<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1409-4142</journal-id>
<journal-title><![CDATA[Revista Costarricense de Cardiología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. costarric. cardiol]]></abbrev-journal-title>
<issn>1409-4142</issn>
<publisher>
<publisher-name><![CDATA[Asociación Costarricense de Cardiología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1409-41422024000100016</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Cardiomiopatía Amiloide por Transtiretina: Análisis del Rol Terapéutico del Tafamidis]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Zavaleta-Monestel]]></surname>
<given-names><![CDATA[Esteban]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Quesada-Villaseñor]]></surname>
<given-names><![CDATA[Ricardo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[León-Obando]]></surname>
<given-names><![CDATA[Alexa]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Arguedas-Chacón]]></surname>
<given-names><![CDATA[Sebastián]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Anchía-Alfaro]]></surname>
<given-names><![CDATA[Adriana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García-Montero]]></surname>
<given-names><![CDATA[Jonathan]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Salas-Segura]]></surname>
<given-names><![CDATA[Juliana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gutiérrez Jaikel]]></surname>
<given-names><![CDATA[Luis Abel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Clínica Bíblica  ]]></institution>
<addr-line><![CDATA[ San José]]></addr-line>
<country>CRI</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Clínica Bíblica Departamento de Investigación ]]></institution>
<addr-line><![CDATA[ San José]]></addr-line>
<country>CRI</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad de Iberoamérica Facultad de Farmacia ]]></institution>
<addr-line><![CDATA[ San José]]></addr-line>
<country>CRI</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital Clínica Bíblica Departamento de Cardiología ]]></institution>
<addr-line><![CDATA[ San José]]></addr-line>
<country>CRI</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<volume>26</volume>
<numero>1</numero>
<fpage>16</fpage>
<lpage>24</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_arttext&amp;pid=S1409-41422024000100016&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_abstract&amp;pid=S1409-41422024000100016&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_pdf&amp;pid=S1409-41422024000100016&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN Introducción y objetivo: El tratamiento de la amiloidosis cardíaca por transtiretina (TTR) se basa en dos estrategias fundamentales: la terapia de apoyo y la terapia modificadora de la enfermedad. La terapia de apoyo tiene como objetivo prevenir y controlar las complicaciones de la enfermedad, brindando soporte a los órganos afectados y mejorando la calidad de vida del paciente. Por otro lado, la terapia modificadora de la enfermedad se enfoca en interrumpir o ralentizar la producción y acumulación de las fibrillas amiloides, responsables del daño tisular. Esta terapia emplea medicamentos que actúan en diferentes etapas de la amiloidogénesis: silenciamiento del gen TTR para reducir la producción de la proteína, estabilización de la TTR circulante para impedir su disociación y formación de monómeros, y destrucción y reabsorción de depósitos amiloides ya formados. Se evaluarán rigurosamente sus beneficios, eficacia y seguridad como tratamiento para la amiloidosis cardíaca. Métodos: Se realizó una revisión de la literatura de estudios publicados entre 2013 y 2024 en bases de datos electrónicas como PubMed, Google Scholar, ELSEVIER, UpToDate y ClinicalKey, con el objetivo principal de evaluar la eficacia y relevancia clínica de tafamidis en la amiloidosis cardíaca. Resultados: Los estudios clínicos han demostrado que tafamidis meglumina es un medicamento seguro y eficaz para el tratamiento de la ATTR por mutación V30M y ATTR-CM. El medicamento ha sido asociado con una reducción en la tasa de hospitalizaciones por causas cardiovasculares, una mejoría de la función cardíaca y una mejor calidad de vida en los pacientes. Conclusiones: Tafamidis ha demostrado ser una terapia eficaz y segura para la amiloidosis cardíaca por transtiretina, mejorando significativamente la supervivencia y calidad de vida de los pacientes. Aunque enfrenta desafíos de accesibilidad debido a su costo, se posiciona como un pilar en el tratamiento de esta enfermedad. La investigación continua es crucial para optimizar su uso y desarrollar terapias complementarias.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT Introduction and Objective: The treatment of transthyretin (TTR) cardiac amyloidosis is based on two fundamental strategies: supportive therapy and disease-modifying therapy. Supportive therapy aims to prevent and control disease complications, providing support to affected organs and improving patient quality of life. In contrast, disease-modifying therapy focuses on interrupting or slowing the production and accumulation of amyloid fibrils responsible for tissue damage. This therapy employs drugs that act at different stages of amyloidogenesis: silencing the TTR gene to reduce protein production, stabilizing circulating TTR to prevent its dissociation and monomer formation, and destroying and reabsorbing already formed amyloid deposits. The benefits, efficacy, and safety of these therapies as treatments for cardiac amyloidosis will be rigorously evaluated. Methods: An literature review of studies published between 2013 and 2024 was conducted in electronic databases such as PubMed, Google Scholar, ELSEVIER, UpToDate, and ClinicalKey, with the primary objective of evaluating the efficacy and clinical relevance of fafamidis in cardiac amyloidosis. Results: Clinical trials have shown that tafamidis meglumine is a safe and effective medication for the treatment of ATTRv30M and ATTR-CM. The drug has been associated with a reduction in the rate of cardiovascular-related hospitalizations, improved cardiac function, and better quality of life in patients. Conclusions: Tafamidis has proven to be an effective and safe therapy for transthyretin cardiac amyloidosis, significantly improving patient survival and quality of life. Although it faces accessibility challenges due to its cost, it stands as a cornerstone in the treatment of this disease. Continuous research is crucial to optimize its use and develop complementary therapies.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Amiloidosis cardiaca]]></kwd>
<kwd lng="es"><![CDATA[Tafamidis]]></kwd>
<kwd lng="es"><![CDATA[tetramérica]]></kwd>
<kwd lng="es"><![CDATA[monómeros]]></kwd>
<kwd lng="es"><![CDATA[estabilizador de transtiretina]]></kwd>
<kwd lng="es"><![CDATA[mutación V30M.]]></kwd>
<kwd lng="en"><![CDATA[Cardiac amyloidosis]]></kwd>
<kwd lng="en"><![CDATA[Tafamidis]]></kwd>
<kwd lng="en"><![CDATA[Tetramer]]></kwd>
<kwd lng="en"><![CDATA[Monomers]]></kwd>
<kwd lng="en"><![CDATA[Transthyretin stabilizer]]></kwd>
<kwd lng="en"><![CDATA[V30M mutation.]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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