Features and evolution of patients with systemic amyloidosis and cardiac involvement

Abstract

Objective:The study aims to estimate the prevalence of cardiac amyloidosis in patients with systemic amyloidosis. Compare survival rates based on whether they show cardiac involvement. Methods: A retrospective cohort study of patients with systemic amyloidosis from the Institutional Amyloidosis Registry of the Hospital Italian of Buenos Aires from 2010 to 2019. Heart involvement is considered to be the presence of symptoms and/or images consistent with amyloidosis, and there is no other reason to explain it. Death for all causes were evaluated. The survival rate was estimated by Kaplan-Meier. Cox regression model was used to evaluate factors related to mortality. Heart transplantation was evaluated in a competitive risk regression model. Results: The prevalence of heart involvement is 63%. For the group with heart involvement, the death rate was 14/1000 person-months, and for patients without damage, the death rate was 5/1000 person-months. The 5-year overall survival rate for patients with heart involvement was 44%, while that for patients without damage was 67% (p = 0.02). Crude hazard ratio (HR) for heart involvement was 2.09 (p= 0.02). Age showed an adjusted HR of 1.06 (p <0.01). The sub-HR estimated by the competitive risk regression model was 1.86 (95% confidence intervals 0.99-3.49) p = 0.05. Conclusion: Cardiac involvement is an important prognostic factor in patients with amyloidosis.

The role of registries in improving health and bridging healthcare, research, education, innovation and development: a research department perspective

“…The network has published articles, won awards at conferences, taught courses and/ or seminars in Latin American countries, presented work at congresses and symposia, developed clinical practice guidelines on the diagnosis and treatment of amyloidosis in our region and published related articles on different aspects of amyloidosis, including documentation of the existence and magnitude of the disease, [34][35][36] the characteristics of the disease, [37][38][39] the understanding of the patterns of diagnosis and treatment, 40,41 and the development of strategies that seek to resolve or mitigate these problems and/or monitor their evolution. [42][43][44][45][46][47] The To address current gaps in knowledge, innovation and development are being conducted on diagnostic methods to identify the amyloid protein with local resources.…”