Right ventricular involvement and the extent of left ventricular enhancement with magnetic resonance predict adverse outcome in pulmonary sarcoidosis

Abstract

AimsCardiac involvement is the main determinant of poor outcomes in sarcoidosis. Right ventricular (RV) dysfunction and left ventricular (LV) late gadolinium enhancement (LGE) have been reported to be predictive of adverse outcome in non‐ischaemic cardiomyopathies. The aim of our study was to determine whether delayed RV LGE with cardiovascular magnetic resonance would be predictive of adverse events in addition to LV LGE during the long‐term follow‐up of pulmonary sarcoidosis patients.Methods and resultsEighty‐four consecutive biopsy‐proven pulmonary sarcoidosis patients were followed for a median of 56 months [38–74] after baseline delayed contrast‐enhanced cardiac magnetic resonance. The composite primary endpoint consisted of admission for congestive heart failure, sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator therapy, pacemaker implantation for high degree atrio‐ventricular block, or cardiac death. The composite secondary endpoint included all‐cause mortality in addition to the primary endpoint. RV and LV LGE were demonstrated in respectively 12 and 27 patients. Five of 10 events included in the primary endpoint occurred in the group with RV LGE. RV LGE, LV, or biventricular LGE yielded Cox hazard ratios of 8.71 [95% confidence interval (CI) 1.90–23.81], 9.22 (95% CI 1.96–43.45), and 12.09 (95% CI 3.43–42.68) for the composite primary endpoint. In a multivariate model, the predictive value of biventricular LGE for the composite primary and secondary endpoints was strongest. Kaplan–Meier event‐free survival curves were most significant for RV LGE and biventricular LGE (log rank with P < 0.001).ConclusionsBiventricular LGE at presentation is the strongest, independent predictor of adverse outcome during long‐term follow‐up. Asymptomatic myocardial scar <8% of LV mass carried a favourable long‐term outcome.

Cardiac Sarcoidosis

“…Multiple studies have demonstrated that late gadolinium enhancement is associated with increased all-cause mortality and increased ventricular arrhythmias when compared with those without LGE [7][8][9]. Quantity of LGE also appears to be related to the risk of future SCD, for example, in one study, absence of > 8% LGE of LV myocardium had a 95% negative predictive value [39]. The presence of FDG uptake and perfusion defects on PET is associated with higher risk of death or VT [12].…”

Cardiac Sarcoidosis

“…7). 84 Location may also be important; in 51 patients with CS and LVEF >35%, the presence of RV-delayed enhancement was associated with an increased risk of VT/VF or death. 85 Cardiac PET is also emerging as a modality that may help with risk stratification.…”

Current Diagnostic Techniques in Sarcoidosis

“…PET scan has also been evaluated in predicting supraventricular arrhythmias, and it was noted that patients with left atrial enlargement were associated with increased likelihood of supraventricular arrhythmias [11]. Smedema et al reported that biventricular late gadolinium enhancement was the strongest predictor of adverse outcome, and an asymptomatic myocardial scar of less than 8% in the left ventricular mass was associated with a favorable outcome in patients with pulmonary sarcoidosis [12].…”