Abstract 2400: Late Gadolinium Enhancement on MRI is a Prognostic Marker of Poor Survival in Light Chain Amyloidosis: Results from Long-Term Follow Up
Light chain amyloidosis (AL) is a rare disease associated with poor survival especially in the presence of cardiac involvement. Late gadolinium enhancement (LGE) on cardiac MRI was recently shown to correspond to cardiac amyloid deposition but the long term prognostic implication of this finding has not been established. The aim is to compare long-term survival between AL subjects with and without LGE. 25 biopsy-proven AL subjects (12 females, 61 ± 3 years) underwent cardiac MRI while undergoing workup for AL. Survival status was followed 24 ± 10 months post-MRI and compared between LGE positive and negative subjects. LGE was present in 19 (76%) AL subjects. 13 (52%) were in Heart Failure (HF) Class I with 7 (28%), 2 (8%) and 3 (12%) in Class II, III, IV respectively. LVEF was 65 ± 18% in LGE positive and 69 ± 12% LGE negative patients (p = 0.6). On follow up 10/19 LGE positive and 0/6 LGE negative patients died (log rank p = 0.03). LGE and initial presenting HF class (log rank p = 0.001) were associated with poor survival (see figure⇓). There was no significant association between LGE and initial presenting HF class (Fisher’s exact p = 0.4). This is the first long-term study demonstrating the prognostic significance of LGE in AL amyloidosis. Although small sample size precludes modeling the independent prognostic value of LGE, the lack of strong association with HF class and the presence of LGE in HF Class I subjects suggest additional utility of LGE in assessing the prognosis of AL subjects.