<?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>1683-9803</journal-id>
<journal-title><![CDATA[Pediatría (Asunción)]]></journal-title>
<abbrev-journal-title><![CDATA[Pediatr. (Asunción)]]></abbrev-journal-title>
<issn>1683-9803</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Paraguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1683-98032023000200120</article-id>
<article-id pub-id-type="doi">10.31698/ped.50022023008</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Miocardiopatía hipertrófica en la edad pediátrica. Revisión a propósito de una serie de casos]]></article-title>
<article-title xml:lang="en"><![CDATA[Hypertrophic cardiomyopathy in the pediatric population. Review of a series of cases]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rohden Both]]></surname>
<given-names><![CDATA[Franciele Regiane]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
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<contrib contrib-type="author">
<name>
<surname><![CDATA[Chaparro Garay]]></surname>
<given-names><![CDATA[Gabriela Alexandra]]></given-names>
</name>
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</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Álvarez Núñez]]></surname>
<given-names><![CDATA[Soledad de los Ángeles]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Verón Coronel]]></surname>
<given-names><![CDATA[Carlos Miguel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Garay Echeverría]]></surname>
<given-names><![CDATA[Nancy Mercedes]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital General Pediátrico &#8220;Niños de Acosta Ñu&#8221;  ]]></institution>
<addr-line><![CDATA[San Lorenzo ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2023</year>
</pub-date>
<volume>50</volume>
<numero>2</numero>
<fpage>120</fpage>
<lpage>133</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S1683-98032023000200120&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S1683-98032023000200120&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S1683-98032023000200120&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  La miocardiopatía hipertrófica (MCH) constituye una enfermedad compleja y variable en cuanto a morfología, fisiopatología, pronóstico y sobrevida. Se caracteriza por una hipertrofia del ventrículo izquierdo, aunque en ocasiones puede ser biventricular o ventricular derecha aislada. En la edad pediátrica predominan las causas no sarcoméricas. La ecocardiografía es el método diagnóstico más utilizado para caracterizar la morfología y repercusión hemodinámica, sin embargo, la resonancia magnética cardíaca (RMC) es de elección por aportar mayores ventajas morfológicas y funcionales. Las pruebas genéticas son de gran importancia en la determinación del diagnóstico etiológico, manejo terapéutico y pronóstico. El tratamiento tiene como objetivo principal mejorar la capacidad funcional y aliviar los síntomas. Los betabloqueantes no vasodilatadores son considerados de primera líneay en algunos casos se puede recurrir a terapias avanzadas como disopiramida o terapia de reducción septal (TRS), uso del cardiodesfibrilador implantable (CDI), y en ocasiones se debe considerar trasplante cardiaco en pacientes con síntomas refractarios al tratamiento médico y en MCH del tipo obstructivo.  Materiales y métodos: Estudio observacional, descriptivo, corte transversal.  Resultados: Se estudiaron 12 pacientes, desde enero de 2020 a diciembre de 2022, con edades comprendidas desde 5 meses hasta 16 años, diagnosticados con MCH de presentación clínica variable y de distintas etiologías.  Conclusión:  La MCH de causas no sarcoméricas como los síndromes malformativos y errores innatos del metabolismo son las que tienen peor pronóstico y sobrevida. La finalidad de detectar precozmente la MCH es el inicio temprano de la terapia específica, para así retrasar el compromiso cardiovascular y mejorar la sobrevida.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction: Hypertrophic cardiomyopathy (HCM) is a complex and variable disease in terms of morphology, pathophysiology, prognosis and survival. It is characterized by left ventricular hypertrophy, although it can sometimes be biventricular or isolated right ventricular. In the pediatric population, non-sarcomeric causes predominate. Echocardiography is the most widely used diagnostic method to characterize morphology and hemodynamic repercussions; however, cardiac magnetic resonance imaging (CMR) is preferred because it provides greater morphological and functional advantages. Genetic tests are of great importance in determining the etiological diagnosis, therapeutic management and prognosis. The main objective of the treatment is to improve functional capacity and alleviate symptoms. Non-vasodilator beta-blockers are considered first-line treatment and in some cases advanced therapies such as disopyramide or septal reduction therapy (SRT), use of implantable cardioverter-defibrillator (ICD) can be used; heart transplantation should be considered in patients with symptoms refractory to medical treatment and in HCM of the obstructive type.  Materials and methods: This was an observational, descriptive, cross-sectional study.  Results:  12 patients were studied, from January 2020 to December 2022, with ages ranging from 5 months to 16 years, diagnosed with HCM of variable clinical presentation and of different etiologies.  Conclusion: HCM from non-sarcomeric causes such as malformation syndromes and inborn errors of metabolism have the worst prognosis and survival. The purpose of early detection of HCM is the early initiation of specific therapy, in order to delay cardiovascular compromise and improve survival.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Miocardiopatía hipertrófica]]></kwd>
<kwd lng="es"><![CDATA[muerte súbita cardiaca]]></kwd>
<kwd lng="es"><![CDATA[arritmia cardiaca]]></kwd>
<kwd lng="es"><![CDATA[estudios genéticos]]></kwd>
<kwd lng="es"><![CDATA[técnicas de diagnóstico cardiovascular]]></kwd>
<kwd lng="en"><![CDATA[Hypertrophic cardiomyopathy]]></kwd>
<kwd lng="en"><![CDATA[sudden cardiac death]]></kwd>
<kwd lng="en"><![CDATA[cardiac arrhythmia]]></kwd>
<kwd lng="en"><![CDATA[genetic studies]]></kwd>
<kwd lng="en"><![CDATA[cardiovascular diagnostic techniques]]></kwd>
</kwd-group>
</article-meta>
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