Recomendaciones de la Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas en relación con el uso de medicamentos en el trastorno por déficit de atención e hiperactividad en niños y adolescentes con cardiopatías conocidas y en la población pediátrica general, posicionamiento de la Asociación Española de Pediatría / Recommendations of the Spanish Society of Paediatric Cardiology and Congenital Heart Disease as regards the use of drugs in attention deficit hyperactivity disorder in children and adolescents with a known heart disease, as well as in the general paediatric population: Position statement by the Spanish Paediatric Association

INTRODUCCIÓN: Los fármacos aprobados para el trastorno por déficit de atención con hiperactividad (TDAH) en España son: metilfenidato, lisdexanfetamina, atomoxetina y guanfacina. Debido a los efectos adversos cardiovasculares que pueden producir, principalmente aumento de la presión arterial y la frecuencia cardíaca, su uso en pacientes con cardiopatías conocidas o no diagnosticadas puede ser controvertido. OBJETIVO: Realización de un documento de consenso de la Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas (SECPCC) y expertos de otras agencias y sociedades como instrumento para el cardiólogo infantil y los médicos que tratan niños y adolescentes con TDAH. METODOLOGÍA: Análisis de la bibliografía y las guías de práctica clínica, fichas técnicas aprobadas por la Agencia Española del Medicamento y Productos Sanitarios y Guía del Ministerio de Sanidad español. Formación de un grupo de trabajo con un coordinador, miembros de los grupos de trabajo de Cardiología Clínica y Arritmias de la SECPCC. Este grupo realizó un documento que fue revisado por un grupo de expertos externos y un grupo de expertos internos de la SECPCC, llegando a un consenso para la obtención del documento final. RESULTADOS: Se presentan las recomendaciones de la SECPCC y el grupo de expertos sobre la evaluación cardiovascular previa al tratamiento en niños y adolescentes sin enfermedad cardiovascular conocida y con enfermedad cardiovascular conocida. Se presentan las recomendaciones de la SECPCC y el grupo de expertos sobre el uso de medicamentos para el TDAH en niños y adolescentes con síntomas cardiológicos sin evidencia de cardiopatía, cardiopatías congénitas, miocardiopatías, síndrome de Marfan y otras aortopatías, hipertensión arterial y arritmias

INTRODUCTION: Approved drugs for attention deficit hyperactivity disorder (ADHD) in Spain are methylphenidate, lisdexamphetamine, atomoxetine and guanfacine. Due to adverse cardiovascular effects, mainly increased blood pressure and heart rate, its use in patients with known or undiagnosed heart disease may be controversial. OBJECTIVE: To obtain a consensus document from the Spanish Society of Paediatric Cardiology and Congenital Heart Diseases (SECPCC) and experts from other Agencies and Societies as a guide for the paediatric cardiologist and physicians who treat children and adolescents with ADHD. METHODOLOGY: An analysis was performed on the bibliography and Clinical Practice Guidelines, technical data sheets approved by the Spanish Agency of Medicines and Health Devices, and the Spanish Ministry of Health Guidelines. A Working Group was formed, with a Coordinator, as well as members of the Clinical Cardiology Working Group and Arrhythmia Group of the SECPCC. This Group produced a preliminary document that was reviewed by a group of external experts and a group of internal experts of the SECPCC with a consensus being reached on the final document. RESULTS: The recommendations of the SECPCC and the group of experts are presented on cardiovascular evaluation prior to treatment in children and adolescents with no known cardiovascular disease and with known cardiovascular disease. The recommendations of the SECPCC and the group of experts are also presented on the use of medications for ADHD in children and adolescents with cardiological symptoms with no evidence of heart disease, congenital heart disease, cardiomyopathy, Marfan syndrome and other aortic diseases, hypertension, and arrhythmias

[Recommendations of the Spanish Society of Paediatric Cardiology and Congenital Heart Disease as regards the use of drugs in attention deficit hyperactivity disorder in children and adolescents with a known heart disease, as well as in the general paediatric population: Position statement by the Spanish Paediatric Association]. / Recomendaciones de la Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas en relación con el uso de medicamentos en el trastorno por déficit de atención e hiperactividad en niños y adolescentes con cardiopatías conocidas y en la población pediátrica general, posicionamiento de la Asociación Española de Pediatría.

