Amiloidosis cardiaca: reporte de un caso

Abstract

Cardiac amyloidosis is a rare clinical entity and, therefore, almost unknown. It shows as a restrictive cardiomyopathy, since it is an infiltrative disease caused by the extracellular deposition of amyloids in the cardiac wall. Although it may be systemic, the heart condition determines its poor prognosis. This is the case of a 38-year-old patient who was admitted for dyspnea and severe fatigue. He was diagnosed with IgA multiple myeloma, lambda light chains, clinical stage IIIB, and initiated treatment with cyclophosphamide, thalidomide and dexamethasone. The patient stopped the treatment, and returned after one year and 2 months with an advanced disease and presence of amyloidosis. The patient progressed torpidly and eventually died. A review of the medical literature is performed concerning amyloidosis types, physiopathology, diagnostic methods and treatment.

Contracciones ventriculares prematuras: caso clínico

“…El paciente abandonó el tratamiento y regresó luego de un año y 2 meses con la enfermedad avanzada y con presencia de amiloidosis. El paciente evoluciona tórpidamente y fallece (24) . A su vez Eris y Pac presentaron un caso clínico de un niño asintomático de 10 años de edad que fue remitido al servicio de cardiología pediátrica por su médico de cabecera para un examen cardíaco antes de participar en deportes competitivos.…”

Enhanced quantitative method for the diagnosis of grade 1 cardiac amyloidosis in 99mTc-DPD scintigraphy

“…Cardiac amyloidosis (CA), considered until recently a rare and underdiagnosed disease, has been a known entity for more than 300 years; with an approximate incidence of 3 to 5 patients per million inhabitants per year and an average age of onset of around 65 years 1 , 2 . The term amyloidosis involves a heterogeneous group of subtypes (hereditary or acquired, localized or systemic) 2 , characterized by extracellular deposits of insoluble abnormal fibrils, which receive the pathophysiological name of amyloid deposits 3 .…”

“…The term amyloidosis involves a heterogeneous group of subtypes (hereditary or acquired, localized or systemic) 2 , characterized by extracellular deposits of insoluble abnormal fibrils, which receive the pathophysiological name of amyloid deposits 3 . It can affect multiple organs, being the heart the second most affected in frequency (50–60%) 1 .…”

“…From the thirty known amyloid proteins, only a few have cardiac involvement, being the transthyretin (TTR) and the light chain (LC), the most frequently related to the heart 2 , 3 . Cardiac amyloidosis due to LC deposits is generally associated with lymphoproliferative disorders 1 and has been considered to date the most prevalent disease form in developed countries. However, due to non-invasive diagnostic tests and the progressive aging of the population, amyloidosis due to TTR deposits (TTR) has become the most frequent 2 .…”