Heart Failure Resulting From Age-Related Cardiac Amyloid Disease Associated With Wild-Type Transthyretin

Background—Heart failure caused by wild-type transthyretin amyloidosis (ATTRwt) is an underappreciated cause of morbidity and mortality in the aging population. The aims of this study were to examine features of disease and to characterize outcomes in a large ATTRwt cohort. Methods and Results—Over 20 years, 121 patients with ATTRwt were enrolled in a prospective, observational study. Median age at enrollment was 75.6 years (range, 62.6–87.8 years); 97% of patients were white. The median survival, measured from biopsy diagnosis, was 46.69 months (95% confidence interval, 41.95–56.77); 78% of deaths were attributable to cardiac causes. By Kaplan–Meier analysis, 5-year survival was 35.7% (95% confidence interval, 25–46). Impaired functional capacity (mean Vo2max, 13.5 mL·kg−1·min−1) and atrial fibrillation (67%) were common clinical features. Multivariate predictors of reduced survival were elevated serum brain natriuretic peptide (482±337 pg/mL) and uric acid (8.2±2.6 mg/dL), decreased left ventricular eje...