INTRODUCTION: Approved drugs for attention deficit hyperactivity disorder (ADHD) in Spain are methylphenidate, lisdexamphetamine, atomoxetine and guanfacine. Due to adverse cardiovascular effects, mainly increased blood pressure and heart rate, its use in patients with known or undiagnosed heart disease may be controversial. OBJECTIVE: To obtain a consensus document from the Spanish Society of Paediatric Cardiology and Congenital Heart Diseases (SECPCC) and experts from other Agencies and Societies as a guide for the paediatric cardiologist and physicians who treat children and adolescents with ADHD. METHODOLOGY: An analysis was performed on the bibliography and Clinical Practice Guidelines, technical data sheets approved by the Spanish Agency of Medicines and Health Devices, and the Spanish Ministry of Health Guidelines. A Working Group was formed, with a Coordinator, as well as members of the Clinical Cardiology Working Group and Arrhythmia Group of the SECPCC. This Group produced a preliminary document that was reviewed by a group of external experts and a group of internal experts of the SECPCC with a consensus being reached on the final document. RESULTS: The recommendations of the SECPCC and the group of experts are presented on cardiovascular evaluation prior to treatment in children and adolescents with no known cardiovascular disease and with known cardiovascular disease. The recommendations of the SECPCC and the group of experts are also presented on the use of medications for ADHD in children and adolescents with cardiological symptoms with no evidence of heart disease, congenital heart disease, cardiomyopathy, Marfan syndrome and other aortic diseases, hypertension, and arrhythmias.

Incidencia y evolución de las cardiopatías congénitas en España durante 10 años (2003-2012) / Incidence and evolution of congenital heart disease in Spain from 2003 until 2012

INTRODUCCIÓN Y OBJETIVOS: Las cardiopatías congénitas (CC) son las malformaciones congénitas más frecuentes. En España no hay datos de su incidencia a nivel nacional. El objetivo del estudio es analizar la incidencia y evolución de las CC en España. MÉTODOS: Estudio observacional retrospectivo utilizando el conjunto mínimo básico de datos durante 10 años (2003-2012), en menores de un año, seleccionando las altas hospitalarias con códigos de CC (Clasificación Internacional de Enfermedades, 9.a revisión, modificación clínica). Se describió la evolución anual de la incidencia acumulada y su distribución geográfica, y se analizó mediante riesgos relativos de incidencia y razón de incidencias estandarizadas por comunidad autónoma. RESULTADOS: Durante el periodo analizado 64.831 menores de un año fueron diagnosticados de CC al alta hospitalaria sobre 4.766.325 nacimientos con una incidencia del 13,6‰. La incidencia excluyendo la comunicación interauricular fue del 7,29‰. Los códigos más frecuentes fueron: comunicación interauricular (6,31‰), comunicación interventricular (3,48‰), ductus arteriosus persistente (2,71‰), coartación de aorta (0,55‰), estenosis pulmonar (0,50‰), trasposición de grandes vasos (0,49‰), canal auriculoventricular (0,45‰) y tetralogía de Fallot (0,41‰). La distribución geográfica de las cardiopatías graves y muy graves no resultó uniforme presentando Castilla y León junto con Extremadura la mayor incidencia, y Madrid y Cantabria la menor. CONCLUSIONES: Durante el tiempo de estudio se observa un aumento de cardiopatías leves que puede estar influido por la mejora de las técnicas diagnósticas, el uso extendido de la ecocardiografía y la propia codificación Clasificación Internacional de Enfermedades, 9.a revisión, modificación clínica y una disminución de las cardiopatías muy graves cuyo análisis presenta mayor validez al estar menos influido por factores externos. La incidencia de cardiopatías graves y muy graves no fue uniforme en España

