Imaging modalities for the diagnosis of pulmonary hypertension in systemic sclerosis

Nature Reviews Rheumatology

Volumn 8, Issue 4, 2012, Pages 203-213

  Dimitroulas, Theodoros ^a   Mavrogeni, Sophie ^b   Kitas, George D ^c  

^a RUSSELLS HALL HOSPITAL   (United Kingdom)

^b ONASSIS CARDIAC SURGERY CENTER   (Greece)

^c UNIVERSITY OF MANCHESTER   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CARDIAC IMAGING; CARDIOVASCULAR MAGNETIC RESONANCE; DIAGNOSTIC IMAGING; DOPPLER ECHOCARDIOGRAPHY; DOPPLER STRAIN IMAGING; EARLY DIAGNOSIS; ECHOCARDIOGRAPHY; HEART FAILURE; HEART MUSCLE CONTRACTILITY; HEART MUSCLE FIBROSIS; HEART RIGHT VENTRICLE FUNCTION; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HUMAN; LUNG SCINTISCANNING; NON INVASIVE MEASUREMENT; OVERALL SURVIVAL; PATIENT IDENTIFICATION; PRIORITY JOURNAL; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR; SINGLE PHOTON EMISSION COMPUTER TOMOGRAPHY; SYSTEMIC SCLEROSIS; THORAX RADIOGRAPHY; TISSUE DOPPLER IMAGING; TREATMENT FAILURE;

ECHOCARDIOGRAPHY; HUMANS; HYPERTENSION, PULMONARY; MAGNETIC RESONANCE IMAGING; SCLERODERMA, SYSTEMIC; TOMOGRAPHY, EMISSION-COMPUTED, SINGLE-PHOTON; TOMOGRAPHY, X-RAY COMPUTED; VENTILATION-PERFUSION RATIO;

EID: 84859440453     PISSN: 17594790     EISSN: 17594804     Source Type: Journal    
DOI: 10.1038/nrrheum.2012.2     Document Type: Review

References (98)

