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Abstract 15242: Increased Mortality Among African American Patients With Heart Failure Caused by Hereditary Transthyretin Amyloid Cardiomyopathy


 Introduction:
 Hereditary transthyretin amyloid cardiomyopathy (pV142I hATTR-CM) is an under-recognized cause of heart failure (HF) in elderly patients of African origin. While we have previously demonstrated that the endogenous TTR ligand retinol binding protein 4 (RBP4) identifies patients with hATTR-CM, its capacity to predict outcomes is unexplored. Further, comparative data are lacking describing clinical outcomes in hATTR-CM vs. matched patients with HF.
 
 
 Hypothesis:
 Reduced survival is associated with pV142I hATTR-CM when compared to an age, race, sex matched patients with HF.
 
 
 Methods:
 Retrospective, single center, cohort study of self-reported African American patients ≥ 60 years of age with normal
 TTR
 genotype, HF, and LV wall thickness ≥ 12mm (n=84) were compared to a cohort with biopsy-proven pV142I hATTR-CM (n=30). Patients did not receive ATTR-specific therapies, as study occurred prior to FDA approvals. Baseline laboratory values were measured. All-cause mortality was estimated and compared between groups based on a median followup of 45.4 months. Analyses were conducted with Fisher Exact, Kruskal Wallis, and Kaplan-Meier with log-rank testing.
 
 
 Results:
 hATTR-CM patients were slightly older but less likely to have concomitant HTN or DM (Table 1). Baseline RBP4 concentration was lower while troponin level higher in patients with hATTR-CM (Table 1). hATTR-CM patients had a higher hazard of all-cause mortality after adjusting for age (HR=3.5, 95% CI: 1.8 – 6.7, p<0.001). Baseline RBP4 was not a significant predictor of mortality (p-value=0.195).
 
 
 Conclusions:
 Patients pV142I hATTR-CM demonstrate significantly reduced survival (a 3.5-fold increased risk of death, 43% 4-year survival) compared to control subjects with HF in the absence of amyloidosis. Baseline RBP4 concentration did not associate with survival. These data suggest that hATTR-CM recognition is essential to permit implementation of available therapies that can improve survival.
 
 
 
