Detection of Myocardial Fibrosis in Systemic Sclerosis by Contrast-Enhanced Magnetic Resonance Imaging

Zusammenfassung

Ziel: Ziel der vorliegenden Studie war es, die Prävalenz sowie das Muster einer späten myokardialen Kontrastmittelanreicherung (Late Gadolinium Enhancement; LGE) als In-vivo-Korrelat einer myokardialen Fibrose bei kardial asymptomatischen Patienten mit systemischer Sklerodermie zu untersuchen. Material und Methoden: Eine kardiale MRT wurde erfolgreich in 34 Patienten (30 Frauen, 14 Männer, mittleres Alter 54 ± 14 Jahre) mit gesicherter systemischer Sklerodermie sowie in einem nach Alter, Geschlecht und kardiovaskulären Risikofaktoren gematchten Kontrollkollektiv durchgeführt. Alle Untersuchungen wurden an einem 1,5T-MR-Scanner (Avanto, Siemens Medical Solutions, Erlangen, Deutschland) durchgeführt. Zehn bis 15 min nach intravenöser Injektion von 0,2 mmol Gd-DTPA pro Kg Körpergewicht wurde eine 2-D-Inversion-Recovery präparierte gespoilte Gradientenechosequenz (TR 8,0 ms, TE 4,0 ms, TI 180 – 240 ms, FA 20°, Schichtdicke 8 mm, Auflösung 1,2 × 1,2 mm²) in Standardkurz- und Langachsenschichten aufgenommen, um ein myokardiales LGE zu detektieren. Ergebnisse: Fleckige unscharf begrenzte sowie scharf begrenzte Kontrastmittelanreicherungen fanden sich in 5 von 34 (15 %) Sklerodermie-Patienten vorwiegend intramural unter Aussparung des subendo- und subepikardialen Myokards in den basalen Schichten des linken Ventrikels. Eine fokale Kontrastmittelanreicherung wurde in einer Kontrolle (3 %, p > 0,05) im apikalen Septum beobachtet. Insgesamt wurden Kontrastmittelanreicherungen in 15 Segmenten der Sklerodermie-Patienten und nur in 1 Segment der Kontrollen beobachtet (p < 0,005). Schlussfolgerung: Unsere Studie zeigt eine signifikant höhere Anzahl an Segmenten mit pathologischer Kontrastmittelanreicherung bei Patienten mit systemischer Sklerodermie im Vergleich zu unserem Kontrollkollektiv und belegt damit die Fähigkeit der kontrastverstärkten kardialen MRT, das entscheidende Kennzeichen einer kardialen Beteiligung bei systemischer Sklerodermie – die myokardiale Fibrose – in vivo nachzuweisen.

Abstract

Purpose: Our study aimed to assess the prevalence and pattern of late gadolinium enhancement (LGE) as an in vivo correlate of myocardial fibrosis in cardiac asymptomatic patients with systemic sclerosis (SSc). Materials and Methods: Cardiac magnetic resonance imaging was successfully performed in 34 patients (30 female, 4 male, mean age of 54 ± 14 years) with proven SSc and in controls with matching age, sex and cardiovascular risk factors. All examinations were performed on a 1.5 T MR system (Avanto, Siemens, Germany). 2D inversion recovery spoiled gradient echo images (TR 8.0 msec, TE 4.0 msec, TI 180 – 240 msec, FA 20°, slice thickness 8 mm, in-plane resolution 1.2 × 1.2 mm²) were acquired 10 to 15 min after injection of 0.2 mmol Gd-DTPA per kg body weight to detect myocardial LGE. Results: Poorly defined, patchy as well as well-defined focal areas of LGE were detected predominantly in the mid-myocardial layer of the basal left ventricular segments in 5 of 34 (15 %) SSc patients. A focal area of LGE was observed within the apical septum in one control (3 %, p > 0.05). LGE was observed in a total of 15 segments of the SSc patients and in 1 segment of the controls (p < 0.005). Conclusion: Our results revealed a significantly higher number of LGE positive segments in patients with SSc compared to matched controls, and demonstrate the ability of contrast-enhanced MRI to detect myocardial fibrosis, the hallmark of scleroderma heart disease, in-vivo.

