Rabdomiomas cardíacos en la esclerosis tuberosa: manifestaciones clínicas y evolución de 18 casos diagnosticados en la infancia
Cardiac rhabdomyomas in tuberous sclerosis: clinical symptoms and followup in 18 cases diagnosed in infancy and childhood
Unidad de Arritmias. Servicio de Cardiología Pediátrica. Hospital Infantil La Paz. Madrid
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Fernández Navarro, B. Pozuelo Muñoz, P. Ortí Martínez, L. López Ferrer, A. Cañete Nieto, V. Verdeguer Miralles, V. Castel Sánchez" "autores" => array:7 [ 0 => array:2 [ "nombre" => "J.M." "apellidos" => "Fernández Navarro" ] 1 => array:2 [ "nombre" => "B." "apellidos" => "Pozuelo Muñoz" ] 2 => array:2 [ "nombre" => "P." "apellidos" => "Ortí Martínez" ] 3 => array:2 [ "nombre" => "L." "apellidos" => "López Ferrer" ] 4 => array:2 [ "nombre" => "A." "apellidos" => "Cañete Nieto" ] 5 => array:2 [ "nombre" => "V." "apellidos" => "Verdeguer Miralles" ] 6 => array:2 [ "nombre" => "V." "apellidos" => "Castel Sánchez" ] ] ] ] ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1695403300772894?idApp=UINPBA00005H" "url" => "/16954033/0000005200000001/v1_201307051856/S1695403300772894/v1_201307051856/es/main.assets" ] "itemAnterior" => array:18 [ "pii" => "S1695403300772870" "issn" => "16954033" "doi" => "10.1016/S1695-4033(00)77287-0" "estado" => "S300" "fechaPublicacion" => "2000-01-01" "aid" => "77287" "copyright" => "Asociación Española de Pediatría" "documento" => "article" "crossmark" => 0 "subdocumento" => "fla" "cita" => "An Pediatr (Barc). 2000;52:31-5" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 3152 "formatos" => array:3 [ "EPUB" => 66 "HTML" => 2720 "PDF" => 366 ] ] "es" => array:10 [ "idiomaDefecto" => true "titulo" => "Suicidio en la edad pediátrica: presentación de 30 casos" "tienePdf" => "es" "tieneTextoCompleto" => 0 "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "31" "paginaFinal" => "35" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Suicide in pediatric patients: 30 cases" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contienePdf" => array:1 [ "es" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "B. Osona Rodríguez, M. González Vicent, S. Mencía Bartolomé, J. Casado Flores" "autores" => array:4 [ 0 => array:2 [ "nombre" => "B." "apellidos" => "Osona Rodríguez" ] 1 => array:2 [ "nombre" => "M." "apellidos" => "González Vicent" ] 2 => array:2 [ "nombre" => "S." "apellidos" => "Mencía Bartolomé" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Casado Flores" ] ] ] ] ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1695403300772870?idApp=UINPBA00005H" "url" => "/16954033/0000005200000001/v1_201307051856/S1695403300772870/v1_201307051856/es/main.assets" ] "es" => array:14 [ "idiomaDefecto" => true "titulo" => "Rabdomiomas cardíacos en la esclerosis tuberosa: manifestaciones clínicas y evolución de 18 casos diagnosticados en la infancia" "tieneTextoCompleto" => 0 "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "36" "paginaFinal" => "40" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "S. Jiménez Casso, F. Benito Bartolomé, C. Sánchez Fernández-Bernal" "autores" => array:3 [ 0 => array:3 [ "nombre" => "S." "apellidos" => "Jiménez Casso" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "F." "apellidos" => "Benito Bartolomé" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "Sánchez Fernández-Bernal" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Unidad de Arritmias. Servicio de Cardiología Pediátrica. Hospital Infantil La Paz. Madrid" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "*" "correspondencia" => "Correspondencia: Enrique Casado, 8. 