Skip to main content
Log in

Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients

  • Chest
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objectives

To correlate the severity of lung neovascularity (Sheehan vessels) with the cause and haemodynamic severity of pulmonary arterial hypertension (PAH), pulmonary artery (PA) size and heart disease type in patients with PH associated with congenital heart diseases (PAH-CHD) and idiopathic PH (IPAH).

Methods

We reviewed the HRCT and CT pulmonary angiography studies of 87 patients with PAH-CHD and 111 with IPAH; all had undergone right heart catheterisation. We evaluated the PA size and severity of neovascularity on CT.

Results

Neovascularity, which was found in 72% of PAH-CHD (56% with Eisenmenger’s syndrome) and in 22% of IPAH patients, is significantly related to the severity of PH and all patients with severe neovascularity had intermediate or high PH. All PAH-CHD patients had a dilated PA with a greater risk of developing severe dilatation (diameter >5 cm). The neovascularity correlated with the PA size only in IPAH.

Conclusions

Neovascularity even if not pathognomonic for PAH-CHD, is significantly more common in these patients, especially in Eisenmenger’s syndrome. It is often the first CT sign to indicate the severity of PH in PAH-CHD and IPAH. A neovascularity ≥5 on CT indicates a intermediate or high PH.

Key Points

Large retrospective studying specific lung disorders in patients with pulmonary arterial hypertension.

Neovascularity is often the first CT sign indicating the severity of pulmonary hypertension Alterations of lung parenchyma on CT in pulmonary hypertension are described

The first study to assess the severity of pulmonary hypertension by CT

If substantiated, CT might eventually replace some cardiac catheterisation for evaluating PH

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.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. 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 

  2. Kovacs G, Berghold A, Scheidl S et al (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects. A systematic review. Eur Respir J 34:888–894

    Article  PubMed  CAS  Google Scholar 

  3. Naeije R, Melot C, Niset G et al (1993) Mechanisms of improved arterial oxygenation after peripheral chemoreceptor stimulation during hypoxic exercise. J Appl Physiol 74:1666–1671

    PubMed  CAS  Google Scholar 

  4. Sheehan R, Perloff JK, Fishbein MC et al (2005) Pulmonary neovascularity, a distinctive radiographic finding in Eisenmenger syndrome. Circulation 112:2778–2785

    Article  PubMed  Google Scholar 

  5. Griffin N, Allen D, Wort J et al (2007) Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology? Clin Radiol 62:587–595

    Article  PubMed  CAS  Google Scholar 

  6. Perloff JK, Hart EM, Greaves M et al (2003) Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 92:182–187

    Article  PubMed  Google Scholar 

  7. International Labour Office (ILO) (2002) Guidelines for the use of the ILO International Classification of Radiographs of Pneumoconioses, Revised Edition 2000 (Occupational Safety and Health Series, No. 22). International Labour Office, Geneva

    Google Scholar 

  8. Castañer E, Gallardo X, Rimola F et al (2006) Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics 26:349–371

    Article  PubMed  Google Scholar 

  9. Guthaner DF, Wexler L, Harell G (1979) CT demonstration of cardiac structures. AJR Am J Roentgenol 133:75–81

    PubMed  CAS  Google Scholar 

  10. Kuriyama K, Gamsu G, Stern RG et al (1984) CT-determined pulmonary artery diameters in predicting pulmonary hypertension. Invest Radiol 19:16–22

    Article  PubMed  CAS  Google Scholar 

  11. Haimovici JB, Trotman-Dickenson B, Halpern EF et al (1997) Relationship between pulmonary artery diameter at computed tomography and pulmonary artery pressures at right-sides heart catheterization. Massachusetts General Hospital Lung Transplantation Program. Acad Radiol 4:327–334

    Article  PubMed  CAS  Google Scholar 

  12. Devaraj A, Wells AU, Meister MG et al (2008) The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 249:1042–1049

    Article  PubMed  Google Scholar 

  13. Zisman DA, Karlamangla AS, Ross DJ et al (2007) High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 132:773–779

    Article  PubMed  Google Scholar 

  14. Ng CS, Wells AU, Padley SP (1999) A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imag 14:270–278

    Article  CAS  Google Scholar 

  15. Chang SM, Lin CC, Hsiao SH et al (2007) Pulmonary hypertension and left heart function: insights from tissue Doppler imaging and myocardial performance index. Ecocardiography 24:366–373

    Article  Google Scholar 

  16. Frazier AA, Galvin JR, Franks TJ et al (2000) From the archives of the AFIP: pulmonary vasculature-hypertension and infarction. Radiographics 20:491–524

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Cecilia Modolon.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Modolon, C., Attinà, D., Buia, F. et al. Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients. Eur Radiol 22, 1059–1066 (2012). https://doi.org/10.1007/s00330-011-2347-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-011-2347-4

Keywords

Navigation