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Myocardial perfusion defects in scleroderma detected by contrast-enhanced cardiovascular magnetic resonance

  • Cardiac Radiology
  • Published:
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An Erratum to this article was published on 15 July 2014

Abstract

Purpose

The authors investigated whether contrast-enhanced cardiovascular magnetic resonance (CMR) imaging may be used to detect early cardiac involvement in patients with systemic sclerosis (SSc).

Materials and methods

Twenty-six SSc patients (nine with diffuse cutaneous SSc and 17 with limited cutaneous SSc) and 13 sex- and age-matched healthy controls (HC) were studied. Contrast-enhanced CMR allowed the analysis of first-pass images (areas of hypo-enhancement indicating perfusion defects) and delayed images (persistent hyper-enhancement indicating fibrosis). Clinical variables including disease duration and presence of major visceral complications of SSc were investigated in each patient.

Results

Perfusion defects were detected in 53.8 % of SSc patients but in none of the HC. Perfusion abnormalities were detected in 28.6 % of SSc patients with disease duration less than 2 years and in 29.2 % of asymptomatic SSc patients. Delayed contrast enhancement was present in 25 % of SSc patients but not in HC. All patients with delayed contrast enhancement showed first-pass hypoperfusion. Right ventricular wall thickness was significantly increased in all SSc patients when compared to HC (p < 0.001); a similar trend was observed when SSc patients without pulmonary arterial hypertension were analysed (p < 0.04). A trend to lower end-diastolic and end-systolic right ventricular volumes in SSc versus HC was observed (p < 0.05 and p < 0.04, respectively).

Conclusions

Myocardial hypoperfusion is common in SSc and occurs early in the course of the disease. Co-localisation of perfusion defects and delayed contrast enhancement indicative of fibrosis suggests that myocardial hypoxia may play a role in the pathogenesis of myocardial fibrosis.

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References

  1. Black CM, Denton CP (1998) Systemic sclerosis and related disorders in adults and children. In: Maddison PG, Isenberg DA, Woo P, Glass DN (eds) Oxford Textbook of Rheumatology, Oxford University Press, Inc. 2nd edn. vol 2, pp 1217–1247

  2. Deswal A, Follansbee WP (1996) Cardiac involvement in scleroderma. Rheum Dis Clin North Am 22:841–860

    Article  CAS  PubMed  Google Scholar 

  3. Coghlan JG, Mukerjee D (2001) The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. Curr Opin Rheumatol 13:495–499

    Article  CAS  PubMed  Google Scholar 

  4. Steen V (2004) The heart in systemic sclerosis. Curr Rheumatol Rep 6:137–140

    Article  PubMed  Google Scholar 

  5. Medsger TA Jr, Masi AT (1973) Survival with scleroderma-II. A life-table analysis of clinical and demographic factors in 358 male US veteran patients. J Chron Dis 26:647–660

    Article  PubMed  Google Scholar 

  6. Follansbee WP, Miller TR, Curtiss EL et al (1990) A controlled clinico-pathologic study of myocardial fibrosis in systemic sclerosis (scleroderma). J Rheumatol 17:656–662

    CAS  PubMed  Google Scholar 

  7. Marzullo P, Parodi O, Sambuceti G et al (1995) Myocardial viability: nuclear medicine versus stress echocardiography. Echocardiography 12:291–302

    Article  CAS  PubMed  Google Scholar 

  8. Valentini G, Vitale DF, Giunta A et al (1996) Diastolic abnormalities in systemic sclerosis: evidence for associated defective cardiac functional reserve. Ann Rheum Dis 55:455–460

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Armstrong GP, Whalley GA, Doughty RN et al (1996) Left ventricular function in scleroderma. Br J Rheumatol 35:983–988

    Article  CAS  PubMed  Google Scholar 

  10. Ferri C, Di Bello V, Martini A et al (1998) Heart involvement in systemic sclerosis: an ultrasonic tissue characterisation study. Ann Rheum Dis 57:296–302

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  11. Pennell D (2001) Imaging techniques. Cardiovascular magnetic resonance. Heart 85:581–589

