OBJECTIVES: This study sought to determine the prognostic value of cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) in amyloid light chain (AL) cardiac amyloidosis. Cardiac involvement is the major determinant of mortality in AL amyloidosis. CMR LGE is a marker of amyloid infiltration of the myocardium. The purpose of this study was to evaluate retrospectively the prognostic value of CMR LGE for determining all-cause mortality in AL amyloidosis and to compare the prognostic power with the biomarker stage.
METHODS: Seventy-six patients with histologically proven AL amyloidosis underwent CMR LGE imaging. LGE was categorized as global, focal patchy, or none. Global LGE was considered present if it was visualized on LGE images or if the myocardium nulled before the blood pool on a cine multiple inversion time (TI) sequence. CMR morphologic and functional evaluation, echocardiographic diastolic evaluation, and cardiac biomarker staging were also performed. Subjectsâ€™ charts were reviewed for all-cause mortality. Cox proportional hazards analysis was used to evaluate survival in univariate and multivariate analysis.
RESULTS: There were 40 deaths, and the median study follow-up period was 34.4 months. Global LGE was associated with all-cause mortality in univariate analysis (hazard ratio = 2.93; p < 0.001). In multivariate modeling with biomarker stage, global LGE remained prognostic (hazard ratio = 2.43; p = 0.01).
CONCLUSIONS: Diffuse LGE provides incremental prognosis over cardiac biomarker stage in patients with AL cardiac amyloidosis.
COMPETENCY IN MEDICAL KNOWLEDGE: Among patients with systemic AL amyloidosis, global LGE in the myocardium predicts all-cause mortality incrementally over biomarker stage.
TRANSLATIONAL OUTLOOK: Many variables are prognostic in patients with AL amyloidosis. Future studies will be needed to determine the optimal prognostic strategy in these patients.