[Spanish consensus document on diagnosis, stabilisation and treatment of pediatric multisystem inflammatory syndrome related to SARS-CoV-2 (SIM-PedS)]. / Documento español de consenso sobre diagnóstico, estabilización y tratamiento del síndrome inflamatorio multisistémico pediátrico vinculado a SARS-CoV-2 (SIM-PedS).

A new paediatric multisystem inflammatory syndrome, linked to SARS-CoV-2, has been described. The clinical picture is variable and is associated with an active or recent infection due to SARS-CoV-2. A review of the existing literature by a multidisciplinary group of paediatric specialists is presented in this document. Later, they make recommendations on the stabilisation, diagnosis, and treatment of this syndrome.

[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.

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.

[Current situation of the organisation, resources and activity in paediatric cardiology in Spain]. / Situación actual de la organización, recursos y actividad en cardiología pediátrica en España.

INTRODUCTION: The results are presented on the «current situation of the organisation, resources and activity in paediatric cardiology in Spain¼. It was promoted by the Spanish Society of Paediatric Cardiology and Congenital Heart disease. MATERIAL AND METHODS: An analysis was carried out on the results obtained from a specifically designed questionnaire, prepared by the Spanish Society of Paediatric Cardiology and Congenital Heart disease, that was sent to all hospitals around the country that offer the speciality of paediatric cardiology. RESULTS: A total of 86 questionnaires were obtained, including 14 hospitals that perform cardiac surgery on children. A total of 190 paediatric cardiology consultants, 40 cardiac surgeons, and 27 middle grade doctors performing their paediatric residency (MIR program) were identified. All hospitals had adequate equipment to perform an optimal initial evaluation of any child with a possible cardiac abnormality, but only tertiary centres could perform complex diagnostic procedures, interventional cardiology, and cardiac surgery. In almost all units around the country, paediatric cardiology consultants were responsible for outpatient clinics and hospital admissions, whereas foetal cardiology units were still mainly managed by obstetricians. The number of diagnostic and therapeutic procedures was similar to those reported in the first survey, except for a slight decrease in the total number of closed cardiac surgery procedures, and a proportional increase in the number of therapeutic catheterisations. CONCLUSIONS: Paediatric Cardiology in Spain is performed by paediatric cardiology consultants that were trained initially as general paediatricians, and then completed a paediatric cardiology training period. Almost all units have adequate means for diagnosis and treatment. Efforts should be directed to create a national registry that would not only allow a prospective quantification of diagnostic and therapeutic procedures, but also focus on their clinical outcomes.

[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.

[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.

[National consensus on the cardiological treatment and follow-up of Kawasaki disease]. / Consenso nacional sobre diagnóstico, tratamiento y seguimiento cardiológico de la enfermedad de Kawasaki.

Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient's clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.