Fabio Fernandes et al. Caio Cafezeiro Renata Margarida do Val Alexandra Patrícia Zilli Vieira … Wilson Marques Edileide Barros Correia Alzira Alves Siqueira Carvalho Antonio Carlos Palandrini Chagas Acary Souza Bulle Oliveira Paulo Victor Sgobi de Souza Wladimir Bocca Vieira de Resende Pinto Ariane Vieira Scarlatelli Macedo Murillo Oliveira Antunes Pedro Vellosa Schwartzmann Sandrigo Mangini Marcus Vinicius Simões
Abstract
Background
Amyloidosis is a systemic disease that involves multiple organs, characterized by the deposition of amyloid fibrils. Knowledge regarding the epidemiological, clinical, and genetic profile of the population affected by amyloidosis throughout the country is of fundamental importance for establishing diagnostic and therapeutic strategies.
Objective
To evaluate the epidemiological, clinical, laboratory, imaging, and treatment variables of patients with TTR cardiac amyloidosis.
Methods
A multicenter, retrospective, prospective, and observational study based on collection of data on the natural history of patients with TTR amyloidosis, followed in the state of São Paulo.
Results
To make it possible to map the regional distribution of the disease, increasing knowledge about the disease among clinicians and specialists in different areas. To evaluate patients with hereditary and wild-type TTR amyloidosis, in addition to following individuals with positive genotype and negative phenotype.
Conclusion
The information collected may show greater awareness of the disease, development of new diagnostic and treatment flowcharts with a direct impact on knowledge of the natural history of the disease and patient prognosis.
