Características y evolución de los pacientes con amiloidosis sistémica y compromiso cardíaco

Abstract

Objective: 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 amyloi-dosis 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. All deaths due to 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 damage, the death rate was 14/1,000 per-son-months, and for patients without damage, the death rate was 5/1,000 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). The original HR for heart involvement was 2.09 (p = 0.02). Age showed that HRa was 1.06 (p <0.01). The sub-HR estimated by the competitive risk regression model are 1.86 (95% CI 0.99-3.49) p = 0.05. Conclusion: Cardiac involvement is an important prognostic factor in patients with amyloidosis.