlusive disease. Eur Respir J 34:1348–1356

    Article  CAS  PubMed  Google Scholar 

  23. O’Callaghan DS, Dorfmuller P, Jaïs X (2011) Pulmonary veno-occlusive disease: the bête noire of pulmonary hypertension in connective tissue diseases? Presse Med 40:65–78

    Google Scholar 

  24. Montani D, Achouh L, Dorfmuller P et al (2008) Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine 87:220–233

    Article  PubMed  Google Scholar 

  25. Montani D, Price LC, Dorfmuller P et al (2009) Pulmonary veno-occlusive disease. Eur Respir J 33:189–200

    Article  CAS  PubMed  Google Scholar 

  26. Montani D, O’Callaghan D, Savale L et al (2010) Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med 104:23–32

    Article  Google Scholar 

  27. Rabiller A, Jaïs X, Hamid A (2006) Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J 27:108–113

    Article  CAS  PubMed  Google Scholar 

Download references

Conflict of interest

Giangaspare Mineo, Domenico Attinà, Martina Mughetti, Caterina Balacchi, Fiorella De Luca, Fabio Niro, Federica Ciccarese, Luigi Lovato, Vincenzo Russo, Francesco Buia, Cecilia Modolon, Alessandra Manes, Massimiliano Palazzini, Nazareno Galiè, Maurizio Zompatori declare no conflict of interest with the publication of this article.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Giangaspare Mineo.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mineo, G., Attinà, D., Mughetti, M. et al. Pulmonary veno-occlusive disease: the role of CT. Radiol med 119, 667–673 (2014). https://doi.org/10.1007/s11547-013-0363-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11547-013-0363-y

Keywords

Navigation