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  12. Panting JR, Gatehouse PD, Yang GZ et al (2002) Abnormal subendocardial perfusion in cardiac syndrome X detected by cardiovascular magnetic resonance imaging. N Engl J Med 346:1948–1953

    Article  PubMed  Google Scholar 

  13. Kim RJ, Fieno DS, Parrish TB et al (1999) Relationship of MRI delayed contrast enhancement to irreversible injury, infarct age and contractile function. Circulation 100:1992–2002

    Article  CAS  PubMed  Google Scholar 

  14. Karwatowski S, Chronos A, Sinclaire H et al (2000) Effect of systemic sclerosis on left ventricular long-axis motion and left ventricular mass assessed by magnetic resonance. J Cardiovasc Magn Reson 2:109–117

    Article  CAS  PubMed  Google Scholar 

  15. Moon J, Coghlan JG, Pennell DJ (2001) Systemic sclerosis involving the heart. Heart 86:308

    CAS  PubMed Central  PubMed  Google Scholar 

  16. Dziadzio M, Giovagnoni A, Pomponio G et al (2003) Acute myocarditis associated with adenoviral infection in a patient with scleroderma. Clin Rheumatol 22:487–490

    Article  PubMed  Google Scholar 

  17. Plastiras SC, Kelekis N, Tzelepis GE (2006) Magnetic resonance imaging for the detection of myocardial fibrosis in scleroderma. N Engl J Med 354:2194–2196

    Article  CAS  PubMed  Google Scholar 

  18. Tzelepis GE, Kelekis NL, Plastiras SC et al (2007) Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 56:3827–3836

    Article  PubMed  Google Scholar 

  19. Hachulla AL, Launay D, Gaxotte V et al (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68:1878–1884

    Article  PubMed Central  PubMed  Google Scholar 

  20. Kobayashi H, Yokoe I, Hirano M et al (2009) Cardiac magnetic resonance imaging with pharmacological stress perfusion and delayed enhancement in asymptomatic patients with systemic sclerosis. J Rheumatol 36:106–112

    PubMed  Google Scholar 

  21. Mavrogeni S, Bratis K, van Wijk K et al (2012) Myocardial perfusion-fibrosis pattern in systemic sclerosis assessed by cardiac magnetic resonance. Int J Cardiol 159:e56–e58

    Article  PubMed  Google Scholar 

  22. Subcommitee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590

    Article  Google Scholar 

  23. LeRoy EC, Black CM, Fleishmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205

    CAS  PubMed  Google Scholar 

  24. Medsger TA Jr (2003) Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being. Rheum Dis Clin N Am 29:255–273

    Article  Google Scholar 

  25. Valentini G, Medsger TA Jr, Silman AJ, Bombardieri S (2003) Conclusion and identification of the core set of variables to be used in clinical investigations. Clin Exp Rheumatol 21(Suppl. 29):S47–S56

    CAS  PubMed  Google Scholar 

  26. Appendix. Manual of signs, symptoms, methods and procedures for the assessment of the patient with SSc. Clin Exp Rheumatol 2003;21(Suppl.29):S49–S56

  27. Ferri C, Emdin M, Nielsen H, Bruhlmann P (2003) Assessment of heart involvement. Clin Exp Rheumatol 21(Suppl. 29):S24–S28

    CAS  PubMed  Google Scholar 

  28. Committee Guidelines (2003) European Society of hypertension-European Society of Cardiology guidelines for the management of arterial hypertension. J Hypertens 21:1011–1053

    Article  Google Scholar 

  29. Sahn DJ, De Maria A, Kissi S, Wayman A (1978) Recommendations regarding quantification in M-mode echocardiography: results of a series of echocardiographic measurements. Circulation 58:1072–1083

    Article  CAS  PubMed  Google Scholar 

  30. Barst RJ, McGoon M, Torbicki A et al (2004) Diagnosis and differential assessment of pulmonary arterial hypertension. JACC 43(Suppl S):40S–47S

