Recomendación para la sospecha, manejo y seguimiento del compromiso cardiovascular en pacientes con Síndrome Inflamatorio Multisistémico en Pediatría asociado a COVID-19 (PIMS-TC). Declaración de Posición de la Sociedad Chilena de Pediatría (SOCHIPE) y Sociedad Chilena de Cardiología y Cirugía Cardiovascular (SOCHICAR) / Recommendation for the recognition, management and follow up of cardiovascular compromise in patients with Pediatric Multisystemic Inflammatory Syndrome associated with COVID-19 (PIMS-CT). Position statement of Chilean Scientific Societies

Desde la aparición de la pandemia por SARS-CoV-2, la población pediátrica ha sido menos afectada por la enfermedad tanto en frecuencia como en severidad. Sin embargo, desde abril de este año se han reportado casos de presentación y gravedad variables, caracterizados por fenómenos inflamato rios que afectan múltiples órganos, condición denominada Síndrome Inflamatorio Multisistémico Pediátrico (PIMS). La literatura describe frecuente compromiso cardíaco, hasta en un 80%. Este se caracteriza por injuria miocárdica con significativa elevación de biomarcadores: Troponinas séricas I/T, BNP o NT-ProBNP, unido a diversos grados de disfunción ventricular, pericarditis, valvulitis y arritmias. Además, se ha evidenciado la presencia de compromiso coronario el cual puede ocurrir hasta en un 23% de los casos, en un rango que va desde dilataciones hasta aneurismas. El seguimien to cardiológico hospitalizado y ambulatorio se ha sistematizado en base a los fenotipos clínicos de presentación: injuria miocárdica (miocarditis, valvulitis, pericarditis), shock (habitualmente de tipo "vasopléjico"), manifestaciones tipo Enfermedad de Kawasaki y aquellos casos PIMS que no cumplen con la clínica de los tres precedentes. Este último grupo es el que representa el mayor desafío en el cor to, mediano y seguimiento a largo plazo. Por esta razón se requiere un equipo multidisciplinario para su manejo. Considerando la alta frecuencia del compromiso cardíaco en el PIMS y la importancia de lograr un consenso en su manejo y seguimiento, se presentan estas recomendaciones según el estado actual del conocimiento de esta patología recientemente descrita.

Since the onset of the SARS-CoV-2 pandemic, the pediatric population has been less affected by the disease both in frequency and severity. However, since April cases of variable presentation and severity characterized by inflammatory phenomena that affect multiple organs have been reported, a condition called Multisystem Inflammatory Syndrome in Children (MIS-C). The literature has reported frequent cardiac involvement, up to 80%. This is characterized by myocardial injury with a significant increase of biomarkers such as serum troponins I and T, BNP, or NT-ProBNP coupled with varying degrees of ventricular dysfunction, pericarditis, valvulitis, and arrhythmias. Coronary compromise has also been described, which can occur in up to 23% of cases, and ranges from dila tations to aneurysms. Inpatient and outpatient cardiology follow-up has been systematized based on the clinical phenotypes such as myocardial injury (myocarditis, valvulitis, pericarditis), shock (usua lly vasoplegic), Kawasaki disease-type manifestations, and those MIS-C that do not comply with the clinic of the previous three. This last group represents the main challenge in the short-, medium- and long-term follow-up, therefore, it is necessary a multidisciplinary team for managing these patients. Considering the high frequency of cardiac compromise in MIS-C, and the importance of reaching a consensus regarding its management and follow-up, we present these recommendations according to the current state of knowledge regarding this recently described pathology.

[Recommendation for the recognition, management and follow up of cardiovascular compromise in patients with Pediatric Multisystemic Inflammatory Syndrome associated with COVID-19 (PIMS-CT). Position statement of Chilean Scientific Societies]. / Recomendación para la sospecha, manejo y seguimiento del compromiso cardiovascular en pacientes con Síndrome Inflamatorio Multisistémico en Pediatría asociado a COVID-19 (PIMS-TC). Declaración de Posición de la Sociedad Chilena de Pediatría (SOCHIPE) y Sociedad Chilena de Cardiología y Cirugía Cardiovascular (SOCHICAR).

