319 - Improvements in Cardiac Biomarkers are Associated with Better Health-Related Quality of Life in Patients with Light Chain Amyloidosis

Abstract

Introduction: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. B-type natriuretic peptide (BNP) or N-terminal proB-type natriuretic peptide (NT-proBNP) is used to monitor disease progression and response to treatment among patients with cardiac involvement from AL amyloidosis. The relationships between these cardiac biomarkers and health-related quality of life (HRQoL) are poorly understood in this condition. Hypothesis: Patterns of HRQoL in patients with cardiac AL amyloidosis will differ significantly by changes in BNP or NT-proBNP. Methods: We obtained cardiac biomarker and HRQoL data from patients with AL amyloidosis from two data sources. A subsample of patients with cardiac involvement was drawn from a community-based study of AL amyloidosis patients (n = 108). HRQoL scores (based on the SF-36® Health Survey [SF-36] and the Kansas City Cardiomyopathy Short Form [KCCQ-12]) were examined by patients with and without a history of ≥30% decrease in NT-proBNP. Bivariate analyses were conducted using analysis of variance. HRQoL scores were reported relative to existing benchmarks, including: 1) mean SF-36 scores from both a general population sample and patients with congestive heart failure (CHF); and 2) mean KCCQ scores according to New York Heart Association (NYHA) functional classes. Analysis of variance was used to compare SF-36 scores to age- and sex- adjusted benchmarks. Data were obtained from a subsample of patients with AL amyloidosis seen at the Amyloidosis Center at Boston University between 1994 and 2014 who had pre- and post- assessments (n = 95). Associations between changes in HRQoL and changes in BNP were assessed longitudinally with generalized linear models that controlled for baseline HRQoL scores. Results: In the community-based sample, HRQoL scores differed significantly by NT-proBNP response for all SF-36 and KCCQ scales and summary scores (P < .05 for all) (Fig. 1). Patients without a NT-proBNP response had SF-36 scores that were comparable to CHF benchmarks. Longitudinal analyses based on BNP further supported associations between changes in cardiac biomarkers and changes in HRQoL. Conclusions: Although these cardiac biomarkers have been clinically validated as surrogate treatment measures for survival in AL amyloidosis, these data further support that they may also be useful surrogates for clinical measures such as HRQoL and NYHA status.