    Article  PubMed  Google Scholar 

  31. Lin CC, Ding HJ, Chen YW et al (2003) Usefulness of technetium-99m sestamibi myocardial perfusion SPECT in detection of cardiovascular involvement in patients with systemic lupus erythematosus or systemic sclerosis. Int J Cardiol 92:157–161

    Article  PubMed  Google Scholar 

  32. Nakajima K, Kawano M, Hasegawa M et al (2006) Myocardial damages in systemic sclerosis detected by gated myocardial perfusion SPECT and sympathetic imaging. Circ J 70:1481–1487

    Article  PubMed  Google Scholar 

  33. Sulli A, Ghio M, Bezante GP et al (2004) Blunted coronary flow reserve in systemic sclerosis. Rheumatology 43:505–509

    Article  CAS  PubMed  Google Scholar 

  34. D’Angelo WA, Fries JF, Masi AT, Shulman LE (1969) Pathologic observations in systemic sclerosis (scleroderma): a study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 46:428–440

    Article  PubMed  Google Scholar 

  35. Akram MR, Handler CE, Williams M et al (2006) Angiographically proven coronary artery disease in scleroderma. Rheumatology 45:1395–1398

    Article  CAS  PubMed  Google Scholar 

  36. Avouac J, Fransen J, Walker UA et al (2011) Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann Rheum Dis 70:476–481

    Article  CAS  PubMed  Google Scholar 

  37. Di Cesare E, Battisti S, Di Sibio A et al (2013) Early assessment of sub-clinical cardiac involvement in systemic sclerosis (SSc) using delayed enhancement cardiac magnetic resonance (CE-MRI). Eur J Radiol 82:e268–e273

    Article  PubMed  Google Scholar 

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Acknowledgments

This study was supported by grants from Fondazione Italiana Ricerca Artrite (FIRA) and Ministero Italiano per l’Università e la Ricerca Scientifica (MIUR) to A.G., and from Fondazione di Medicina Molecolare e Terapia Cellulare, Università Politecnica delle Marche, Ancona, Italy.

Conflict of interest

N. Schicchi, G. Valeri, G. Moroncini, G. Agliata, L. Salvolini, A. Gabrielli and A. Giovagnoni declare that they have no conflict of interest.

Ethical statement

The research was carried out in compliance with the Helsinky Declaration. The study was approved by the Institutional Ethics Committee of Azienda Ospedali Riuniti, Università Politecnica delle Marche, Ancona, Italy, and informed consent was obtained from all subjects participating in this study.

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Authors and Affiliations

  1. Radiologia Pediatrica e Specialistica, Azienda Ospedaliero Universitaria, Ospedali Riuniti di Ancona, Via Conca 71, 60126, Torrette, Ancona, Italy

    Nicolò Schicchi & Andrea Giovagnoni

  2. Clinica di Radiologia, d’Urgenza e dell’Area Oncologica, Ospedali Riuniti Ancona, Ancona, Italy

    Gianluca Valeri

  3. Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy

    Gianluca Moroncini & Armando Gabrielli

  4. Clinica Medica, Ospedali Riuniti Ancona, Ancona, Italy

    Gianluca Moroncini & Armando Gabrielli

  5. Scuola di Specializzazione in Radiodiagnostica, Università Politecnica delle Marche, Ancona, Italy

    Giacomo Agliata

  6. Clinica di Radiologia, d’Urgenza e dell’Area Oncologica, Azienda Ospedaliero Universitaria, Ospedali Riuniti di Ancona, Ancona, Italy

    Luca Salvolini

Authors
  1. Nicolò Schicchi
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  2. Gianluca Valeri
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  3. Gianluca Moroncini
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  4. Giacomo Agliata
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  6. Armando Gabrielli
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  7. Andrea Giovagnoni
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Correspondence to Nicolò Schicchi.

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Schicchi, N., Valeri, G., Moroncini, G. et al. Myocardial perfusion defects in scleroderma detected by contrast-enhanced cardiovascular magnetic resonance. Radiol med 119, 885–894 (2014). https://doi.org/10.1007/s11547-014-0419-7

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  • DOI: https://doi.org/10.1007/s11547-014-0419-7

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