Left and right ventricular myocardial deformation and late gadolinium enhancement: incremental prognostic value in amyloid light-chain amyloidosis.

Background: Previous cardiac magnetic resonance (CMR) studies have shown that both late gadolinium enhancement (LGE) and left ventricular (LV) strain have prognostic value in amyloid light-chain (AL) amyloidosis, but the right ventricular (RV) strain has not yet been studied. We aim to determine the incremental prognostic value of LV and RV LGE and strain in AL amyloidosis. Methods: This prospective study recruited 87 patients (age, 56.9 +/- 9.1 years; M/F, 56/31) and 20 healthy subjects (age, 52.7 +/- 8.1 years; M/F, 11/9) who underwent CMR. The LV LGE was classified into no, patchy and global groups. The RV LGE was classified into negative and positive groups. Myocardial deformation was measured using a dedicated software. Follow-up was performed for all-cause mortality using Cox proportional hazards regression and Kaplan-Meier curves. Results: During a median follow-up of 21 months, 34 deaths occurred. Presence of LV LGE [HR 2.44, 95% confidence interval (CI), 1.10-5.45, P=0.029] and global longitudinal strain (GLS) (HR 1.13 per 1% absolute decrease, 95% CI, 1.02-1.25, P=0.025) were independent LV predictors. RV LGE (HR 4.07, 95% CI, 1.09-15.24, P=0.037) and GLS (HR 1.10 per 1% absolute decrease, 95% CI, 1.00-1.21, P=0.047) were independent RV predictors. Complementary to LV LGE, LV GLS impairment or RV LGE further reduced survival (both log rank P<0.001). Conclusions: This study confirms the incremental prognostic value of LV GLS and RV LGE in AL amyloidosis, which refines the conventional risk evaluation based on LV LGE. GLS based on non-contrast-enhanced CMR are promising new predictors.