28250 Torrelodones. Madrid" ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Cardiac rhabdomyomas in tuberous sclerosis: clinical symptoms and followup in 18 cases diagnosed in infancy and childhood" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "1999-05-31" "fechaAceptado" => "1999-10-31" "PalabrasClave" => array:2 [ "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec187627" "palabras" => array:4 [ 0 => "Rabdomioma" 1 => "Esclerosis tuberosa" 2 => "Ecocardiografía" 3 => "Tumores cardíacos" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key words" "identificador" => "xpalclavsec187628" "palabras" => array:4 [ 0 => "Rhabdomyoma" 1 => "Tuberous sclerosis" 2 => "Echocardiography" 3 => "Cardiac tumors" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "es" => array:1 [ "resumen" => "<span class="elsevierStyleSectionTitle">Objetivos</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">La esclerosis tuberosa es un síndrome neurocutáneo autosómico dominante con una incidencia de 1/10.000 recién nacidos. La ecocardiografía 2-D ha evidenciado una incidencia de rabdomiomas cardíacos en un 50–64% de los pacientes con esclerosis tuberosa. El objetivo es describir la sintomatología y la evolución de los rabdomiomas cardíacos en los niños afectados de esclerosis tuberosa.</p> <span class="elsevierStyleSectionTitle">Métodos</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Se estudian retrospectivamente 39 niños diagnosticados de esclerosis tuberosa entre 1970 y 1998. Por ecocardiograma se visualizaron rabdomiomas cardíacos en 18 casos (9 niños y 9 niñas) que fueron seguidos entre un mes y 14 años.</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">La localización más frecuente fue ventricular, principalmente en el tabique, aunque también se encontraron en las aurículas. Un lactante presentó obstrucción subaórtica severa que precisó resección quirúrgica. Se evidenciaron diferentes alteraciones electrocardiográficas: extrasístoles auriculares, ventriculares, trastornos de la conducción y síndromes de preexcitación. Dos recién nacidos presentaron arritmias graves, uno con taquicardia supraventricular ortodrómica por vía accesoria lateral izquierda oculta refractaria al tratamiento médico, que precisó ablación con radiofrecuencia, y otro con bloqueo auriculoventricular completo con insuficiencia cardíaca congestiva, que precisó implantación de marcapasos epicárdico. Tras un seguimiento medio de 5,1 ± 4,5 años, se observó regresión de los tumores en 12 de los 18 casos, con desaparición completa en 2. En la actualidad, todos se encuentran asintomáticos.</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Los rabdomiomas cardíacos presentan una escasa sintomatología y un buen pronóstico, con regresión parcial o total en la mayoría de los casos. Precisan tratamiento invasivo únicamente aquellos con sintomatología severa en el período neonatal cuando el tamaño del tumor es máximo.