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%;

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.

Informations de copyright

© 2024 The Authors.

Déclaration de conflit d'intérêts

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.PerspectivesCOMPETENCY IN MEDICAL KNOWLEDGE: Hereditary ATTR-CM related to the Val122Ile genetic variant is a highly morbid cause of HF in Black individuals and leads to worse outcomes compared with wild-type ATTR-CM disease. Black patients from the most socioeconomically deprived areas had the poorest event-free survival compared with other groups. Among patients with ADI > 25, the hazard of HF hospitalization or mortality was significantly higher in Black compared with White patients, even when adjusting for disease severity at diagnosis. TRANSLATIONAL OUTLOOK: Black patients are underrepresented in contemporary ATTR-CM clinical trials. Future trial designs need to focus on increasing representation of Black patients as well as those with Val122Ile disease to identify effective therapies for patients at risk for worse outcomes to patients with wild-type disease. Moreover, a greater understanding of the more aggressive pathobiology of Val122Ile is needed in this vulnerable patient subset.