1. Steen, V. D. & Medsger, T. A. Changes in causes of death in systemic sclerosis, 1972-2002. Ann. Rheum. Dis. 66, 940-944 (2007).
2. Tyndall, A. J. et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann. Rheum. Dis. 69, 1809-1815 (2010).
3. Denton, C. P. & Black, C. M. Pulmonary hypertension in systemic sclerosis. Rheum. Dis. Clin. North Am. 29, 335-349 (2003).
4. Galiè, N. et al. 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 (2009).
5. Schachna, L. & Wigley, F. M. Targeting mediators of vascular injury in scleroderma. Curr. Opin. Rheumatol. 14, 686-693 (2002).
6. Overbeek, M. J. et al. Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. Eur. Respir. J. 34, 371-379 (2009).
7. Dorfmüller, P. et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum. Pathol. 38, 893-902 (2007).
8. Chatterjee, S. Pulmonary hypertension in systemic sclerosis. Semin. Arthritis Rheum. 41, 19-37 (2011).
9. Fernandes, F. et al. Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J. Card. Fail. 9, 311-317 (2003).
10. Kahan, A. et al. Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis. N. Engl. J. Med. 314, 1397-1402 (1986).
11. Champion, H. C. The heart in scleroderma. Rheum. Dis. Clin. North Am. 34, 181-190 (2008).
12. Fisher, M. R. et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 54, 3043-3050 (2006).
13. Dorfmüller, P., Montani, D. & Humbert, M. Beyond arterial remodelling: pulmonary venous and cardiac involvement in patients with systemic sclerosis-associated pulmonary arterial hypertension. Eur. Respir. J. 35, 6-8 (2010).
14. Hachulla, E. & Denton, C. P. Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management. Eur. Respir. Rev. 19, 314-320 (2010).
15. Mathai, S. C. & Hassoun, P. M. Therapy for pulmonary arterial hypertension associated with systemic sclerosis. Curr. Opin. Rheumatol. 21, 642-648 (2009).
16. Clements, P. J. et al. on behalf of the Pulmonary Arterial Hypertension Quality Enhancement Research Initiative (PAH-QuERI) Investigators. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated, PAH. Ann. Rheum. Dis.71, 249-252 (2011).
17. Avouac, J., Wipff, J., Kahan, A. & Allanore, Y. Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann. Rheum. Dis. 67, 808-814 (2008).
18. Mukerjee, D. et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann. Rheum. Dis. 62, 1088-1093 (2003).
19. Phung, S. et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Intern. Med. J. 39, 682-691 (2009).
20. Hachulla, E. et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 52, 3792-3800 (2005).
21. Avouac, J. et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J. Rheumatol. 37, 2290-2298 (2010).
22. Hoeper, M. M. et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J. Am. Coll. Cardiol. 48, 2546-2552 (2006).
23. Keysser, G., Schwerdt, C. & Taege, C. Right-heart failure after right heart catheterization in a patient with scleroderma and suspected pulmonary hypertension. Rheumatol. Int. 28, 1269-1271 (2008).
24. Dimitroulas, T., Giannakoulas, G., Karvounis, H., Settas, L. & Kitas, G. D. Biomarkers in systemic sclerosis-related pulmonary arterial hypertension. Curr. Vasc. Pharmacol. 9, 213-219 (2011).
25. Kepez, A. et al. Detection of subclinical cardiac involvement in systemic sclerosis by echocardiographic strain imaging. Echocardiography 25, 191-197 (2008).
26. D'Andrea, A. et al. Early impairment of myocardial function in systemic sclerosis: noninvasive assessment by Doppler myocardial and strain rate imaging. Eur. J. Echocardiogr. 6, 407-418 (2005).
27. Bezante, G. P. et al. Cardiac magnetic resonance imaging detects subclinical right ventricular impairment in systemic sclerosis. J. Rheumatol. 34, 2431-2437 (2007).
28. Masuyama, T. et al. Continuous-wave Doppler echocardiographic detection of pulmonary regurgitation and its application to noninvasive estimation of pulmonary artery pressure. Circulation 74, 484-492 (1986).
29. Ghofrani, H. A., Wilkins, M. W. & Rich, S. Uncertainties in the diagnosis and treatment of pulmonary arterial hypertension. Circulation 118, 1195-1201 (2008).
30. Arcasoy, S. M. et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am. J. Respir. Crit. Care Med. 167, 735-740 (2003).
31. Fisher, M. R. et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am. J. Respir. Crit. Care Med. 179, 615-621 (2009).
32. Sánchez-Román, J. et al. Screening for PAH in patients with systemic sclerosis: focus on Doppler echocardiography. Rheumatology (Oxford) 47 (Suppl. 5), v33-v35 (2008).
33. Denton, C. P., Cailes, J. B. & Phillips, G. D. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br. J. Rheumatol. 36, 239-243 (1997).
34. Mukerjee, D. et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology 43, 461-466 (2004).
35. de Azevedo, A. B., Sampaio-Barros, P. D., Torres, R. M. & Moreira, C. Prevalence of pulmonary hypertension in systemic sclerosis. Clin. Exp. Rheumatol. 23, 447-454 (2005).
36. Pope, J. E. et al. Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J. Rheumatol. 32, 1273-1278 (2005).
37. Murata, I. et al. Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. A Doppler echocardiographic study of 135 Japanese patients. Chest 111, 36-43 (1997).
38. Dimitroulas, T. et al. Neurohormonal activation in patients with systemic sclerosis-related pulmonary arterial hypertension. Int. J. Cardiol. 121, 135-137 (2007).
