1. In this retrospective cohort study, among 504 patients with biopsy proven sarcoidosis who underwent cardiovascular magnetic resonance imaging (CMR), the phenotype with pathology-frequent late gadolinium enhancement (LGE) was associated with a high risk of arrhythmic and heart failure events.

2. The absence of the pathology-frequent LGE phenotype was associated with a low-risk of arrhythmic events, even in the presence of LGE or abnormal left ventricular ejection fraction (LVEF).

Evidence Rating Level: 2 (Good)

Study Rundown: Cardiac sarcoidosis (CS) is an inflammatory condition characterized by impaired quality of life and high-risk of mortality. Suspected CS is frequently diagnosed and prognosticated using CMR. In studies of suspected CS investigated using CMR, cardiac damage identified as LGE has been associated with ventricular arrhythmic events. However, only a small fraction of patients with LGE have been found to experience these events. Hence, the objective of this study was to assess whether CMR phenotypes based on LVEF and LGE in patients with suspected CS are associated with adverse outcomes. A total of 504 patients with histologically proven sarcoidosis who underwent CMR for the evaluation of suspected CS between 2004 to 2020 were included in this study. The main outcomes were composite of ventricular arrhythmic and heart failure events. There were 4 distinct CMR phenotypes that were identified: normal LVEF and no LGE (n=290), abnormal LVEF and no LGE (n=53), pathology-frequent LGE (n=103), and pathology-rare LGE (n=58). The pathology-frequent LGE phenotype was found to be associated with a high risk of arrhythmic events independent of LVEF and extent of left ventricular late gadolinium enhancement (LVLGE) and was also associated with a high risk of heart failure. Both pathology-frequent LGE and LVLGE extent were independently associated with the arrhythmic endpoint, while only pathology-frequent LGE was independently associated with the heart failure endpoint. A limitation to this study was that cardiac monitoring of patients was not universally used during this study, and thus self-limiting ventricular arrhythmias could have been missed. A strength was that all patients identified for this study had a histologically-proven diagnosis of sarcoidosis.

Click to read the study in JAMA Cardiology

Relevant Reading: Presence of late gadolinium enhancement by cardiac magnetic resonance among patients with suspected cardiac sarcoidosis is associated with adverse cardiovascular prognosis

In-Depth [retrospective cohort]: A total of 504 patients with sarcoidosis (mean [SD] age, 54.1 years; 242 [48%] female and 262 [52%] male), who underwent CMR for the evaluation of suspected CS were included in this study. It assessed whether CMR phenotypes based on LVEF and LGE in patients with suspected CS were associated with adverse outcomes during follow-up. There were 4 distinct phenotypes identified: normal LVEF and no LGE (n=290; 57.5%), abnormal LVEF and no LGE (n=53; 10.5%), pathology-frequent LGE (n=103; 20.4%), and pathology-rare LGE (n=58; 11.5%). The pathology-frequent LGE phenotype was associated with a high risk of arrhythmic events (HR, 12.12; 95% CI, 3.62-40.57; P < .001) independent of LVEF and extent of left ventricular LGE. On Cox multivariable analyses, the same phenotype was also associated with a high risk of heart failure events (HR, 2.49; 95% CI, 1.19-5.22; P= .02). Among the patients with pathology-frequent LGE, a negative correlation was found between LVEF and left ventricular LGE extent with a Pearson correlation coefficient r of -0.58 (95% CI, -0.69 to -0.43; P < .001). At the median (IQR) follow-up period of 4.3 (2.4-6.9) years, 30 patients reached the arrhythmic end point. At the median (IQR) follow-up of 4.2 years, 60 patients reached the heart failure endpoint.

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