Number 10-11 Cardiac Sarcoidosis - CMR assessment in the Acute Phase and At Follow-Up
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Number 10-11 Cardiac Sarcoidosis - CMR assessment in the Acute Phase and At Follow-Up

Case from: Florian von Knobelsdorff-Brenkenhoff, Jeanette Schulz-Menger
Institute: Working group on Cardiovascular Magnetic Resonance, Charité Medical University Berlin, HELIOS Klinikum Berlin-Buch, Dept. of Cardiology and Nephrology, Berlin, Germany.

Clinical history: A 32-year-old man presented with chest pain, fever, erythema nodosum of the lower extremities and swelling of both ankles. ECG showed ST-elevation in leads I and aVL (Image 1) associated with a troponin rise (up to 40-fold).



Image 1



Movie 1                                                Movie 2                                             Movie 3


Urgent coronary angiogram ruled out coronary artery disease (Movies 1 & 2), but revealed regional wall motion abnormalities anteriorly, and possibly apically (Movie 3). CMR was requested to further investigate the nature of the regional wall motion abnormality.



Movie 4                                                        Movie 5


Cine CMR: standard SSFP cine images showed mildly impaired LV systolic function with hypokinesia of the anterior, lateral and inferior walls. A small pericardial effusion was also noted (Movies 4 & 5).



IMAGE 2                                      IMAGE 3                      IMAGE 4                           IMAGE 5                            IMAGE 6


T2-weighted imaging revealed increased signal intensity suggestive of myocardial oedema of the anterior wall (Images 2 & 3, double white arrows).
LGE imaging detected extensive disseminated subepicardial areas of myocardial enhancement (Images 4,5 and 6, double white arrows) of the anterior and infero-lateral walls, suggestive of myocardial sarcoidosis.
Overall, these imaging features (pattern of LGE and corresponding myocardial eodema) are typical of acute myocardial sarcoidosis.  

Management and clinical course: Both the clinical and CMR findings were consistent with acute cardiac sarcoidosis. Computed tomography of the chest confirmed hilar lymphadenopathy (Image 7). No additional organs were involved.





The patient was consequently started on steroids and completely recovered from a clinical point of view. A repeated CMR was performed after 2 months.



Movie 6                                                   Movie 7


Cine CMR after (2-month follow-up): Standard SSFP cines showed complete resolution of the previously noticed regional wall motion abnormalities, and a restored LV ejection fraction (Movie 6 & 7).



IMAGE 8                                                                      IMAGE 9                                                      IMAGE 10                


T2-weighted imaging (2-month follow-up) demonstrated normal myocardial signal intensity, confirming the absence of myocardial oedema (Image 8).
LGE imaging (2-month follow-up) showed a significant reduction in size and signal intensity of the sub-epicardial lesions (Image 9 & 10).

Perspective: Sarcoidosis is a common disease (5-40/100.000) [1] and affects the heart in about 25% of cases [2], thereby constituting the most frequent cause of death in this patient population. CMR reveals specific tissue alterations in cardiac sarcoidosis [3] [4] and allows both to confirm the diagnosis (without need for endomyocardial biopsy), and to monitor the course of disease [5] [6], which is useful to guide immunosuppressive treatment.

1) Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007;357:2153-65.
2) Kim JS, Judson MA, Donnino R, Gold M, Cooper LT, Prystowsky EN, Prystowsky S. Cardiac sarcoidosis. Am Heart J 2009;157:9-21
3) Schulz-Menger J, Wassmuth R, Abdel-Aty H, Siegel I, Franke A, Dietz R, Friedrich MG. Patterns of myocardial inflammation and scarring in sarcoidosis as assessed by cardiovascular magnetic resonance. Heart 2006;92;399-400
4) Patel MR, Cawley PJ, Heitner JF, Klem I, Parker MA, Jaroudi WA, Meine TJ, White JB, Elliott MD, Kim HW, Judd RM, Kim RJ. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120:1969-77
5) Vignaux O, Dhote R, Duboc D, Blanche P, Dusser D, Weber S, Legmann P. Clinical significance of myocardial magnetic resonance abnormalities in patients with sarcoidosis: a 1-year follow-up study. Chest 2002;122:1895-901.
6) Zagrosek A, Abdel-Aty H, Boyé P, Wassmuth R, Messroghli D, Utz W, Rudolph A, Bohl S, Dietz R, Schulz-Menger J. Cardiac magnetic resonance monitors reversible and irreversible myocardial injury in myocarditis. JACC Cardiovasc Imaging 2009;2:131-8.

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