Main Article Content
Catheterization; Spiral CT scan; Doppler echocardiography; Pulmonary arterial hypertension
Background: Because of invasive nature of catheterization, using other noninvasive tools is more preferred to assess pulmonary arterial hypertension (PAH). The present study assessed the value of chest spiral CT scan and Doppler echocardiography compared to right heart catheterization (RHC) to predict PAH in patients with scleroderma.
Methods: This cross-sectional study was performed on 15 patients with limited scleroderma. All subjects underwent Doppler echocardiography (to assess PAP) and chest spiral CT scan without injection (to assess pulmonary trunk length or PUL), followed by RHC to assess PAH.
Results: Comparing PUL in spiral CT scan with PAP in RHC yielded a sensitivity of 75.0% and a specificity of 100% for predicting PAH. Similarly, comparing PAP value in echocardiography with PAP in RHC achieved a sensitivity of 100% and a specificity of 63.6% to discriminate PAH from normal PAP condition. Analysis of the area under the ROC curve showed high power of CT scan to predict PAH (AUC = 1.000). The best cutoff point for PUL to predict PAH was 29.95 yielding a sensitivity of 100% and a specificity of 100%. Also, ROC curve analysis showed high value of echocardiography to discriminate PAH from normal PAP status (AUC = 0.841) that considering a cutoff value of 22.88 for PAP assessed by echocardiography reached to a sensitivity of 72.7% and a specificity of 100%.
Conclusion: Both chest spiral CT scan and Doppler echocardiography are very useful to diagnose PAH and its severity in patients with scleroderma.
2. Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003; 62:1088–93.
3. Phung S, Strange G, Chung LP, Leong J, Dalton B, Roddy J, et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Intern Med J 2009; 39:682–91. doi: 10.1111/j.1445-5994.2008.01823.x.
4. Avouac J, Airò P, Meune C, Beretta L, Dieude P, Caramaschi P, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and meta-analysis of 5 studies. J Rheumatol 2010; 37:2290–8.
5. Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, et al. Isolated pulmonary hypertension in scleroderma. Intern Med J 2005; 35:28–33.
6. Robert-Thomson PJ, Mould TL, Walker JG, Smith MD, Ahern MJ. Clinical utility of telangiectasia of hands in scleroderma and other rheumatic disorders. Asian Pac J Allergy Immunol 2002; 20: 7–12.
7. Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. J Rheumatol 2010; 37:98–104.
8. Ong YY, Nikoloutsopoulos T, Bond CP, Smith MD, Ahern MJ, Roberts-Thomson PJ. Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension. Asian Pac J Allergy Immunol 1998; 16:81–6.
9. Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2009; 34:1219–63.
10. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003; 48:516–22.
11. Kawut SM, Taichman DB, rcher-Chicko CL, Palevsky HI, Kimmel SE, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123:344–50.
12. Koh ET, Lee P, Gladman DD, bu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; 35:989–93.
13. Rich S. Primary pulmonary hypertension. Curr Treat Options Cardiovasc Med 2000; 2:135–40.
14. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for Scleroderma Criteria of the ARA Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980; 23:581-90.
15. Janda S, Shahidi N, Gin K, Swiston J.Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis.Heart. 2011; 97:612-22.
16. Taleb M, Khuder S, Tinkel J, KhouriSJ. The diagnostic accuracy of Doppler echocardiography in assessment of pulmonary artery systolic pressure: a meta-analysis.Echocardiography. 2013; 30:258-65.
17. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002; 39:1214–9.
18. Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985;6: 359–65.
19. Currie PJ, Seward JB, Chan KL, Fyfe DA, Hagler DJ, Mair DD, et al. Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol 1985; 6:750–6.
20. Fisher MR, Criner GJ, Fishman AP, Hassoun PM, Minai OA, Scharf SM, et al. Estimating pulmonary artery pressures by echocardiography in patients with emphysema. Eur Respir J 2007; 30: 914–21. Epub 2007 Jul 25.
21. Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J RespirCrit Care Med 2003; 167: 735–40.
22. Frazier AA , Galvin JR , Franks TJ , Rosado-de-Christenson ML . Pulmonary vasculature: hypertension and infarction. Radiographics 2000; 20:491–524
23. 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
24. 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