INTRODUCTION AND OBJECTIVES: Congenital heart disease (CHD) represents the most common congenital malformation. The objective of this study was to analyse the incidence of CHD in Spain, and it is the first nationwide study so far. METHODS: A retrospective observational study was performed in order to evaluate the incidence of CHD in Spain. The administrative database (minimum basic data set) from 2003 to 2012 was analysed in children less than one year old admitted to hospital with codes of CHD (International Classification of Diseases, 9 th Revision, clinical modification). Cumulative incidence, Incidence relative risk, and standardised incidence ratio were calculated to study geographic variations. RESULTS: There were 64,831 infants with CHD among the 4,766,325 births analysed during the period studied, with an incidence of 13.6‰. The incidence excluding atrial septal defect was 7.29 ‰.The most frequent CHD were atrial septal defect (6.31‰), ventricular septal defect (3.48‰), patent ductus arteriosus (2.71‰), coarctation of the aorta (0.55‰), pulmonary stenosis (0.50‰), transposition of the great arteries (0.49‰), atrioventricular septal defect (0.45‰), and tetralogy of Fallot (0.41‰). Castilla and Leon, together with Extremadura, showed the highest risks for severe and very severe CHD, while Madrid and Cantabria showed the lowest. CONCLUSIONS: An increase of mild CHD was observed during the period analysed. This could have been influenced by improvements in diagnostic techniques, extended use of echocardiography, and the International Classification of Diseases, 9 th Revision, clinical modification coding system, and to a decrease in very severe CHD, which is less influenced by external factors. Significant geographical differences were found in the incidence of severe and very severe CHD

Mortalidad de las cardiopatías congénitas en España durante 10 años (2003-2012) / Congenital heart disease mortality in Spain during a 10 year period (2003-2012)

Introducción y objetivos: Las cardiopatías congénitas suponen una de las principales causas de mortalidad infantil en países desarrollados. En España no hay publicaciones sobre mortalidad de las cardiopatías congénitas a nivel nacional. El objetivo del estudio es determinar la tasa de mortalidad infantil de las cardiopatías congénitas, así como la letalidad de las distintas cardiopatías, durante un periodo de 10 años. Material y métodos: Estudio observacional retrospectivo utilizando el conjunto mínimo básico de datos de 2003 a 2012 en niños ingresados con diagnóstico de cardiopatía congénita. Se realiza un análisis descriptivo de los pacientes y se calcula la tasa de mortalidad y los riesgos relativos de mortalidad mediante regresión de Poisson. Resultados: Fallecieron 2.970 menores de un año sobre una población de 64.831 pacientes con cardiopatías congénitas (4,58%). El 73,8% fallecieron la primera semana de vida. La tasa de mortalidad infantil en niños con cardiopatías congénitas fue de 6,23 por 10.000 nacidos vivos, cifra que se mantuvo constante a lo largo de los 10 años estudiados y que supone el 18% de la mortalidad infantil total. Las cardiopatías con mayor letalidad fueron el síndrome del corazón izquierdo hipoplásico (41,4%), la interrupción del arco aórtico (20%) y el drenaje venoso pulmonar anómalo total (16,8%), y las de menor letalidad la comunicación interauricular (1%) y la estenosis pulmonar (1,1%). Conclusiones: Las cardiopatías congénitas constituyen una causa importante de mortalidad infantil, que no ha presentado variaciones importantes durante el tiempo de estudio. La proporción de niños que fallecen es similar a la de los países de nuestro entorno. Pese a los actuales avances, algunas cardiopatías presentan letalidad elevada

Introduction and objectives: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. Methods: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012. Mortality rate and relative risk of mortality were estimated by Poisson regression. Results: There were 2,970 (4.58%) infant deaths in a population of 64,831 patients with congenital heart disease, with 73.8% of deaths occurring during first week of life. Infant mortality rate in patients with congenital heart disease was 6.23 per 10,000 live births, and remained constant during the ten-year period of the study, representing 18% of total infant mortality rate in Spain. The congenital heart diseases with highest mortality rates were hypoplastic left heart syndrome (41.4%), interruption of aortic arch (20%), and total anomalous pulmonary drainage (16.8%). Atrial septal defect (1%) and pulmonary stenosis (1.1%) showed the lowest mortality rate. Conclusions: Congenital heart disease was a major cause of infant mortality with no variations during the study period. The proportion of infants who died in our study was similar to other similar countries. In spite of current medical advances, some forms of congenital heart disease show very high mortality rates

[Incidence and evolution of congenital heart disease in Spain from 2003 until 2012]. / Incidencia y evolución de las cardiopatías congénitas en España durante 10 años (2003-2012).