39. Hachulla, E. et al. Itinér AIR-Sclérodermie Study Group. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum. 60, 1831-1839 (2009).
40. Kowal-Bielecka, O. et al. Echocardiography as an outcome measure in scleroderma-related pulmonary arterial hypertension: a systematic literature analysis by the EPOSS group. J. Rheumatol. 37, 105-115 (2010).
41. Tei, C. et al. Doppler echocardiographic index for assessment of global right ventricular function. J. Am. Soc. Echocardiogr. 9, 838-847 (1996).
42. Vonk, M. C. et al. Right ventricle Tei-index: a tool to increase the accuracy of non-invasive detection of pulmonary arterial hypertension in connective tissue diseases. Eur. J. Echocardiogr. 8, 317-321 (2007).
43. Dimitroulas, T. et al. Left atrial volume and N-terminal pro-B type natriuretic peptide are associated with elevated pulmonary artery pressure in patients with systemic sclerosis. Clin. Rheumatol. 29, 957-964 (2010).
44. Forfia, P. R. et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 174, 1034-1041 (2006).
45. Mathai, S. C. et al. Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension. J. Rheumatol. 38, 2410-2418 (2011).
46. Serra, W. et al. Echocardiography may help detect pulmonary vasculopathy in the early stages of pulmonary artery hypertension associated with systemic sclerosis. Cardiovasc. Ultrasound. 8, 25 (2010).
47. Lee, C. Y. et al. Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion. Echocardiography 24, 118-125 (2007).
48. Boueiz, A., Mathai, S. C., Hummers, L. K. & Hassoun, P. M. Cardiac complications of systemic sclerosis: recent progress in diagnosis. Curr. Opin. Rheumatol. 22, 696-703 (2010).
49. de Groote, P. et al. for the ItinerAIR-Scleroderma Investigators. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann. Rheum. Dis. 67, 31-36 (2008).
50. Mele, D. et al. Abnormalities of left ventricular function in asymptomatic patients with systemic sclerosis using Doppler measures of myocardial strain. J. Am. Soc. Echocardiogr. 21, 1257-1264 (2008).
51. Allanore, Y. et al. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patients. Ann. Rheum. Dis. 69, 218-221 (2010).
52. Poanta, L., Dadu, R., Tiboc, C., Rednic, S. & Dumitrascu, D. Systolic and diastolic function in patients with systemic sclerosis. Eur. J. Intern. Med. 20, 378-382 (2009).
53. Dimitroulas, T. et al. Early detection of cardiac involvement in systemic sclerosis assessed by tissue-Doppler echocardiography: relationship with neurohormonal activation and endothelial dysfunction. J. Rheumatol. 37, 993-999 (2010).
54. Meune, C. et al. Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients. Arthritis Rheum. 58, 1803-1809 (2008).
55. Hsiao, S. H., Lee, C. Y., Chang, S. M., Lin, S. K. & Liu, C. P. Right heart function in scleroderma: insights from myocardial Doppler tissue imaging. J. Am. Soc. Echocardiogr. 19, 507-514 (2006).
56. Matias C, et al. Speckle-tracking-derived strain and strain-rate analysis: a technique for the evaluation of early alterations in right ventricle systolic function in patients with systemic sclerosis and normal pulmonary artery pressure. J. Cardiovasc. Med. (Hagerstown) 10, 129-134 (2009).
57. Schattke, S. et al. Early right ventricular systolic dysfunction in patients with systemic sclerosis without pulmonary hypertension: a Doppler tissue and speckle tracking echocardiography study. Cardiovasc. Ultrasound 8, 3 (2010).
58. Ramagopalan, S. V., Wotton, C. J., Handel, A. E., Yeates, D. & Goldacre, M. J. Risk of venous thromboembolism in people admitted to hospital with selected immune-mediated diseases: record-linkage study. BMC Med. 9, 1 (2011).
59. Montani, D. et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore) 87, 220-233 (2008).
60. Wells, A. U. High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford) 47 (Suppl. 5), v59-v61 (2008).
61. Strollo, D. & Goldin, J. Imaging lung disease in systemic sclerosis. Curr. Rheumatol Rep. 12, 156-161 (2010).
62. El-Shafie, M. M., Salem, S. S. & Moghazi, A. A. Left ventricular myocardial ischemia in collagen disease associated with pulmonary hypertension: an evaluation by rest-stress gated SPECT and coronary angiography. Nucl. Med. Commun. 32, 641-648 (2011).
63. Hoeper, M. M. et al. Evaluation of right ventricular performance with a right ventricular ejection fraction thermodilution catheter and MRI in patients with pulmonary hypertension. Chest 120, 502-507 (2001).
64. Roeleveld, R. J. et al. Effects of epoprostenol on right ventricular hypertrophy and dilatation in pulmonary hypertension. Chest 125, 572-579 (2004).
65. Vonk-Noordegraaf, A. et al. Early changes of cardiac structure and function in COPD patients with mild hypoxemia. Chest 127, 1898-1903 (2005).
66. Vonk Noordegraaf, A. et al. The effect of right ventricular hypertrophy on left ventricular ejection fraction in pulmonary emphysema. Chest 112, 640-645 (1997).
67. van Wolferen, S. A. et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur. Heart J. 28, 1250-1257 (2007).
68. Hachulla AL et al. Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann. Rheum. Dis. 68, 1878-1884 (2009).
69. Katz, J. et al. Estimation of right ventricular mass in normal subjects and in patients with primary pulmonary hypertension by nuclear magnetic resonance imaging. J. Am. Coll. Cardiol. 21, 1475-1481 (1993).