Since the onset of the SARS-CoV-2 pandemic, the pediatric population has been less affected by the disease both in frequency and severity. However, since April cases of variable presentation and severity characterized by inflammatory phenomena that affect multiple organs have been reported, a condition called Multisystem Inflammatory Syndrome in Children (MIS-C). The literature has reported frequent cardiac involvement, up to 80%. This is characterized by myocardial injury with a significant increase of biomarkers such as serum troponins I and T, BNP, or NT-ProBNP coupled with varying degrees of ventricular dysfunction, pericarditis, valvulitis, and arrhythmias. Coronary compromise has also been described, which can occur in up to 23% of cases, and ranges from dila tations to aneurysms. Inpatient and outpatient cardiology follow-up has been systematized based on the clinical phenotypes such as myocardial injury (myocarditis, valvulitis, pericarditis), shock (usua lly vasoplegic), Kawasaki disease-type manifestations, and those MIS-C that do not comply with the clinic of the previous three. This last group represents the main challenge in the short-, medium- and long-term follow-up, therefore, it is necessary a multidisciplinary team for managing these patients. Considering the high frequency of cardiac compromise in MIS-C, and the importance of reaching a consensus regarding its management and follow-up, we present these recommendations according to the current state of knowledge regarding this recently described pathology.

Propranolol en el tratamiento de los hemangiomas de la infancia / Propranolol in the treatment of infantile hemangioma

Infantile Hemangiomas (IH) are common vascular tumors in children. Only 10-15 percent) should be treated by any vital, functional or aesthetic complications. Oral corticosteroids are the treatment of choice. However, in the last two years an excellent response to treatment of hemangiomas with oral Propranolol has been reported. Objective: evaluate this new therapy in a limited number of chilean patients with IH with an indication of systemic treatment and present this experience. Patients and Methods: We present 30 cases of IH treated at our institution with oral Propranolol, using a dose of 2 mg/kg/day divided in 2 daily doses. Results: 11 percent of patients were female, 40 percent hemangiomas had segmental distribution, 90 percent were solitary, 61 percent were located in the head and neck and 23 percent were ulcerated. The treatment started at an average of 119 days of life and lasted for an average of 7 months. In all our patients the use of Propranolol accelerated the onset of involution of the hemangiomas and decreased color, brightness and growth too. 43 percent of the patients had irritability and sleep disorders. No significant adverse effects were reported in any of our patients. Conclusión: Propranolol treatment was highly effective and safe in our series of patients and we propose, taking into account published reports based on this data, it should become the treatment of choice for IH.

Los Hemangiomas de la Infancia (HI) son tumores vasculares frecuentes en la edad pediátrica. Sólo un 10-15 por cientoo debe ser tratado por eventuales consecuencias vitales, funcionales o estéticas. Los corticoides orales son el tratamiento de elección actual. Sin embargo, en los últimos 2 años se ha reportado la excelente respuesta de los hemangiomas al tratamiento con Propranolol oral. Objetivo: Evaluar esta nueva terapéutica en un número limitado de pacientes Chilenos con hemangiomas de la infancia con indicación de tratamiento sistémico y presentar esta experiencia. Pacientes y Método: Presentamos 30 casos de HI tratados en nuestra institución con Propranolol oral, usando una dosis de 2 mg/kg/día en 2 tomas diarias. Resultados: Un 77 por ciento de los pacientes eran de sexo femenino. El 40 por ciento de los hemangiomas eran segmentarios, 90 por ciento eran únicos, 67 por ciento estaban ubicados en cabeza y cuello y 23 por ciento estaban ulcerados. El tratamiento se inició en promedio a los 119 días de vida y duró en promedio 7 meses. En la totalidad de nuestros pacientes el uso de Propranolol aceleró el inicio de la involución del hemangioma y disminuyó el color, brillo y crecimiento. El 43 por ciento de los pacientes presentó irritabilidad y trastornos del sueño. No se presentaron efectos adversos importantes en ninguno de nuestros pacientes. Conclusión: El tratamiento con Propranolol fue altamente efectivo y seguro en nuestra serie de pacientes y proponemos, tomando en cuenta los reportes publicados al respecto, que debiera ser el tratamiento de elección para los hemangiomas de la infancia.

