Prognostic implication of late gadolinium enhancement on cardiac MRI in light chain (AL) amyloidosis on long term follow up

Twenty nine consecutive patients (14 females; 62 ± 11 years) with biopsy-proven AL undergoing cardiac MRI with gadolinium as part of AL workup were included. Survival was prospectively followed 29 months (median) following MRI and compared between those with and without LGE by Kaplan-Meier and log-rank analyses.LGE was positive in 23 subjects (79%) and negative in 6 (21%). Left ventricular ejection fraction was 66 ± 17% in LGE-positive and 69 ± 12% in LGE-negative patients (p = 0.8). Overall 1-year mortality was 36%. On follow-up, 14/23 LGE-positive and none of LGE-negative patients died (log rank p = 0.0061). Presenting New York Heart Association heart failure class was also associated with poor survival (p = 0.0059). Survival between two LGE groups stratified by heart failure class still showed a significant difference by a stratified log-rank test (p = 0.04).Late gadolinium enhancement is common and is associated with poor long-term survival in light chain amyloidosis, even after adjustment for heart failure class presentation. The prognostic significance of late gadolinium enhancement in this disease may be useful in patient risk-stratification.Light chain or primary amyloidosis (AL) is a plasma cell dyscrasia with production and abnormal deposition of insoluble fibrillar proteins derived from immunoglobulin light chains [1,2]. It is a rare disease but can be fatal. Amyloid protein deposition in various organs such as the heart, kidneys, gut and nervous system cause multi-organ dysfunction. Cardiac involvement was reported in 50% of cases [3] and is associated with the worst prognosis; median survival is about 4 months in the setting of advanced heart failure [4,5]. Amyloid deposition in the heart is confirmed by invasive endomyocardial biopsy. Noninvasive imaging demonstrate that some AL patients have diffuse subendocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (MRI) [6]. Late gadolinium enhancement was found to be highly corre