Features and evolution of patients with systemic amyloidosis and cardiac involvement




Marcelina Carretero, Servicio de Clínica Médica, Área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, BA, Argentina
María A. Aguirre, Servicio de Clínica, Área de Investigación en Medicina Interna, Instituto de Medicina Traslacional e Ingeniería Biomédica, CONICET, Instituto Universitario del Hospital Italiano, Ciudad Autónoma de Buenos Aires, Argentina
Eugenia Villanueva, Servicio de Cardiología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Elsa M. Nucifora, Servicio de Hematología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Mª Lourdes Posadas-Martínez, Servicio de Clínica Médica, Área de Investigación en Medicina Interna; Departamento de Investigación, Instituto de Medicina Traslacional e Ingeniería Biomédica, CONICET-Instituto Universitario del Hospital Italiano. Hospital Italiano de Buenos Aires, BA, Argentina


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 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. 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 person- 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.



Keywords: Amyloidosis. Immunoglobulin light chain amyloidosis. Familial amyloidosis. Restrictive cardiomyopathy.