Cierre percutáneo del ductus arterioso con coils: diagnóstico y seguimiento ecocardiográfico a largo plazo / Percutaneous closure of arterious ductus with coils: echocardiographic diagnosis and follow up

Objectives: We report about the percutaneous closure with coils of small and medium sized ductus arteriosus, and their long term follow up in a single center. Patients and Method: This is a retrospective study, including 291 patients in an intention to treat basis, between 1998 and 2006. Results: The median age and weight at time of procedure was 45 months and 16,7 Kg respectively. The immediate rate of occlusion was 82 percent and 91,3 percent at 1 year follow up. Up to 98 percent of cases needed just 1 coil to close the defect. The result was considered sub optimal in 33 cases (11,6 percent) almost half of this group was evaluated as having mild residual shunt at one year, all of them closed with additional coils percutaneously. Only the size of ductus was related to this type of result (>3 mm). Conclusions: The percutaneous closure with coils, is a highly eficacious alternative, for treatment of ductus arteriosus up to 2,5-3 mm, as minor diameter, with a very convenient cost/benefit rate. Beyond these limits the advantages disappear, and alternative devices become the treatment of choice.

Objetivos: Se reporta sobre el cierre percutáneo de ductus pequeños y medianos con coils en un solo centro, su diagnóstico y seguimiento ecocardiográfico a largo plazo. Pacientes y método: Se realizó un estudio retrospectivo sobre 291 pacientes portadores de ductus arterioso diagnosticado ecocardiográficamente en quienes se intentó el cierre percutáneo entre 1998y 2006. Resultados: La mediana de edad y peso de los pacientes al momento del procedimiento fue de 45 meses y 16,7 kg. La tasa de oclusión inmediata fue de 82 por ciento y de 91,3 por ciento a un año. En el 98 por ciento de los casos se utilizó un solo coil para cerrar el defecto. El resultado fue sub óptimo en 33 casos (11,6 por ciento) siendo casi la mitad de estos casos correspondientes a shunt residual leve a un año, todos cerrados con coil en procedimiento percutáneo adicional. Sólo el tamaño del ductus estuvo relacionado con este resultado (>3mm). Conclusiones: El cierre con coils sigue siendo un tratamiento altamente eficaz para ductus de hasta 2,5-3 mm de diámetro menor, con una relación costo/beneficio muy favorable al compararse con dispositivos alternativos. Más allá de estos límites las ventajas mencionadas tienden a disminuir en forma considerable y se hace discutible su uso.

Atresia pulmonar con septum ventricular intacto: espectro morfológico y diagnóstico prenatal / Pulmonary atresia with ventricular septum intact: morphologic phantom and prenatal diagnosis

We describe the morphologic variability and fetal diagnosis in pulmonary atresi with intact ventricular septum, within a chilean peadiatric cardiovacular center. An uncommon disease, that shows considerable morphologic heterogeneity, and with no local reports about this topic. We studied a consecutive series of 28 cases, in a five year period (1997-2002), at the Luis Calvo Mackenna Cardiovascular center. Morphologic feactures of each case were evaluated by direct review of the echocardiograms, angiocardiograms and from surgical reports. Among 28 infants, atresia was membranous in 82 per cent and muscular in 18 per cent. Right ventricle was bipartite in 30 per cent, unipartite in 6 per cent and tripartite in 64 per cent of cases. Coronary arterial abnormalities were identified in 36 per cent, and right ventricular dependent coronary circulation in 7,1 per cent. Median tricuspid annulus valvar size was -3. Fetal diagnosis was made in six of the 28 cases (21,4). In all of these prenatally diagnosed patients arrengements were made in order for the delivery to be fullfilled near by a cardiovascular center. This study shows data about the morphologic diversity and efforts on early fetal diagnose made in our country, in order asses this uncommon diseases