70. Grothues, F. et al. Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular magnetic resonance. Am. Heart J. 147, 218-223 (2004).
71. Semelka, R. C. et al. Interstudy reproducibility of dimensional and functional measurements between cine magnetic resonance studies in the morphologically abnormal left ventricle. Am. Heart J. 119, 1367-1373 (1990).
72. Hagger, D. et al. Ventricular mass index correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis-associated pulmonary arterial hypertension. Rheumatology (Oxford) 48, 1137-1142 (2009).
73. Saba, T. S., Foster, J., Cockburn, M., Cowan, M. & Peacock, A. J. Ventricular mass index using magnetic resonance imaging accurately estimates pulmonary artery pressure. Eur. Respir. J. 20, 1519-1524 (2002).
74. Roeleveld, R. J. et al. A comparison of noninvasive MRI-based methods of estimating pulmonary artery pressure in pulmonary hypertension. J. Magn. Reson. Imaging 22, 67-72 (2005).
75. King, M. E., Braun, H., Goldblatt, A., Liberthson, R. & Weyman, A. E. Interventricular septal configuration as a predictor of right ventricular systolic hypertension in children: a cross-sectional echocardiographic study. Circulation 68, 68-75 (1983).
76. Mavrogeni, S. et al. Myocardial inflammation in autoimmune diseases: investigation by cardiovascular magnetic resonance and endomyocardial biopsy. Inflamm. Allergy Drug Targets 8, 390-397 (2009).
77. Nassenstein, K. et al. Detection of myocardial fibrosis in systemic sclerosis by contrast-enhanced magnetic resonance imaging. Rofo. 180, 1054-1060 (2008).
78. Silva, C. et al. Myocardial late gadolinium enhancement in specific cardiomyopathies by cardiovascular magnetic resonance: a preliminary experience. J. Cardiovasc. Med. (Hagerstown) 8, 1076-1079 (2007).
79. Tzelepis, G. E. et al. Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum. 56, 3827-3836 (2007).
80. Kuehne, T. et al. Magnetic resonance imaging analysis of right ventricular pressure - volume loops: in vivo validation and clinical application in patients with pulmonary hypertension. Circulation 110, 2010-2016 (2004).
81. McCann, G. P., Beek, A. M., Vonk-Noordegraaf, A. & van Rossum, A. C. Delayed Contrast- Enhanced Magnetic Resonance Imaging in Pulmonary Arterial Hypertension. Circulation 112, e268 (2005).
82. McCann, G. P. et al. Extent of MRI delayed enhancement of myocardial mass is related to right ventricular dysfunction in pulmonary artery hypertension. AJR Am. J. Roentgenol. 188, 349-355 (2007).
83. Bogren, H. G. et al. Pulmonary artery distensibility and blood flow patterns: a magnetic resonance study of normal subjects and of patients with pulmonary arterial hypertension. Am. Heart J. 118, 990-999 (1989).
84. Kondo, C. et al. Pulmonary hypertension: pulmonary flow quantification and flow profile analysis with velocity-encoded cine MR imaging. Radiology 183, 751-758 (1992).
85. Ley, S. et al. Assessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRI. Eur. Radiol. 15, 1575-1580 (2005).
86. Jardim, C. et al. Pulmonary artery distensibility in pulmonary arterial hypertension: a MRI pilot study. Eur. Respir. J. 29, 476-481 (2007).
87. Gan, C. T. et al. Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary arterial hypertension. Chest 132, 1906-1912 (2007).
88. Kang, K. W. et al. Cardiac magnetic resonance imaging-derived pulmonary artery distensibility index correlates with pulmonary artery stiffness and predicts functional capacity in patients with pulmonary arterial hypertension. Circ. J. 75, 2244-2251 (2011).
89. McLure, L. E. & Peacock, A. J. Cardiac magnetic resonance imaging for the assessment of the heart and pulmonary circulation in pulmonary hypertension. Eur. Respir. J. 33, 1454-1466 (2009).
90. Kobayashi, H. et al. Cardiac magnetic resonance imaging with pharmacological stress perfusion and delayed enhancement in asymptomatic patients with systemic sclerosis. J. Rheumatol. 36, 106-112 (2009).
91. Vogel-Claussen J. et al. Right and left ventricular myocardial perfusion reserves correlate with right ventricular function and pulmonary hemodynamics in patients with pulmonary arterial hypertension. Radiology 258, 119-127 (2011).
92. Michelakis, E. D. et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 108, 2066-2069 (2003).
93. Gan, C. T. et al. Right ventricular diastolic dysfunction and the acute effects of sildenafil in pulmonary hypertension patients. Chest 132, 11-17 (2007).
94. Chin, K. M. et al. Changes in right ventricular structure and function assessed using cardiac magnetic resonance imaging in bosentan-treated patients with pulmonary arterial hypertension. Am. J. Cardiol. 101, 1669-1672 (2008).
95. Allanore, Y. et al. Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and Tissue- Doppler echography study. J. Rheumatol. 33, 2464-2469 (2006).
96. Champion, H. C., Michelakis, E. D. & Hassoun, P. M. Comprehensive invasive and noninvasive approach to the right ventricle-pulmonary circulation unit: state of the art and clinical and research implications. Circulation 120, 992-1007 (2009).
97. Garin, M. C., Highland, K. B., Silver, R. M. & Strange, C. Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J. Rheumatol. 36, 330-336 (2009).
98. Dimitroulas, T., Giannakoulas, G., Karvounis, H., Gatzoulis, M. A. & Settas, L. Natriuretic peptides in systemic sclerosis-related pulmonary arterial hypertension. Semin. Arthritis Rheum. 39, 278-284 (2010).