| Autor: |
Villanueva E; Servicio de Cardiología, Hospital Italiano de Buenos Aires, Argentina. E-mail: eugenia.villanueva@hospitalitaliano.org.ar., Carretero M; Servicio de Clínica Médica, área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, Argentina., Aguirre MA; Servicio de Clínica Médica, Hospital Italiano de Buenos Aires, Argentina., Negro A; Servicio de Clínica Médica, área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, Argentina., Belziti CA; Servicio de Cardiología, Hospital Austral, Buenos Aires, Argentina., Posadas-Martínez ML; Instituto Universitario del Hospital Italiano de Buenos Aires-CONICET, Buenos Aires, Argentina., Nucifora EM; Servicio de Hematología, Hospital Italiano Buenos Aires, Argentina., Baratta S; Servicio de Cardiología, Hospital Austral, Buenos Aires, Argentina., Costabel JP; Servicio de Cardiología, Instituto Cardiovascular Buenos Aires, Argentina., Higa C; Servicio de Cardiología, Hospital Alemán, Buenos Aires, Argentina., Rivas C; Servicio de Cardiología, Hospital Alemán, Buenos Aires, Argentina., Fernández A; Servicio de Cardiología, Fundación Favaloro, Buenos Aires, Argentina., Quiroga A; Servicio de Cardiología, Fundación Favaloro, Buenos Aires, Argentina., Dumont CA; Hospital Privado de Rosario, Grupo Gamma, Santa Fe, Argentina., Volberg VI; Servicio de Cardiología, Hospital de Clínicas José de San Martin, Buenos Aires, Argentina., Streitenberg GM; Servicio de Cardiología, Hospital El Cruce, Buenos Aires, Argentina., Perez de Arenaza D; Servicio de Cardiología, Hospital Italiano de Buenos Aires, Argentina. |
| Abstrakt: |
This clinical practice guideline for treating transthyretin amyloid (ATTR) cardiomyopathy is based on the best available evidence of clinical effectiveness. The PICO format was used to generate a list of questions focused on the effectiveness and safety of the specific treatment of patients with ATTR cardiomyopathy. The search was conducted in PubMed, Cochrane and Epistemokus, between July-August 2020, and selected articles between 2000-2020, in English and Spanish. The level of evidence and recommendations were analyzed and classified by the GRADE system. The following drugs were included in the analysis: tafamidis, diflunisal, inotersen, patisiran y doxycycline and ursodeoxycholic acid. The expert panel had an agreement that tafamidis 80mg/daily is the only available drug with moderate evidence and weak recommendation for the reduction of total mortality, cardiovascular morbidity, heart failure hospitalization and progression of the disease in patients with ATTR cardiomyopathy and NYHA class = 3. In contrast, tafamidis 20 mg/daily had low-quality evidence in this group of patients. The expert panel did not recommend inotersen, patisiran and diflunisal in patients with ATTR cardiomyopathy due to the lack of supporting evidence, local drug availability, and the potential risk of toxicity. When patients did not have access to tafamidis, the expert panel stated a weak recommendation to use doxycycline and ursodeoxycholic acid in patients with ATTR cardiomyopathy. |
