Multimodality Imaging of Cardiac Sarcoidosis Before and After Steroid Therapy
A 65-year-old woman was referred to our hospital because of chest pain. Right ventricular bundle-block was noted on the ECG. Angiotensin-converting enzyme level was elevated (33.2 IU/L). Delayed-enhanced magnetic resonance imaging (MRI) using an inversion-recovery segmented gradient echo sequence performed 15 minutes after gadolinium contrast injection (0.15 mmol/kg of gadodiamide) disclosed hyperenhancement, mainly in the outer layer of the septal, inferior, and anterolateral walls (Figure 1A). Cine MRI revealed wall motion abnormalities in septal and inferior walls (Figure 1B and 1C; Movie I). Left ventricular ejection fraction was 44%. Resting 201thallium single photon emission tomography revealed perfusion defects in these walls (Figure 1D). Positron emission tomography with 18fluorodeoxyglucose (FDG PET) imaging performed in a fasting condition showed FDG accumulation in the septal, inferior, and anterolateral walls, corresponding to areas with late enhancement (Figure 1E). Whole-body FDG PET imaging depicted significant FDG uptake not only in the heart but also in the hilar, mediastinal, and cervical lymph nodes (Figure 2, Movie II, left). Cardiac involvement of sarcoidosis was confirmed by histological analysis of endomyocardial biopsy. After 6 months of steroid therapy, her angiotensin-converting enzyme level was decreased (13.1 IU/L). Areas with delayed enhancement were markedly diminished (Figure 3A). Wall motion abnormalities (Figure 3B and 3C; Movie III) and perfusion abnormalities (Figure 3D) in the septal and inferior walls were not significantly changed. Her left ventricular ejection fraction was 41%. FDG accumulation was surprisingly decreased in the heart and lymph nodes (Figure 4, Movie II, right).
Before steroid therapy, areas with hyperenhancement in the delayed-enhanced images corresponded to the areas with decreased 201thallium defects, increased FDG uptake, and wall motion abnormalities. Thus, delayed enhancement in contrast-enhanced MRI is considered to reflect fibrogranulomatous tissues of sarcoidosis replacing the normal myocardium. The number of areas with late enhancement and FDG uptake in the heart was surprisingly decreased after the suppression of the disease activities by the steroid therapy, whereas wall motion abnormalities were not significantly improved. The pathophysiological condition of cardiac sarcoidosis has been clearly identified by multimodality imaging.
The online-only Data Supplement, which contains 3 movies, is available at http://circ.ahajournals.org/cgi/content/full/113/20/e771/DC1.