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Evolución a mediano plazo de pacientes con diagnóstico de cardiopatía amiloidótica por transtiretina;
A Prospective Multi-Center Registry of Patients Hospitalized for Non-ST Segment Elevation Acute Coronary Syndrome in High Complexity Centers. In-hospital and 6-month Outcomes (Buenos Aires I)

Creators:Costabel, Juan Pablo, Lorenzo, Cristina, Mondragón, Ignacio, Talavera, Luján, Meretta, Alejandro, Mitrione, Soledad, Daquarti, Gustavo, Talavera, Luján, Vrancic, Mariano, Diez, Mirta

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Descripción

Introducción: El compromiso cardíaco es la principal causa de morbimortalidad en la amiloidosis, independientemente de lapatogenia productora del amiloide subyacente. La amiloidosis por transtiretina (TTR) es una de las variantes más frecuentes,por lo cual el objetivo de este trabajo fue evaluar las características de una cohorte de pacientes con diagnóstico de cardiopatíaamiloidótica por TTR (CA-TTR)Material y métodos: Se recabaron datos de los estudios basales, de la metodología diagnóstica y de la evolución de 49 pacientesen seguimiento en la Clínica de Miocardiopatías de nuestra institución.Resultados: La mediana del tiempo de seguimiento fue de 1258 días (410-2004) y la edad promedio de 79 ± 9 años. Al iniciodel seguimiento, el 57% de los pacientes estaban en clase funcional I, el 26%, en II y el 16%, en III-IV. El diagnóstico se basóen centellograma con difosfonatos en el 92%; en el 24% requirió biopsia. La mortalidad global fue del 19%, con 15% de muertecardiovascular. La tasa de internación por insuficiencia cardíaca fue 29%; el 63% de los pacientes empeoró su clase funcional.Conclusiones: El seguimiento de nuestros pacientes con CA-TTR expresa los cambios que ha sufrido el proceso diagnóstico,con una reducción de estudios invasivos y tiempo para la caracterización. El diagnóstico de pacientes en etapas “tempranas”de la enfermedad parece impactar en los resultados a mediano plazo.

Background: Cardiac involvement is the main cause of morbidity and mortality in amyloidosis, regardless of the underlyingpathogenesis of amyloid production, and transthyretin (TTR) amyloidosis is one of the most frequent variants.Objective: The aim of this study was thus to assess the characteristics of a cohort of patients with diagnosis of TTR cardiacamyloidosis (ATTR-CA).Methods: Baseline data and diagnostic and follow-up methodology were collected from 49 patients treated at the cardiomyopathyclinic of our institution.Results: Median follow-up was 1,258 days (410-2004). Mean age was 79±9 years, and 57% of patients were in functional class(FC) I, 26% in FC II and 16% in FC III-IV at follow-up onset. Diagnosis was made with diphosphonate scintigraphy in 92% ofpatients and 24% required a biopsy. Overall mortality was 19%, with 15% of cardiovascular death. The rate of hospitalizationfor heart failure was 29% and 63% of patients worsened their FC.Conclusions: Follow-up of patients with ATTR-CA expresses the changes undergone by the diagnostic process, with a reductionof invasive studies and time to characterization. The diagnosis of patients at “early stages of the disease” seems to havean impact on mid-term outcomes.

Metadatos destacados

Colecciones
Argentine Journal of Cardiology

Editor

Sociedad Argentina de Cardiología

Fuente

Revista Argentina de Cardiología; Vol 88, No 5 (2020); 423-428, Argentine Journal of Cardiology; Vol 88, No 5 (2020); 423-428

Citación

Costabel, Juan Pablo et al., “Evolución a mediano plazo de pacientes con diagnóstico de cardiopatía amiloidótica por transtiretina,” Archivo PPCT, consulta 1 de abril de 2026, http://archivoppct.caicyt.gov.ar/items/show/9729.

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Autor

Costabel, Juan Pablo
Lorenzo, Cristina
Mondragón, Ignacio
Talavera, Luján
Meretta, Alejandro
Mitrione, Soledad
Daquarti, Gustavo
Talavera, Luján
Vrancic, Mariano
Diez, Mirta

Fuente

Revista Argentina de Cardiología; Vol 88, No 5 (2020); 423-428
Argentine Journal of Cardiology; Vol 88, No 5 (2020); 423-428

Editor

Sociedad Argentina de Cardiología

Fecha

2020-11-27

Derechos

Los que firman al pié, certificamos que tenemos total responsabilidad por la conducción de este estudio y por el diseño y la interpretación de los datos. Nosotros escribimos el manuscrito y somos responsables por la decisión acerca del mismo. Cada uno de nosotros cumple la definición de autor como se afirma en el Comité Internacional de Editores de Revistas Médicas (International Committee of Medical Journal Editors, ver www.icmje.org). Nosotros hemos visto y aprobado el manuscrito final. Ni el artículo, ni ninguna parte esencial del mismo, incluido las tablas y las figuras, será publicado o admitido para arbitrar a otra parte antes de aparecer en la Revista.También notificamos haber leído la sección “conflicto de intereses”, y revelaríamos cualquiera que existiera. Dejamos constancia que si nuestro artículo se publicara en la RAC, cederíamos los derechos (copyright) a la Revista.Los documentos publicados en esta revista están bajo la licencia Creative Commons Atribución-NoComercial-Compartir-Igual 2.5 Argentina.
Those signing below certify that we have full responsibility for the conduction of this study and for the design and interpretation of the information. We wrote the manuscript and are responsible for its decision. Each of us fulfills the definition of authorship as stated by the International Committee of Medical Journal Editors ( www.icmje.org). We have signed and approved the final manuscript. Neither the manuscript, nor any essential part thereof, including tables and figures, will be published or accepted for refereeing elsewhere before being published in the Journal. We have also read the "Conflict of Interest" section and would disclose any existing. We state that if our manuscript is published in the RAC, we shall transfer the copyright to the Journal.

Idioma

eng
spa

Tipo

info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion