Skip to main content
SpringerLink
Log in

Imaging of Pulmonary Hypertension

  • Chapter
  • First Online:
Clinically Oriented Pulmonary Imaging

Part of the book series: Respiratory Medicine ((RM))

  • 2195 Accesses

Abstract

Pulmonary hypertension (PH) is a silent disease with many causes that comes to clinical attention late in its course. There are indirect features of PH found on noninvasive imaging studies, but the diagnosis of this disease and its therapeutic management still require right heart catheterization with pressure measurements of the pulmonary artery. In general, with chronic PH, the main pulmonary artery is enlarged, there is tapering of the peripheral pulmonary arteries, there is decreased vessel compliance from muscular hypertrophy of the arterial walls, and there is reduced pulmonary blood flow. This is accompanied by changes in the right heart including right ventricular (RV) hypertrophy, RV enlargement, RV dysfunction, and tricuspid regurgitation. In the acute setting, such as with massive pulmonary emboli, the abrupt change in pulmonary arterial pressure has a dramatic effect on right heart contractility. The peak velocity of the tricuspid regurgitation jet, as measured by echocardiography or MRI, is loosely correlated with pulmonary arterial pressure. Untreated PH results in a rapid clinical decline with death frequently occurring within 3 years of diagnosis. Even with treatment, the mean survival time is still less than 4 years.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, Rubin LJ, Tapson VF, Varga J, Harrington RA, Anderson JL, Bates ER, Bridges CR, Eisenberg MJ, Ferrari VA, Grines CL, Hlatky MA, Jacobs AK, Kaul S, Lichtenberg RC, Moliterno DJ, Mukherjee D, Pohost GM, Schofield RS, Shubrooks SJ, Stein JH, Tracy CM, Weitz HH, Wesley DJ. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task force on expert consensus documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119:2250–94.

    Article  PubMed  Google Scholar 

  2. Champion HC, Michelakis ED, Hassoun PM. Comprehensive invasive and noninvasive approach to the right ventricle-pulmonary circulation unit: state of the art and clinical and research implications. Circulation. 2009;120:992–1007.

    Article  PubMed  Google Scholar 

  3. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, Elliott CG, Gaine SP, Gladwin MT, Jing ZC, Krowka MJ, Langleben D, Nakanishi N, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.

    Article  PubMed  Google Scholar 

  4. Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G. 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. 2009;30:2493–537.

    Article  PubMed  Google Scholar 

  5. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, Rubin LJ, Tapson VF, Varga J. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573–619.

    Article  PubMed  Google Scholar 

  6. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis. 2005;16:13–8.

    Article  PubMed  Google Scholar 

  7. Humbert M. The burden of pulmonary hypertension. Eur Respir J. 2007;30:1–2.

    Article  PubMed  CAS  Google Scholar 

  8. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F, Dromer C, Pison C, Reynaud-Gaubert M, Haloun A, Laurent M, Hachulla E, Simonneau G. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023–30.

    Article  PubMed  Google Scholar 

  9. Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J. 2007;30:1103–10.

    Article  PubMed  CAS  Google Scholar 

  10. Le Pavec J, Humbert M. Reference centers for rare respiratory diseases. Presse Med. 2007;36:933–5.

    Article  PubMed  Google Scholar 

  11. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30:104–9.

    Article  PubMed  CAS  Google Scholar 

  12. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216–23.

    PubMed  CAS  Google Scholar 

  13. Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, Barst RJ, Benza RL, Liou TG, Turner M, Giles S, Feldkircher K, Miller DP, McGoon MD. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376–87.

    Article  PubMed  Google Scholar 

  14. Sztrymf B, Yaici A, Girerd B, Humbert M. Genes and pulmonary arterial hypertension. Respiration. 2007;74:123–32.

    Article  PubMed  CAS  Google Scholar 

  15. Runo JR, Vnencak-Jones CL, Prince M, Loyd JE, Wheeler L, Robbins IM, Lane KB, Newman JH, Johnson J, Nichols WC, Phillips JA 3rd. Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003;167:889–94.

    Article  PubMed  Google Scholar 

  16. Hyduk A, Croft JB, Ayala C, Zheng K, Zheng ZJ, Mensah GA. Pulmonary hypertension surveillance–United States, 1980–2002. MMWR Surveill Summ. 2005;54:1–28.

    PubMed  Google Scholar 

  17. Stricker H, Domenighetti G, Popov W, Speich R, Nicod L, Aubert JD, Soler M. Severe pulmonary hypertension: data from the Swiss Registry. Swiss Med Wkly. 2001;131:346–50.

    PubMed  CAS  Google Scholar 

  18. Souza R, Humbert M, Sztrymf B, Jais X, Yaici A, Le Pavec J, Parent F, Herve P, Soubrier F, Sitbon O, Simonneau G. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J. 2008;31:343–8.

    Article  PubMed  CAS  Google Scholar 

  19. D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343–9.

    PubMed  Google Scholar 

  20. Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, McLaughlin V, Hill N, Tapson VF, Robbins IM, Zwicke D, Duncan B, Dixon RA, Frumkin LR. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;169:441–7.