</p>" ] "en" => array:1 [ "resumen" => "<span class="elsevierStyleSectionTitle">Objetive</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Tuberous sclerosis is a neurocutaneous syndrome with autosomal dominant inheritance. The reported incidence is 1:10000. Recent echocardiographic studies showed a 50% to 64% incidence of cardiac rhabdomyomas in patients with tuberous sclerosis. The objetive is to assess the history of cardiac rhabdomyomas in infants and children with tuberous sclerosis.</p> <span class="elsevierStyleSectionTitle">Methods</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">From 1970 to 1998, 39 patients were diagnosticated of tuberous sclerosis. Cardiac rhabdomyomas were present in 18 of them, 9 girls and 9 boys. Ecocardiografic follow up ranged from 1 month to 14 years.</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Echocardiografic studies showed rhabdomyomas located in the interventricular septum, ventricles, right and left atrium. A newborn, had subaortic obstruction related to a tumor who precised surgery excision. Standard electrocardiogram showed diferent disturbances: premature auricular and ventricular contractions, conduccion disturbances and preexcitation syndromes. Two patients, both neonates, had severe arrhythmias, one of them debuted with supraventricular tachycardia due to an accessory hidden left atrioventricular pathway who undergone sucessfull radiofrecuency ablation; and the other patient was diagnosticated prenataly of fetal complete heart block and precised permanent pacemaker at birth. After a follow up of 5.1 ± 4.5 years, spontaneous regresion of the tumors was shown in 12 of the 18 patients with complete resolution in two of them. All of them are asymptomatic nowadays.</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Our findings confirms that cardiac rhabdomyomas are most often a bening condition in which spontaneous regresion is the rule and surgery is only recommended for patients with life threatening obstruction or refractary disrhythmias in the neonatal period when the tumor size is maximum.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografía" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:19 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1." 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 4 | 16 | 20 |
| 2024 Octubre | 32 | 58 | 90 |
| 2024 Septiembre | 32 | 45 | 77 |
| 2024 Agosto | 42 | 102 | 144 |
| 2024 Julio | 36 | 45 | 81 |
| 2024 Junio | 39 | 36 | 75 |
| 2024 Mayo | 36 | 35 | 71 |
| 2024 Abril | 34 | 36 | 70 |
| 2024 Marzo | 20 | 46 | 66 |
| 2024 Febrero | 31 | 49 | 80 |
| 2024 Enero | 22 | 40 | 62 |
| 2023 Diciembre | 20 | 27 | 47 |
| 2023 Noviembre | 27 | 20 | 47 |
| 2023 Octubre | 31 | 31 | 62 |
| 2023 Septiembre | 34 | 28 | 62 |
| 2023 Agosto | 23 | 21 | 44 |
| 2023 Julio | 35 | 31 | 66 |
| 2023 Junio | 30 | 30 | 60 |
| 2023 Mayo | 37 | 30 | 67 |
| 2023 Abril | 35 | 18 | 53 |
| 2023 Marzo | 23 | 32 | 55 |
| 2023 Febrero | 16 | 17 | 33 |
| 2023 Enero | 15 | 22 | 37 |
| 2022 Diciembre | 22 | 18 | 40 |
| 2022 Noviembre | 40 | 36 | 76 |
| 2022 Octubre | 21 | 42 | 63 |
| 2022 Septiembre | 24 | 35 | 59 |
| 2022 Agosto | 35 | 45 | 80 |
| 2022 Julio | 26 | 36 | 62 |
| 2022 Junio | 23 | 36 | 59 |
| 2022 Mayo | 30 | 36 | 66 |
| 2022 Abril | 32 | 36 | 68 |
| 2022 Marzo | 34 | 52 | 86 |
| 2022 Febrero | 25 | 27 | 52 |
| 2022 Enero | 37 | 43 | 80 |
| 2021 Diciembre | 24 | 42 | 66 |
| 2021 Noviembre | 34 | 48 | 82 |
| 2021 Octubre | 45 | 42 | 87 |