INTRODUCTION AND OBJECTIVES: Congenital heart disease (CHD) represents the most common congenital malformation. The objective of this study was to analyse the incidence of CHD in Spain, and it is the first nationwide study so far. METHODS: A retrospective observational study was performed in order to evaluate the incidence of CHD in Spain. The administrative database (minimum basic data set) from 2003 to 2012 was analysed in children less than one year old admitted to hospital with codes of CHD (International Classification of Diseases, 9th Revision, clinical modification). Cumulative incidence, Incidence relative risk, and standardised incidence ratio were calculated to study geographic variations. RESULTS: There were 64,831 infants with CHD among the 4,766,325 births analysed during the period studied, with an incidence of 13.6‰. The incidence excluding atrial septal defect was 7.29 ‰.The most frequent CHD were atrial septal defect (6.31‰), ventricular septal defect (3.48‰), patent ductus arteriosus (2.71‰), coarctation of the aorta (0.55‰), pulmonary stenosis (0.50‰), transposition of the great arteries (0.49‰), atrioventricular septal defect (0.45‰), and tetralogy of Fallot (0.41‰). Castilla and Leon, together with Extremadura, showed the highest risks for severe and very severe CHD, while Madrid and Cantabria showed the lowest. CONCLUSIONS: An increase of mild CHD was observed during the period analysed. This could have been influenced by improvements in diagnostic techniques, extended use of echocardiography, and the International Classification of Diseases, 9th Revision, clinical modification coding system, and to a decrease in very severe CHD, which is less influenced by external factors. Significant geographical differences were found in the incidence of severe and very severe CHD.

[Congenital heart disease mortality in Spain during a 10 year period (2003-2012)]. / Mortalidad de las cardiopatías congénitas en España durante 10 años (2003-2012).

INTRODUCTION AND OBJECTIVES: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. METHODS: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012. Mortality rate and relative risk of mortality were estimated by Poisson regression. RESULTS: There were 2,970 (4.58%) infant deaths in a population of 64,831 patients with congenital heart disease, with 73.8% of deaths occurring during first week of life. Infant mortality rate in patients with congenital heart disease was 6.23 per 10,000 live births, and remained constant during the ten-year period of the study, representing 18% of total infant mortality rate in Spain. The congenital heart diseases with highest mortality rates were hypoplastic left heart syndrome (41.4%), interruption of aortic arch (20%), and total anomalous pulmonary drainage (16.8%). Atrial septal defect (1%) and pulmonary stenosis (1.1%) showed the lowest mortality rate. CONCLUSIONS: Congenital heart disease was a major cause of infant mortality with no variations during the study period. The proportion of infants who died in our study was similar to other similar countries. In spite of current medical advances, some forms of congenital heart disease show very high mortality rates.

Desconexión de la arteria pulmonar derecha con doble sistema ductal / Disconnection of the right pulmonary artery with bilateral ductus arteriosus

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Totally anomalous pulmonary venous connection to superior caval vein after an intracytoplasmic injection of sperm.

We report the occurrence of non-obstructive totally anomalous pulmonary venous connection to the superior caval vein in a child conceived by intracytoplasmic injection of sperm, a type of assisted reproductive technology. Totally anomalous pulmonary venous connection is an uncommon congenital anomaly, in which all the pulmonary veins connect to the morphologically right atrium, or one of its tributaries. To our knowledge, this malformation has not been previously described in a child conceived on the basis of assisted reproductive technology.