    Article  PubMed  Google Scholar 

  21. Beghetti M, Galie N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;53:733–40.

    Article  PubMed  Google Scholar 

  22. Musewe NN, Poppe D, Smallhorn JF, Hellman J, Whyte H, Smith B, Freedom RM. Doppler echocardiographic measurement of pulmonary artery pressure from ductal Doppler velocities in the newborn. J Am Coll Cardiol. 1990;15:446–56.

    Article  PubMed  CAS  Google Scholar 

  23. Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet. 2003;361:1533–44.

    Article  PubMed  Google Scholar 

  24. Hui-li G. The management of acute pulmonary arterial hypertension. Cardiovasc Ther. 2011;29:153–75.

    Article  PubMed  Google Scholar 

  25. Galie N, Seeger W, Naeije R, Simonneau G, Rubin LJ. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:81S–8S.

    Article  PubMed  Google Scholar 

  26. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:63S–71S.

    Article  PubMed  Google Scholar 

  27. Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:35S–62S.

    Article  PubMed  Google Scholar 

  28. Bush A, Gray H, Denison DM. Diagnosis of pulmonary hypertension from radiographic estimates of pulmonary arterial size. Thorax. 1988;43:127–31.

    Article  PubMed  CAS  Google Scholar 

  29. Sleeper JC, Orgain ES, Mc IH. Primary pulmonary hypertension. Review of clinical features and pathologic physiology with a report of pulmonary hemodynamics derived from repeated catheterization. Circulation. 1962;26:1358–69.

    PubMed  CAS  Google Scholar 

  30. Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, Al-Nahhas A. Ventilation–perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48:680–4.

    Article  PubMed  Google Scholar 

  31. Kume N, Suga K, Uchisako H, Matsui M, Shimizu K, Matsunaga N. Abnormal extrapulmonary accumulation of 99 mTc-MAA during lung perfusion scanning. Ann Nucl Med. 1995;9:179–84.

    Article  PubMed  CAS  Google Scholar 

  32. Krowka MJ, Wiseman GA, Burnett OL, Spivey JR, Therneau T, Porayko MK, Wiesner RH. Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO(2) response to 100% oxygen, and brain uptake after (99m)Tc MAA lung scanning. Chest. 2000;118:615–24.

    Article  PubMed  CAS  Google Scholar 

  33. Habib G, Torbicki A. The role of echocardiography in the diagnosis and management of patients with pulmonary hypertension. Eur Respir Rev. 2010;19:288–99.

    Article  PubMed  CAS  Google Scholar 

  34. McConnell MV, Solomon SD, Rayan ME, Come PC, Goldhaber SZ, Lee RT. Regional right ventricular dysfunction detected by echocardiography in acute pulmonary embolism. Am J Cardiol. 1996;78:469–73.

    Article  PubMed  CAS  Google Scholar 

  35. Sosland RP, Gupta K. Images in cardiovascular medicine: McConnell’s Sign. Circulation. 2008;118:e517–8.

    Article  PubMed  Google Scholar 

  36. Fisher MR, Forfia PR, Chamera E, Housten-Harris T, Champion HC, Girgis RE, Corretti MC, Hassoun PM. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009;179:615–21.

    Article  PubMed  Google Scholar 

  37. Cho EJ, Jiamsripong P, Calleja AM, Alharthi MS, McMahon EM, Khandheria BK, Belohlavek M. Right ventricular free wall circumferential strain reflects graded elevation in acute right ventricular afterload. Am J Physiol Heart Circ Physiol. 2009;296:H413–20.

    Article  PubMed  CAS  Google Scholar 

  38. Kuriyama K, Gamsu G, Stern RG, Cann CE, Herfkens RJ, Brundage BH. CT-determined pulmonary artery diameters in predicting pulmonary hypertension. Invest Radiol. 1984;19:16–22.

    Article  PubMed  CAS  Google Scholar 

  39. Haimovici JB, Trotman-Dickenson B, Halpern EF, Dec GW, Ginns LC, Shepard JA, McLoud TC. Relationship between pulmonary artery diameter at computed tomography and pulmonary artery pressures at right-sided heart catheterization. Massachusetts General Hospital Lung Transplantation Program. Acad Radiol. 1997;4:327–34.

    Article  PubMed  CAS  Google Scholar 

  40. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Medical College of Wisconsin Lung Transplant Group. Chest. 1998;113:1250–6.

    Article  PubMed  CAS  Google Scholar 

  41. Sanal S, Aronow WS, Ravipati G, Maguire GP, Belkin RN, Lehrman SG. Prediction of moderate or severe pulmonary hypertension by main pulmonary artery diameter and main pulmonary artery diameter/ascending aorta diameter in pulmonary embolism. Cardiol Rev. 2006;14:213–4.

    Article  PubMed  Google Scholar 

  42. Devaraj A, Wells AU, Meister MG, Loebinger MR, Wilson R, Hansell DM. Pulmonary hypertension in patients with bronchiectasis: prognostic significance of CT signs. Am J Roentgenol. 2011;196:1300–4.