late gadolinium enhancement - CMR - systemic sclerosis - scleroderma heart disease

References

• 1 Ferri C, Valentini G, Cozzi F. et al . Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients.  Medicine. 2002;  81 139-153
• 2 Ferri C, Di Bello V, Martini A. et al . Heart involvement in systemic sclerosis: an ultrasonic tissue characterisation study.  Ann Rheum Dis. 1998;  57 296-302
• 3 Follansbee W P, Miller T R, Curtiss E I. et al . A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma).  J Rheumatol. 1990;  17 656-662
• 4 Byers R J, Marshall D A, Freemont A J. Pericardial involvement in systemic sclerosis.  Ann Rheum Dis. 1997;  56 393-394
• 5 Candell-Riera J, Armadans-Gil L, Simeon C P. et al . Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis.  Arthritis Rheum. 1996;  39 1138-1145
• 6 Kazzam E, Caidahl K, Hallgren R. et al . Mitral regurgitation and diastolic flow profile in systemic sclerosis.  Int J Cardiol. 1990;  29 357-363
• 7 Kazzam E, Waldenstrom A, Landelius J. et al . Non-invasive assessment of left ventricular diastolic function in patients with systemic sclerosis.  J Intern Med. 1990;  228 183-192
• 8 Giunta A, Tirri E, Maione S. et al . Right ventricular diastolic abnormalities in systemic sclerosis. Relation to left ventricular involvement and pulmonary hypertension.  Ann Rheum Dis. 2000;  59 94-98
• 9 Meune C, Allanore Y, Devaux J Y. et al . High prevalence of right ventricular systolic dysfunction in early systemic sclerosis.  J Rheumatol. 2004;  31 1941-1945
• 10 Kostis J B, Seibold J R, Turkevich D. et al . Prognostic importance of cardiac arrhythmias in systemic sclerosis.  Am J Med. 1988;  84 1007-1015
• 11 Steen V D. Systemic sclerosis.  Rheum Dis Clin North Am. 1990;  16 641-654
• 12 Fernandes F, Ramires F J, Arteaga E. et al . Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study.  J Card Fail. 2003;  9 311-317
• 13 Hunold P, Kreitner K F, Barkhausen J. “Dead or alive?”: how and why myocardial viability imaging by cardiac MRI works.  Röntgenstr Fortschr. 2007;  179 1016-1024
• 14 Hunold P, Schlosser T, Vogt F M. et al . Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction between infarction scar and non-infarction-related disease.  Am J Roentgenol. 2005;  184 1420-1426
• 15 Mahrholdt H, Goedecke C, Wagner A. et al . Cardiovascular magnetic resonance assessment of human myocarditis: a comparison to histology and molecular pathology.  Circulation. 2004;  109 1250-1258
• 16 Teraoka K, Hirano M, Ookubo H. et al . Delayed contrast enhancement of MRI in hypertrophic cardiomyopathy.  Magn Reson Imaging. 2004;  22 155-161
• 17 Mayes M D. Classification and epidemiology of scleroderma.  Semin Cutan Med Surg. 1998;  17 22-26
• 18 LeRoy E C, Black C, Fleischmajer R. et al . Scleroderma (systemic sclerosis): classification, subsets and pathogenesis.  J Rheumatol. 1988;  15 202-205
• 19 Brennan P, Silman A, Black C. et al . Reliability of skin involvement measures in scleroderma. The UK Scleroderma Study Group.  Br J Rheumatol. 1992;  31 457-460
• 20 Paulus W J. How to diagnose diastolic heart failure. European Study Group on Diastolic Heart Failure.  Eur Heart J. 1998;  19 990-1003
