Abstract
PurposeTo evaluate the diagnostic and prognostic significance of combined cardiac 18F-fluorodeoxyglucose (FDG) PET/MRI with T1/T2 mapping in the evaluation of suspected cardiac sarcoidosis.MethodsPatients with suspected cardiac sarcoidosis were prospectively enrolled for cardiac 18F-FDG PET/MRI, including late gadolinium enhancement (LGE) and T1/T2 mapping with calculation of extracellular volume (ECV). The final diagnosis of cardiac sarcoidosis was established using modified JMHW guidelines. Major adverse cardiac events (MACE) were assessed as a composite of cardiovascular death, ventricular tachyarrhythmia, bradyarrhythmia, cardiac transplantation or heart failure. Statistical analysis included Cox proportional hazard models.ResultsForty-two patients (53 ± 13 years, 67% male) were evaluated, 13 (31%) with a final diagnosis of cardiac sarcoidosis. Among patients with cardiac sarcoidosis, 100% of patients had at least one abnormality on PET/MRI: FDG uptake in 69%, LGE in 100%, elevated T1 and ECV in 100%, and elevated T2 in 46%. FDG uptake co-localized with LGE in 69% of patients with cardiac sarcoidosis compared to 24% of those without, p = 0.014. Diagnostic specificity for cardiac sarcoidosis was highest for FDG uptake (69%), elevated T2 (79%), and FDG uptake co-localizing with LGE (76%). Diagnostic sensitivity was highest for LGE, elevated T1 and ECV (100%). After median follow-up duration of 634 days, 13 patients experienced MACE. All patients who experienced MACE had LGE, elevated T1 and elevated ECV. FDG uptake (HR 14.7, p = 0.002), elevated T2 (HR 9.0, p = 0.002) and native T1 (HR 1.1 per 10 ms increase, p = 0.044) were significant predictors of MACE even after adjusting for left ventricular ejection fraction and immune suppression treatment. The presence of FDG uptake co-localizing with LGE had the highest diagnostic performance overall (AUC 0.73) and was the best predictor of MACE based on model goodness of fit (HR 14.9, p = 0.001).ConclusionsCombined cardiac FDG-PET/MRI with T1/T2 mapping provides complementary diagnostic information and predicts MACE in patients with suspected cardiac sarcoidosis.
Highlights
Sarcoidosis is a multisystem disease characterized by development and accumulation of non-caseating granulomas in various organs
Overall diagnostic performance was highest for focal FDG uptake co-localizing with late gadolinium enhancement (LGE) or with elevated T1
The presence of focal FDG uptake co-localizing with LGE or with elevated T1 was associated with 12 times higher risk of Major adverse cardiac events (MACE) compared to patients without and was the best predictor of MACE among the imaging findings evaluated based on model goodness of fit
Summary
Sarcoidosis is a multisystem disease characterized by development and accumulation of non-caseating granulomas in various organs. Sarcoidosis can involve the heart and other organs (Okada et al 2018). Cardiac sarcoidosis remains a diagnostic challenge (Perry and Vuitch 1995; Rybicki et al 1997). Endomyocardial biopsy remains the reference standard to confirm cardiac involvement, the diagnostic yield is less than 25% (Vignaux 2005). Expert consensus criteria have been proposed for diagnosing cardiac sarcoidosis, these may have limited diagnostic accuracy (Hulten et al 2016). There is no reliable serum biomarker to confirm the diagnosis of cardiac sarcoidosis or assess response to therapy, and clinicians are reliant on imaging for assessment of cardiac involvement and disease activity
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