    Article  Google Scholar 

  43. Edwards PD, Bull RK, Coulden R. CT measurement of main pulmonary artery diameter. Br J Radiol. 1998;71:1018–20.

    PubMed  CAS  Google Scholar 

  44. Beiderlinden M, Kuehl H, Boes T, Peters J. Prevalence of pulmonary hypertension associated with severe acute respiratory distress syndrome: predictive value of computed tomography. Intensive Care Med. 2006;32:852–7.

    Article  PubMed  Google Scholar 

  45. Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imaging. 1999;14:270–8.

    Article  PubMed  CAS  Google Scholar 

  46. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, Simonneau G, Musset D. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am J Roentgenol. 2004;183:65–70.

    Google Scholar 

  47. Swensen SJ, Tashjian JH, Myers JL, Engeler CE, Patz EF, Edwards WD, Douglas WW. Pulmonary venoocclusive disease: CT findings in eight patients. Am J Roentgenol. 1996;167:937–40.

    CAS  Google Scholar 

  48. Dartevelle P, Fadel E, Mussot S, Chapelier A, Herve P, de Perrot M, Cerrina J, Ladurie FL, Lehouerou D, Humbert M, Sitbon O, Simonneau G. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004;23:637–48.

    Article  PubMed  CAS  Google Scholar 

  49. McNeil K, Dunning J. Chronic thromboembolic pulmonary hypertension (CTEPH). Heart. 2007;93:1152–8.

    Article  PubMed  Google Scholar 

  50. Lang IM. Chronic thromboembolic pulmonary hypertension (CTEPH). Dtsch Med Wochenschr. 2008;133(Suppl 6):S206–8.

    Article  PubMed  Google Scholar 

  51. Seyfarth HJ, Halank M, Wilkens H, Schafers HJ, Ewert R, Riedel M, Schuster E, Pankau H, Hammerschmidt S, Wirtz H. Standard PAH therapy improves long term survival in CTEPH patients. Clin Res Cardiol. 2010;99:553–6.

    Article  PubMed  CAS  Google Scholar 

  52. Castaner E, Gallardo X, Ballesteros E, Andreu M, Pallardo Y, Mata JM, Riera L. CT diagnosis of chronic pulmonary thromboembolism. Radiographics. 2009;29:31–50. discussion 50-3.

    Article  PubMed  Google Scholar 

  53. Lin FY, Devereux RB, Roman MJ, Meng J, Jow VM, Simprini L, Jacobs A, Weinsaft JW, Shaw LJ, Berman DS, Callister TQ, Min JK. The right sided great vessels by cardiac multidetector computed tomography: normative reference values among healthy adults free of cardiopulmonary disease, hypertension, and obesity. Acad Radiol. 2009;16:981–7.

    Article  PubMed  Google Scholar 

  54. Oberholzer K, Romaneehsen B, Kunz P, Kramm T, Thelen M, Kreitner KF. Contrast-enhanced 3D MR angiography of the pulmonary arteries with integrated parallel acquisition technique (iPAT) in patients with chronic-thromboembolic pulmonary hypertension CTEPH-sagittal or coronal acquisition? Rofo. 2004;176:605–9.

    Article  PubMed  CAS  Google Scholar 

  55. Ghio S, Gavazzi A, Campana C, Inserra C, Klersy C, Sebastiani R, Arbustini E, Recusani F, Tavazzi L. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol. 2001;37:183–8.

    Article  PubMed  CAS  Google Scholar 

  56. Sanz J, Dellegrottaglie S, Kariisa M, Sulica R, Poon M, O’Donnell TP, Mehta D, Fuster V, Rajagopalan S. Prevalence and correlates of septal delayed contrast enhancement in patients with pulmonary hypertension. Am J Cardiol. 2007;100:731–5.

    Article  PubMed  Google Scholar 

  57. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004;363:1461–8.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, Madison, WI, 53792, USA

    Mark L. Schiebler & Christopher J. François

  2. Department of Pulmonary and Critical Care Medicine, University of Wisconsin School of Medicine and Public Health, 5252 MFCB, 1685 Highland Avenue, Madison, WI, 53705-2281, USA

    James Runo

  3. Diagnostic Radiology, E3/366 Clinical Science Center, University of Wisconsin–Madison, 600 Highland Avenue, Madison, WI, 53792-3252, USA

    Leif Jensen

Authors
  1. Mark L. Schiebler
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James Runo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Leif Jensen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christopher J. François
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mark L. Schiebler .

Editor information

Editors and Affiliations

  1. , Cardiothoracic Radiology, University of Wisconsin Hospital and Cli, 600 Highland Ave., Madison, 53792, USA

    Jeffrey P. Kanne

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Humana Press, a part of Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Schiebler, M.L., Runo, J., Jensen, L., François, C.J. (2012). Imaging of Pulmonary Hypertension. In: Kanne, J. (eds) Clinically Oriented Pulmonary Imaging. Respiratory Medicine. Humana Press. https://doi.org/10.1007/978-1-61779-542-8_9

Download citation

  • DOI: https://doi.org/10.1007/978-1-61779-542-8_9

  • Published:

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-61779-541-1

  • Online ISBN: 978-1-61779-542-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation