Pulmonary Hypertension: How the Radiologist Can Help

Published Online:https://doi.org/10.1148/rg.321105232

The role of radiologists in diagnosing pulmonary hypertension on the basis of the Dana Point classification system and the emerging role of cardiac MR imaging in the evaluation and follow-up of patients with pulmonary hypertension is discussed.

Pulmonary hypertension is defined as an abnormal elevation of pressure in pulmonary circulation, with a mean pulmonary arterial pressure higher than 25 mmHg, regardless of the underlying mechanism. The clinical classification system for pulmonary hypertension was updated at the fourth World Symposium on Pulmonary Hypertension in Dana Point, California, in 2008. In patients with suspected pulmonary hypertension, the diagnostic approach includes four stages: suspicion, detection, classification, and functional evaluation. It is crucial to understand the advantages and disadvantages of the different imaging tools available for the diagnostic work-up and follow-up of patients with pulmonary hypertension. Many conditions that cause pulmonary hypertension have suggestive findings at multidetector computed tomography or magnetic resonance imaging; some causes may be surgically treatable, whereas others may demonstrate adverse reactions to vasodilator therapies used during the course of treatment. Therefore, the radiologist plays an important role in evaluating patients with this disease. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.321105232/-/DC1.

© RSNA, 2012

References

  • 1 Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997;336(2):111–117. Crossref, MedlineGoogle Scholar
  • 2 Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003;361(9368):1533–1544. Crossref, MedlineGoogle Scholar
  • 3 Badesch DB, Champion HC, Sanchez MAet al.. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(1 suppl): S55–S66. Crossref, MedlineGoogle Scholar
  • 4 Simonneau G, Robbins IM, Beghetti Met al.. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54(1 suppl):S43–S54. Crossref, MedlineGoogle Scholar
  • 5 Weir EKME, Archer SL, Rubin LJ. Pulmonary hypertension. 2nd ed. Philadelphia, Pa: Churchill Livingstone, 2000; 1856–1884. Google Scholar
  • 6 Rich S, Rubin LJ, Abenhail Let al.. Executive summary from the World Symposium on Primary Pulmonary Hypertension (Evian, France, September 6-10, 1998) [updated June 2002]. Geneva, Switzerland: World Health Organization, 1998. Google Scholar
  • 7 Smiley I, Rich S, McLaughlin VV, eds. Clinics in chest medicine. Philadelphia, Pa: Saunders, 2001. Google Scholar
  • 8 Moraes D, Loscalzo J. Pulmonary hypertension: newer concepts in diagnosis and management. Clin Cardiol 1997;20(8):676–682. Crossref, MedlineGoogle Scholar
  • 9 Dell'Italia LJ, Santamore WP. Can indices of left ventricular function be applied to the right ventricle? Prog Cardiovasc Dis 1998;40(4):309–324. Crossref, MedlineGoogle Scholar
  • 10 McGoon M, Gutterman D, Steen Vet al.. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126(1 suppl): 14S–34S. Crossref, MedlineGoogle Scholar
  • 11 Nauser TD, Stites SW. Diagnosis and treatment of pulmonary hypertension. Am Fam Physician 2001; 63(9):1789–1798. MedlineGoogle Scholar
  • 12 Bristow MR, Zisman LS, Lowes BDet al.. The pressure-overloaded right ventricle in pulmonary hypertension. Chest 1998;114(1 suppl):101S–106S. Crossref, MedlineGoogle Scholar
  • 13 D'Alonzo GE, Barst RJ, Ayres SMet al.. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 1991;115(5):343–349. Crossref, MedlineGoogle Scholar
  • 14 McLaughlin VV, Sitbon O, Badesch DBet al.. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25(2):244–249. Crossref, MedlineGoogle Scholar
  • 15 Barst RJ, McGoon M, Torbicki Aet al.. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(12 suppl S):40S–47S. Crossref, MedlineGoogle Scholar
  • 16 Trow TK, McArdle JR. Diagnosis of pulmonary arterial hypertension. Clin Chest Med 2007;28(1): 59–73, viii. Crossref, MedlineGoogle Scholar
  • 17 Ulett KB, Marwick TH. Incorporation of pulmonary vascular resistance measurement into standard echocardiography: implications for assessment of pulmonary hypertension. Echocardiography 2007; 24(10):1020–1022. Crossref, MedlineGoogle Scholar
  • 18 Benza R, Biederman R, Murali S, Gupta H. Role of cardiac magnetic resonance imaging in the management of patients with pulmonary arterial hypertension. J Am Coll Cardiol 2008;52(21):1683–1692. Crossref, MedlineGoogle Scholar
  • 19 Biederman RW. Cardiovascular magnetic resonance imaging as applied to patients with pulmonary arterial hypertension. Int J Clin Pract Suppl 2009; (162):20–35. Crossref, MedlineGoogle Scholar
  • 20 McLure LE, Peacock AJ. Imaging of the heart in pulmonary hypertension. Int J Clin Pract Suppl 2007;(156):15–26. Crossref, MedlineGoogle Scholar
  • 21 Ley S, Kreitner KF, Fink C, Heussel CP, Borst MM, Kauczor HU. Assessment of pulmonary hypertension by CT and MR imaging. Eur Radiol 2004;14(3):359–368. Crossref, MedlineGoogle Scholar
  • 22 Haddad F, Hunt SA, Rosenthal DN, Murphy DJ. Right ventricular function in cardiovascular disease: part I—anatomy, physiology, aging, and functional assessment of the right ventricle. Circulation 2008; 117(11):1436–1448. Crossref, MedlineGoogle Scholar
  • 23 Grothues F, Smith GC, Moon JCet al.. Comparison of interstudy reproducibility of cardiovascular magnetic resonance with two-dimensional echocardiography in normal subjects and in patients with heart failure or left ventricular hypertrophy. Am J Cardiol 2002;90(1):29–34. Crossref, MedlineGoogle Scholar
  • 24 Fleischner FG. Pulmonary embolism. Clin Radiol 1962;13:169–182. Crossref, MedlineGoogle Scholar
  • 25 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(5): 1250–1256. Crossref, MedlineGoogle Scholar
  • 26 Edwards PD, Bull RK, Coulden R. CT measurement of main pulmonary artery diameter. Br J Radiol 1998;71(850):1018–1020. Crossref, MedlineGoogle Scholar
  • 27 Devaraj A, Hansell DM. Computed tomography signs of pulmonary hypertension: old and new observations. Clin Radiol 2009;64(8):751–760. Crossref, MedlineGoogle Scholar
  • 28 Ng CS, Wells AU, Padley SPA. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imaging 1999;14(4):270–278. Crossref, MedlineGoogle Scholar
  • 29 Devaraj A, Wells AU, Meister MG, Corte TJ, Wort SJ, Hansell DM. Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination. Radiology 2010;254(2): 609–616. LinkGoogle Scholar
  • 30 Revel MP, Faivre JB, Remy-Jardin M, Delannoy-Deken V, Duhamel A, Remy J. Pulmonary hypertension: ECG-gated 64-section CT angiographic evaluation of new functional parameters as diagnostic criteria. Radiology 2009;250(2):558–566. LinkGoogle Scholar
  • 31 Reid JH, Murchison JT. Acute right ventricular dilatation: a new helical CT sign of massive pulmonary embolism. Clin Radiol 1998;53(9):694–698. Crossref, MedlineGoogle Scholar
  • 32 van der Meer RW, Pattynama PM, van Strijen MJet al.. Right ventricular dysfunction and pulmonary obstruction index at helical CT: prediction of clinical outcome during 3-month follow-up in patients with acute pulmonary embolism. Radiology 2005; 235(3):798–803. LinkGoogle Scholar
  • 33 Groves AM, Win T, Charman SC, Wisbey C, Pepke-Zaba J, Coulden RA. Semi-quantitative assessment of tricuspid regurgitation on contrast-enhanced multidetector CT. Clin Radiol 2004;59(8):715–719. Crossref, MedlineGoogle Scholar
  • 34 Baque-Juston MC, Wells AU, Hansell DM. Pericardial thickening or effusion in patients with pulmonary artery hypertension: a CT study. AJR Am J Roentgenol 1999;172(2):361–364. Crossref, MedlineGoogle Scholar
  • 35 Horton MR, Tuder RM. Primary pulmonary arterial hypertension presenting as diffuse micronodules on CT. Crit Rev Computed Tomogr 2004;45(5-6):335–341. Crossref, MedlineGoogle Scholar
  • 36 Nolan RL, McAdams HP, Sporn TA, Roggli VL, Tapson VF, Goodman PC. Pulmonary cholesterol granulomas in patients with pulmonary artery hypertension: chest radiographic and CT findings. AJR Am J Roentgenol 1999;172(5):1317–1319. Crossref, MedlineGoogle Scholar
  • 37 Resten A, Maître S, Humbert Met al.. Pulmonary arterial hypertension: thin-section CT predictors of epoprostenol therapy failure. Radiology 2002;222(3):782–788. LinkGoogle Scholar
  • 38 Hansell DM. Small-vessel diseases of the lung: CT-pathologic correlates. Radiology 2002;225(3): 639–653. LinkGoogle Scholar
  • 39 Sheehan R, Perloff JK, Fishbein MC, Gjertson D, Aberle DR. Pulmonary neovascularity: a distinctive radiographic finding in Eisenmenger syndrome. Circulation 2005;112(18):2778–2785. Crossref, MedlineGoogle Scholar
  • 40 Lorenz CH, Walker ES, Morgan VL, Klein SS, Graham TP. Normal human right and left ventricular mass, systolic function, and gender differences by cine magnetic resonance imaging. J Cardiovasc Magn Reson 1999;1(1):7–21. Crossref, MedlineGoogle Scholar
  • 41 Frank H, Globits S, Glogar D, Neuhold A, Kneussl M, Mlczoch J. Detection and quantification of pulmonary artery hypertension with MR imaging: results in 23 patients. AJR Am J Roentgenol 1993;161(1):27–31. Crossref, MedlineGoogle Scholar
  • 42 Pennell DJ, Sechtem UP, Higgins CBet al.. Clinical indications for cardiovascular magnetic resonance (CMR): consensus panel report. Eur Heart J 2004; 25(21):1940–1965. Crossref, MedlineGoogle Scholar
  • 43 Marcu CB, Beek AM, Van Rossum AC. Cardiovascular magnetic resonance imaging for the assessment of right heart involvement in cardiac and pulmonary disease. Heart Lung Circ 2006;15(6):362–370. Crossref, MedlineGoogle Scholar
  • 44 Katz J, Whang J, Boxt LM, Barst RJ. Estimation of right ventricular mass in normal subjects and in patients with primary pulmonary hypertension by nuclear magnetic resonance imaging. J Am Coll Cardiol 1993;21(6):1475–1481. Crossref, MedlineGoogle Scholar
  • 45 Hoeper MM, Tongers J, Leppert A, Baus S, Maier R, Lotz J. Evaluation of right ventricular performance with a right ventricular ejection fraction thermodilution catheter and MRI in patients with pulmonary hypertension. Chest 2001;120(2):502–507. Crossref, MedlineGoogle Scholar
  • 46 van Wolferen SA, Marcus JT, Boonstra Aet al.. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007;28(10):1250–1257. Crossref, MedlineGoogle Scholar
  • 47 Saba TS, Foster J, Cockburn M, Cowan M, Peacock AJ. Ventricular mass index using magnetic resonance imaging accurately estimates pulmonary artery pressure. Eur Respir J 2002;20(6):1519–1524. Crossref, MedlineGoogle Scholar
  • 48 Roeleveld RJ, Marcus JT, Faes TJet al.. Interventricular septal configuration at MR imaging and pulmonary arterial pressure in pulmonary hypertension. Radiology 2005;234(3):710–717. LinkGoogle Scholar
  • 49 King ME, Braun H, Goldblatt A, Liberthson R, Weyman AE. Interventricular septal configuration as a predictor of right ventricular systolic hypertension in children: a cross-sectional echocardiographic study. Circulation 1983;68(1):68–75. Crossref, MedlineGoogle Scholar
  • 50 Dellegrottaglie S, Sanz J, Poon Met al.. Pulmonary hypertension: accuracy of detection with left ventricular septal-to–free wall curvature ratio measured at cardiac MR. Radiology 2007;243(1):63–69. LinkGoogle Scholar
  • 51 Alunni JP, Degano B, Arnaud Cet al.. Cardiac MRI in pulmonary artery hypertension: correlations between morphological and functional parameters and invasive measurements. Eur Radiol 2010;20(5): 1149–1159. Crossref, MedlineGoogle Scholar
  • 52 Gan CT, Lankhaar JW, Marcus JTet al.. Impaired left ventricular filling due to right-to-left ventricular interaction in patients with pulmonary arterial hypertension. Am J Physiol Heart Circ Physiol 2006; 290(4):H1528–H1533. Crossref, MedlineGoogle Scholar
  • 53 Sanz J, Kuschnir P, Rius Tet al.. Pulmonary arterial hypertension: noninvasive detection with phase-contrast MR imaging. Radiology 2007;243(1):70–79. LinkGoogle Scholar
  • 54 Blyth KG, Groenning BA, Martin TNet al.. Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertension. Eur Heart J 2005;26(19):1993–1999. Crossref, MedlineGoogle Scholar
  • 55 McCann GP, Gan CT, Beek AM, Niessen HW, Vonk Noordegraaf A, van Rossum AC. Extent of MRI delayed enhancement of myocardial mass is related to right ventricular dysfunction in pulmonary artery hypertension. AJR Am J Roentgenol 2007; 188(2):349–355. Crossref, MedlineGoogle Scholar