• 21 Ladd S C, Wanke I, Gizewski E R. et al . First results of a prospective whole-body MR screening study.  European Radiology. 2007;  17 (supplements) 202
• 22 Hudsmith L E, Petersen S E, Francis J M. et al . Normal human left and right ventricular and left atrial dimensions using steady state free precession magnetic resonance imaging.  J Cardiovasc Magn Reson. 2005;  7 775-782
• 23 Steen V D, Medsger T A. Severe organ involvement in systemic sclerosis with diffuse scleroderma.  Arthritis Rheum. 2000;  43 2437-2444
• 24 Follansbee W P. Organ Involvement: cardiac. Clements PJ, Furst DE Systemic sclerosis Baltimore; Wiliams Wilkins 1996: 333-364
• 25 D’Angelo W A, Fries J F, Masi A T. et al . Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls.  Am J Med. 1969;  46 428-440
• 26 Beer Jr M, Buchner S, Wirbelauer J. et al . MR imaging and MR spectroscopy for characterization of cardiomyopathies in adolescents – preliminary results.  Röntgenstr Fortschr. 2007;  179 932-937
• 27 Seeger A, Doesch C, Klumpp B. et al . MR stress perfusion for the detection of flow-limiting stenoses in symptomatic patients with known coronary artery disease and history of stent implantation.  Röntgenstr Fortschr. 2007;  179 1068-1073
• 28 Schafer R, Wiskirchen J, Guo K. et al . Aptamer-based isolation and subsequent imaging of mesenchymal stem cells in ischemic myocard by magnetic resonance imaging.  Röntgenstr Fortschr. 2007;  179 1009-1015
• 29 Ishida R, Murata Y, Sawada Y. et al . Thallium-201 myocardial SPET in patients with collagen disease.  Nucl Med Commun. 2000;  21 729-734
• 30 Sulli A, Ghio M, Bezante G P. et al . Blunted coronary flow reserve in systemic sclerosis.  Rheumatology. 2004;  43 505-509
• 31 Chung G, Lahiri A. Myocardial perfusion imaging in modern cardiology.  Br J Cardiol. 2000;  7 619-623
• 32 Beranek J T. Pathogenesis of heart fibrosis in systemic sclerosis.  Int J Cardiol. 2001;  80 261-262
• 33 Conroy R M, Pyorala K, Fitzgerald A P. et al . Estimation of ten-year risk of fatal cardiovascular disease in Europe: the SCORE project.  Eur Heart J. 2003;  24 987-1003
• 34 Dumont C A, Monserrat L, Soler R. et al . Clinical significance of late gadolinium enhancement on cardiovascular magnetic resonance in patients with hypertrophic cardiomyopathy.  Rev Esp Cardiol. 2007;  60 15-23
• 35 Blyth K G, Groenning B A, Martin T N. et al . Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertension.  Eur Heart J. 2005;  26 1993-1999
• 36 Dziadzio M, Giovagnoni A, Pomponio G. et al . Acute myocarditis associated with adenoviral infection in a patient with scleroderma.  Clin Rheumatol. 2003;  22 487-490
• 37 Tzelepis G E, Kelekis N L, Plastiras S C. et al . Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study.  Arthritis Rheum. 2007;  56 3827-3836
• 38 Ferri C, Giuggioli D, Sebastiani M. et al . Heart involvement and systemic sclerosis.  Lupus. 2005;  14 702-707
• 39 Assomull R G, Prasad S K, Lyne J. et al . Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy.  J Am Coll Cardiol. 2006;  48 1977-1985
• 40 Allanore Y, Meune C, Vignaux O. et al . Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and Tissue-Doppler echography study.  J Rheumatol. 2006;  33 2464-2469

2 ¹ McNemar test.

5 ² Student-t test.

6 ³ χ²-test.