Race and Socioeconomic Status Impact Diagnosis and Clinical Outcomes in Transthyretin Cardiac Amyloidosis

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with significant mortality. The Val122Ile variant, highly prevalent in Black patients, portends poorer survival compared with other ATTR-CM subtypes. Although Val122Ile is biologically more aggressive, the contribution of race and socioeconomic status (SES) to disease outcomes in patients with ATTR-CM is undefined. Objectives: The aim of this study was to evaluate the impact of race and SES on clinical outcomes in patients with ATTR-CM. Methods: Patients with ATTR-CM who received care at Johns Hopkins Hospital between 2006 and 2022 were included. SES was assessed using area deprivation index (ADI). Associations of race and ADI with heart failure (HF) hospitalization and/or death were measured using multivariable logistic or Cox proportional hazards models. Results: Of 282 patients, 225 (80%) were men, and 129 (46%) were Black. Black vs White patients disproportionately constituted the highest ADI (most deprived) category (66% vs 28%; P = 0.004), and Black patients were more likely to have HF hospitalization or death over 5 years compared with White patients (log-rank P < 0.001). Among those with ADI >25, Black patients had a significantly greater hazard of HF hospitalization or death compared with White patients, independent of disease stage at diagnosis (HR: 2.77; 95% CI: 1.45-5.32; P = 0.002). Conclusions: Black patients with low SES may be at greater risk for underdiagnosis and adverse outcomes compared with White patients. Ongoing efforts are needed to improve outcomes in this subset of patients with ATTR-CM.