  • 56 Sanz J, Dellegrottaglie S, Kariisa Met al.. Prevalence and correlates of septal delayed contrast enhancement in patients with pulmonary hypertension. Am J Cardiol 2007;100(4):731–735. Crossref, MedlineGoogle Scholar
  • 57 Jardim C, Rochitte CE, Humbert Met al.. Pulmonary artery distensibility in pulmonary arterial hypertension: an MRI pilot study. Eur Respir J 2007; 29(3):476–481. Crossref, MedlineGoogle Scholar
  • 58 Perloff JK, Hart EM, Greaves SM, Miner PD, Child JS. Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 2003;92(2):182–187. Crossref, MedlineGoogle Scholar
  • 59 Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. Am J Surg Pathol 2006;30(7):850–857. Crossref, MedlineGoogle Scholar
  • 60 Trobaugh-Lotrario AD, Greffe B, Deterding R, Deutsch G, Quinones R. Pulmonary veno-occlusive disease after autologous bone marrow transplant in a child with stage IV neuroblastoma: case report and literature review. J Pediatr Hematol Oncol 2003;25(5):405–409. Crossref, MedlineGoogle Scholar
  • 61 Montani D, Achouh L, Dorfmüller Pet al.. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore) 2008;87(4):220–233. Crossref, MedlineGoogle Scholar
  • 62 Hoey ET, Gopalan D, Agrawal SK, Screaton NJ. Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT. Eur Radiol 2009; 19(11):2557–2568. Crossref, MedlineGoogle Scholar
  • 63 Hoeper MM, Barberà JA, Channick RNet al.. Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension. J Am Coll Cardiol 2009;54(1 suppl):S85–S96. Crossref, MedlineGoogle Scholar
  • 64 Ryu JH, Krowka MJ, Pellikka PA, Swanson KL, McGoon MD. Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc 2007; 82(3):342–350. Crossref, MedlineGoogle Scholar
  • 65 Thabut G, Dauriat G, Stern JBet al.. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest 2005;127(5):1531–1536. Crossref, MedlineGoogle Scholar
  • 66 Cottin V, Nunes H, Brillet PYet al.. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005;26(4):586–593. Crossref, MedlineGoogle Scholar
  • 67 Grubstein A, Bendayan D, Schactman I, Cohen M, Shitrit D, Kramer MR. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the literature. Respir Med 2005;99(8):948–954. Crossref, MedlineGoogle Scholar
  • 68 Zisman DA, Karlamangla AS, Ross DJet al.. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 2007;132(3): 773–779. Crossref, MedlineGoogle Scholar
  • 69 Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 2008;249(3):1042–1049. LinkGoogle Scholar
  • 70 Castañer E, Gallardo X, Ballesteros Eet al.. CT diagnosis of chronic pulmonary thromboembolism. RadioGraphics 2009;29(1):31–50; discussion 50–53. LinkGoogle Scholar
  • 71 Kreitner KF, Ley S, Kauczor HUet al.. Chronic thromboembolic pulmonary hypertension: pre- and postoperative assessment with breath-hold MR imaging techniques. Radiology 2004;232(2):535–543. LinkGoogle Scholar
  • 72 Reesink HJ, Marcus JT, Tulevskiet al.. Reverse right ventricular remodeling after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: utility of magnetic resonance imaging to demonstrate restoration of the right ventricle. J Thorac Cardiovasc Surg 2007;133(1):58–64. Crossref, MedlineGoogle Scholar
  • 73 Lederman RJ. Cardiovascular interventional magnetic resonance imaging. Circulation 2005;112(19): 3009–3017. Crossref, MedlineGoogle Scholar
  • 74 Yi CA, Lee KS, Choe YH, Han D, Kwon OJ, Kim S. Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease. J Comput Assist Tomogr 2004;28(1): 34–39. Crossref, MedlineGoogle Scholar
  • 75 Chong S, Kim TS, Kim BT, Cho EY, Kim J. Pulmonary artery sarcoma mimicking pulmonary thromboembolism: integrated FDG PET/CT. AJR Am J Roentgenol 2007;188(6):1691–1693. Crossref, MedlineGoogle Scholar
  • 76 Matsunaga N, Hayashi K, Sakamoto I, Ogawa Y, Matsumoto T. Takayasu arteritis: protean radiologicmanifestations and diagnosis. RadioGraphics 1997; 17(3):579–594. LinkGoogle Scholar

Article History

Received: Nov 29 2010
Revision requested: Mar 29 2011
Revision received: July 30 2011
Accepted: Aug 15 2011
Published online: Dec 30 2011
Published in print: Jan 2012