| 2021 Septiembre | 35 | 57 | 92 |
| 2021 Agosto | 36 | 38 | 74 |
| 2021 Julio | 38 | 46 | 84 |
| 2021 Junio | 31 | 39 | 70 |
| 2021 Mayo | 24 | 36 | 60 |
| 2021 Abril | 74 | 49 | 123 |
| 2021 Marzo | 68 | 40 | 108 |
| 2021 Febrero | 55 | 11 | 66 |
| 2021 Enero | 41 | 28 | 69 |
| 2020 Diciembre | 50 | 26 | 76 |
| 2020 Noviembre | 42 | 18 | 60 |
| 2020 Octubre | 42 | 12 | 54 |
| 2020 Septiembre | 22 | 12 | 34 |
| 2020 Agosto | 42 | 8 | 50 |
| 2020 Julio | 41 | 23 | 64 |
| 2020 Junio | 31 | 11 | 42 |
| 2020 Mayo | 51 | 32 | 83 |
| 2020 Abril | 30 | 20 | 50 |
| 2020 Marzo | 29 | 14 | 43 |
| 2020 Febrero | 54 | 22 | 76 |
| 2020 Enero | 38 | 20 | 58 |
| 2019 Diciembre | 81 | 34 | 115 |
| 2019 Noviembre | 26 | 10 | 36 |
| 2019 Octubre | 20 | 25 | 45 |
| 2019 Septiembre | 42 | 13 | 55 |
| 2019 Agosto | 28 | 15 | 43 |
| 2019 Julio | 35 | 24 | 59 |
| 2019 Junio | 71 | 40 | 111 |
| 2019 Mayo | 136 | 64 | 200 |
| 2019 Abril | 197 | 36 | 233 |
| 2019 Marzo | 92 | 34 | 126 |
| 2019 Febrero | 74 | 21 | 95 |
| 2019 Enero | 74 | 31 | 105 |
| 2018 Diciembre | 62 | 29 | 91 |
| 2018 Noviembre | 65 | 31 | 96 |
| 2018 Octubre | 85 | 22 | 107 |
| 2018 Septiembre | 43 | 11 | 54 |
| 2018 Agosto | 1 | 0 | 1 |
| 2018 Julio | 3 | 0 | 3 |
| 2018 Mayo | 10 | 0 | 10 |
| 2018 Abril | 31 | 0 | 31 |
| 2018 Marzo | 49 | 0 | 49 |
| 2018 Febrero | 33 | 0 | 33 |
| 2018 Enero | 31 | 1 | 32 |
| 2017 Diciembre | 25 | 0 | 25 |
| 2017 Noviembre | 26 | 0 | 26 |
| 2017 Octubre | 35 | 0 | 35 |
| 2017 Septiembre | 25 | 0 | 25 |
| 2017 Agosto | 20 | 0 | 20 |
| 2017 Julio | 31 | 1 | 32 |
| 2017 Junio | 31 | 42 | 73 |
| 2017 Mayo | 42 | 24 | 66 |
| 2017 Abril | 50 | 25 | 75 |
| 2017 Marzo | 39 | 35 | 74 |
| 2017 Febrero | 33 | 37 | 70 |
| 2017 Enero | 42 | 12 | 54 |
| 2016 Diciembre | 59 | 22 | 81 |
| 2016 Noviembre | 79 | 21 | 100 |
| 2016 Octubre | 87 | 27 | 114 |
| 2016 Septiembre | 82 | 38 | 120 |
| 2016 Agosto | 76 | 29 | 105 |
| 2016 Julio | 31 | 26 | 57 |
| 2016 Junio | 1 | 0 | 1 |
| 2016 Mayo | 2 | 0 | 2 |
| 2016 Abril | 1 | 0 | 1 |
| 2016 Marzo | 2 | 0 | 2 |
| 2016 Febrero | 1 | 0 | 1 |
| 2016 Enero | 1 | 0 | 1 |
| 2015 Diciembre | 3 | 0 | 3 |
| 2015 Noviembre | 1 | 0 | 1 |
| 2015 Octubre | 2 | 0 | 2 |
| 2015 Septiembre | 2 | 0 | 2 |
| 2015 Agosto | 1 | 0 | 1 |
| 2015 Julio | 4 | 0 | 4 |
| 2015 Junio | 7 | 0 | 7 |
| 2015 Mayo | 9 | 0 | 9 |
| 2015 Abril | 17 | 0 | 17 |
| 2015 Marzo | 8 | 0 | 8 |
| 2015 Febrero | 6 | 0 | 6 |
| 2015 Enero | 11 | 0 | 11 |
| 2014 Diciembre | 11 | 0 | 11 |
| 2014 Noviembre | 3 | 0 | 3 |
| 2014 Octubre | 9 | 0 | 9 |
| 2014 Septiembre | 7 | 0 | 7 |
| 2014 Agosto | 7 | 0 | 7 |
| 2014 Julio | 10 | 0 | 10 |
| 2014 Junio | 12 | 0 | 12 |
| 2014 Mayo | 18 | 0 | 18 |
| 2014 Abril | 14 | 0 | 14 |
| 2014 Marzo | 9 | 0 | 9 |
| 2014 Febrero | 14 | 0 | 14 |
| 2014 Enero | 12 | 0 | 12 |
| 2013 Diciembre | 15 | 0 | 15 |
| 2013 Noviembre | 14 | 0 | 14 |
| 2013 Octubre | 19 | 0 | 19 |
| 2013 Septiembre | 19 | 0 | 19 |
| 2013 Agosto | 9 | 0 | 9 |
| 2013 Julio | 4 | 0 | 4 |
| 2013 Junio | 2 | 0 | 2 |
| 2013 Mayo | 2 | 0 | 2 |
| 2013 Marzo | 4 | 0 | 4 |
| 2013 Febrero | 18 | 0 | 18 |
| 2013 Enero | 7 | 0 | 7 |
| 2012 Diciembre | 6 | 0 | 6 |
| 2012 Noviembre | 4 | 0 | 4 |
| 2012 Octubre | 2 | 0 | 2 |
| 2012 Septiembre | 5 | 0 | 5 |
| 2012 Agosto | 2 | 0 | 2 |
| 2000 Enero | 3634 | 0 | 3634 |
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