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Published on: June 20, 2014
Phenotyping of giant cell myocarditis versus cardiac sarcoidosis using cardiovascular magnetic resonance
Emanuele Bobbio1, Entela Bollano1, Anders Oldfors2
1Department of Cardiology, Sahlgrenska University Hospital, Gothenburg, Sweden; Institute of Medicine, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
Insights
Giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) present similarly on cardiovascular magnetic resonance (CMR) imaging, making differentiation difficult. However, GCM shows more severe clinical presentation and higher biomarker levels than CS.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Inflammatory Diseases
Background:
- Giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare, severe myocardial inflammatory diseases.
- Limited knowledge exists regarding the cardiovascular magnetic resonance (CMR) characteristics of GCM and its differentiation from CS.
Purpose of the Study:
- To compare the clinical and CMR findings of GCM and CS.
- To assess the utility of CMR in distinguishing between GCM and CS.
Main Methods:
- A blinded analysis of clinical data and CMR imaging was performed on 40 patients with biopsy-proven GCM (n=14) and CS (n=26).
- CMR parameters assessed included ventricular dimensions, function, late gadolinium enhancement (LGE), and T2 signal intensity.
Main Results:
- Patients with GCM and CS had similar demographics and CMR findings, including ventricular alterations and LGE patterns (e.g., "hook sign").
- GCM cases exhibited significantly higher troponin T and natriuretic peptide levels and a worse clinical outcome compared to CS.
- Right ventricular involvement with increased T2 signal and/or LGE was most extensive in GCM.
Conclusions:
- CMR imaging reveals highly similar patterns for GCM and CS, challenging differentiation based solely on imaging.
- Clinical presentation and biomarker levels appear more severe in GCM, offering potential distinguishing features.
Background:
Giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare inflammatory diseases of the myocardium with poor prognosis. Little is known about the cardiovascular magnetic resonance (CMR) appearance of GCM and the methods ability to distinguish the two rare entities from one another.
Methods:
We assessed a total of 40 patients with endomyocardial biopsy-proven GCM (n = 14) and CS (n = 26) concerning their clinical and CMR appearance in a blinded manner.
Results:
Patients with GCM and CS were of similar median age (55 vs 56 years), and a male predominance was observed in both groups. In GCM, median levels of troponin T (313 vs 31 ng/L, p < 0.001), and natriuretic peptides (6560 vs 676 pg/mL, p < 0.001) were higher than in CS, and the clinical outcome worse (p = 0.04). On CMR imaging, the observed alterations of left and right ventricular (LV/RV) dimensions and function were similar. GCM showed multifocal LV late gadolinium enhancement (LGE) with a similar longitudinal, circumferential, and radial distribution as in CS, including suggested signature imaging biomarkers of CS like the "hook sign" (71% vs 77%, p = 0.702). The median LV LGE enhanced volume was 17% and 22% in GCM and CS (p = 0.150), respectively. The number of RV segments with pathologically increased T2 signal and/or LGE were most extensive in GCM.
Conclusions:
The CMR appearance of both GCM and CS is highly similar, making the differentiation between the two rare entities solely based on CMR challenging. This stands in contrast to the clinical appearance, which seems to be more severe in GCM.
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