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Abstract
Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.
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Fig. 1.
Acute cardiac sarcoidosis (CS) in a 54-year-old man who presented as an acute atypical chest pain. Short-axis late gadolinium enhanced (LGE) MR images (a) show multifocal patchy enhancement in the anteroseptal and inferoseptal wall of basal LV level (upper row) and inferoseptal wall of midventricuar LV level (lower row). Notice the enhancement is located in the noncoronary vascular territory and preserved subendocardial layer. Short-axis T2-weighted MR images (b) show high signal intensity (arrows) in the corresponding regions with the enhancement area, which is suggested of the myocardial edema.
Fig. 2.
Chronic CS in a 74-year-old woman who complained dyspnea. Short-axis SSFP MR cine image (a) shows decreased wall motion and decreased wall thickness of inferolateral wall (arrows) at the basal LV level. Short-axis LGE MR image (b) shows transmural enhancement at thinned inferolateral wall (arrows) and multifocal patchy enhancement in the inferoseptal wall (arrowheads).
Fig. 3.
Suspected CS in a 36-year-old man who complained orthopnea. Short-axis LGE (a) shows subtle enhancement at midventricular inferoseptal wall and anterolateral wall (arrows). Pre-contrast T1 map (b) and post-contrast T1 map (c) shows increased native T1 value (1320 msec) and increased extracellular volume (35.3%) than normal control value, which is helpful for the early diagnosis of a cardiac involvement of sarcoidosis.
Fig. 4.
Multimodality imaging in a 59-year-old woman with systemic sarcoidosis. Non-ECG gated chest CT images with mediastinal window setting (a) show enlarged lymph nodes along the mediastinum (arrowheads) and lung window setting (b) show scattered several lung nodules in both lung (arrows). Four-chamber LGE MR image (c) shows subepicardial enhancement (arrowheads) at the basal septal wall and midventricular to basal lateral wall. The FDG PET images (d, e) show increased uptake in mediastinal lymph nodes (arrowheads in d) and septal and lateral wall of the LV (arrowheads in e) which are at a corresponding region with enhancement on the LGE MR image (arrowheads in c).
Fig. 5.
Conjoined imaging of PET and MR in a 59-year-old woman with cardiac sarcoidosis. Short-axis SSFP cine image (a) shows hypokinesia at the region of the midventricular inferoseptal wall (arrow), with decreased EF as 48%. Short-axis T2-weighted image (b) shows high signal intensity at inferoseptal wall of the LV (arrow), suggesting edema. The LGE MR image (c) shows subepicardial enhancement at concordant area, indicating fibrosis. Conjoined PET-MR image (d) reveals increased uptake (arrow) at the enhanced area of the inferoseptal wall in LGE MR image, which is well presented in the active inflammatory phase of CS (c and d images are LGE MR image and PET image matching the same level).
Fig. 6.
Acute myocarditis in a 28-year-old man who presented with the syncope and elevated cardiac enzyme. Short-axis LGE images (a) show multifocal patchy enhancement at the subepicardial layer of anterolateral and inferoseptal wall with preserved subendocardial layer. T2-weighted images (b) show high signal intensity in the same regions (arrows) with enhanced area. The condition resembles CS, therefore clinical presentation and other blood tests should be carefully considered.
Fig. 7.
Dilated cardiomyopathy in a 54-year-old woman who complained of dyspnea and a progressed heart failure. Short-axis LGE MR image shows dilated LV chamber and linear enhancement at the midwall region of the interventricular septum (arrows).
Fig. 8.
Hypertrophic cardiomyopathy in a 58-year-old woman with recurrent ventricular tachycardia. Short-axis LGE MR image (a) shows multifocal patchy enhancement (arrows) at hypertrophied anteroseptal wall, around the anterior RV insertion sites. However, the T2-weighted image (b) show no evidence of abnormally increased signal intensity.
Fig. 9.
Cardiac amyloidosis in a 68-year-old man with exertional dyspnea. Short-axis LGE image shows global subendocardial enhancement (arrows) at whole LV wall. Subtle enhancement is also noted in RV free wall (arrowheads).
Table 1.
JMHW 2006 Revised Guidelines for Diagnosis of CS
| Histologic diagnosis group |
| CS is confirmed when EMB specimens show noncaseating epithelioid cell granulomas with histologic clinical diagnosis of extracardiac sarcoidosis |
| Clinical diagnosis group |
| Although EMB specimens do not show noncaseating epithelioid cell granulomas, extracardiac sarcoidosis was diagnosed histologically or clinically and satisfies the following criteria* |
| Major criteria |
| Advanced AV block |
| Basal thinning of the interventricular septum |
| Positive gallium uptake in the heart |
| Depressed LVEF (< 50%) |
| Minor criteria |
| Abnormal ECG findings: ventricular arrhythmias (VT, multifocal or frequent PVC), complete RBBB, axis deviation, or abnormal Q wave |
| Abnormal echocardiographic findings: regional abnormal wall motion or morphologic abnormality (ventricular aneurysm, wall thickening) |
| Nuclear medicine findings: perfusion defect detected with thallium 201 (201Th) or technetium 99m (99mTc) myocardial scintigraphy |
| MR imaging findings: delayed (late) gadolinium-induced enhancement of myocardium EMB findings: interstitial fibrosis or monocyte infiltration of more than moderate grade |
Table 2.
Heart Rhythm Society (HRS) Expert Consensus Recommendations on Criteria for Diagnosis of CS*
Table 3.
The Reported Common Distribution Patterns of Late Gadolinium Enhancement of Myocardium in Patients with Cardiac Sarcoidosis
Table 4.
Differential Diagnosis of CS
| Diagnosis | Clinical characteristics | MRI findings |
|---|---|---|
| CS | Multisystem disease, conduction abnormality, | Subepicardial and mid wall LGE at basal septum or inferolateral wall |
| mediastinal lymphadenopathy | ||
| Myocarditis | Recent history of symptoms from viral infection | Subepicardial LGE in noncoronary pattern, edema in acute setting, |
| such as fever or chills, chest pain mimicking MI, | commonly involves lateral wall* | |
| elevated cardiac enzyme | ||
| Idiopathic | Heart failure, dyspnea on exertion, palpitations, | Dilated LV or both ventricles and reduced systolic function, no LGE or |
| DCM | asymptomatic cardiomegaly | linear mid wall LGE in interventricular septum* |
| HCM | Wide spectrum from asymptomatic to sudden | Increased LV wall thickness >15 mm, LGE at hypertrophied segment, |
| cardiac death | frequently involve at interventricular septum, adjacent RV insertion site* | |
| Amyloidosis | Multisystem disease, heart failure and dyspnea | Global subendocardial LGE |
| Thickening of LV wall, valve leaflets, and papillary muscles | ||
| Interatrial septal thickening and other chamber involvement* | ||
| ARVC | Family history, syncope or tachycardia | RV dilatation and focal dyskinesia or akinesia, fibrofatty infiltration of |
| Frequently RV involve (LV involvement in about | RV, especially in the outflow tract and anterobasal area* | |
| 10% of cases) |
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