Case report: Accelerated regression of giant cardiac rhabdomyomas in neonates with low dose everolimus.

Cardiac rhabdomyoma (CRHM) is the principal cardiac tumor in children and is most often associated with tuberous sclerosis complex (TSC). Mutations in the TSC1 and TSC2 genes cause the overactivation of the mammalian Target of Rapamycin (mTOR). This protein family is responsible for abnormal cell proliferation leading to the formation of CRHMs and hamartomas in other organs. Despite the tendency for spontaneous regression, some CRHMs can cause heart failure and intractable arrhythmias, requiring surgical resection. In recent years, the use of everolimus and sirolimus (mTOR inhibitors) in the treatment of CRHMs has been reported. We report two cases of neonates with giant rhabdomyomas, with hemodynamic repercussions treated with low-dose everolimus (4.5 mg/m2/week). In both cases, we obtained an approximate decrease of 50% in the total area of the mass after three weeks of treatment. Despite rebound growth after stopping the drug, we were able to evidence that the use of low doses of everolimus immediately after birth is effective and safe in the treatment of giant CRHMs, avoiding surgical resection of the tumor and associated morbidity and mortality.

Cor Triatriatum Dexter: an unusual cause of neonatal cyanosis / Cor Triatriatum Dexter: una causa infrecuente de cianosis neonatal

We present the case of a term newborn, with no significant perinatal history, who was taken to the emergency room at 18 days old for intermittent episodes of cyanosis, with no signs of respiratory distress, oxygensaturation of 85%, arterial gases with moderate hypoxemia, and chest X-ray.

Subclinical cardiac alterations detected by echocardiography in Mexican schoolchildren with overweight and obesity.

Objective: The purpose of this study was to evaluate early changes in myocardial function in overweight and obese children without hypertension. Methods: Cross-sectional study involving 150 participants of both sexes between 6 and 15 years old. Anthropometric and biochemical evaluations were performed. Ventricular function was assessed by conventional echocardiographic methods and myocardial deformation analysis by two-dimensional speckle tracking echocardiography. One-way analysis of variance was employed for the global comparison of study variables between groups (children with normal weight, overweight and obesity), and post hoc analysis with Bonferroni correction was used for multiple comparison, considering normal-weight children as the reference category. Results: Overall, 142 participants were included, 50 (35%) with normal weight, 39 (28%) overweight and 53 (37%) obesity. Diastolic diameter of the left ventricular (LV) and interventricular septum, diameter of the left atrium and LV mass were significantly higher in children with obesity compared to those with normal weight. No significant differences in the conventional indicators of LV systolic and diastolic function were found between groups. Significant differences in the regional myocardial deformation between the three groups were observed. Mean global longitudinal myocardial deformation was smaller in patients with obesity (-20.9% vs. -23.5%, p < 0.05) compared to children with normal weight. Conclusions: The childhood obesity was associated with altered myocardial deformation, even in the presence of normal ejection fraction. Myocardial deformation evaluation is relevant in the assessment of pediatric patients with obesity.

Objetivo: El propósito de este estudio fue evaluar los cambios tempranos en la función miocárdica en niños con sobrepeso y obesidad, sin hipertensión arterial. Métodos: Estudio transversal en el que se incluyeron 150 participantes de ambos sexos entre 6 y 15 años. Se realizaron evaluaciones antropométricas, bioquímicas y de función ventricular mediante métodos ecocardiográficos convencionales y análisis de deformación miocárdica con ecocardiografía bidimensional speckle tracking. La comparación global entre los grupos de estudio (niños con peso normal, sobrepeso y obesidad) se llevó a cabo con la prueba de análisis de varianza (ANOVA) de una vía y análisis post hoc con corrección de Bonferroni para las comparaciones múltiples, y se consideró a los niños con peso normal como grupo de referencia. Resultados: La muestra final fue de 142 participantes, 50 (35%) con peso normal, 39 (28%) con sobrepeso y 53 (37%) con obesidad. El diámetro diastólico del ventrículo izquierdo (VI) y el septum interventricular, y el diámetro de la aurícula izquierda (AI) y la masa del VI fueron significativamente más altos en el grupo con obesidad en comparación con el grupo con peso normal. No se observaron diferencias significativas en los indicadores convencionales de la función sistólica y diastólica ventricular izquierda. Se observaron diferencias significativas en la deformación miocárdica regional entre los tres grupos. La media de deformación miocárdica longitudinal global fue más baja en los pacientes con obesidad (−20.9% vs. −23.5%; p < 0.05) en comparación con los niños con peso normal. Conclusiones: La obesidad infantil se asoció a alteraciones en la deformación miocárdica, incluso en presencia de fracción de expulsión normal. La evaluación de la deformación miocárdica es relevante en los pacientes pediátricos con obesidad.

Subclinical cardiac alterations detected by echocardiography in Mexican schoolchildren with overweight and obesity.

Objective: The purpose of this study was to evaluate early changes in myocardial function in overweight and obese children without hypertension. Methods: This was a cross-sectional study involving 150 participants of both sexes between 6 and 15 years old. Anthropometric and biochemical evaluations were performed. Ventricular function was assessed by conventional echocardiographic methods and myocardial deformation analysis by two-dimensional speckle tracking echocardiography. One-way analysis of variance was employed for the global comparison of study variables between groups (children with normal weight, overweight, and obesity), and post hoc analysis with Bonferroni correction was used for multiple comparisons, considering normal weight children as the reference category. Results: Overall, 142 participants were included, 50 (35%) with normal weight, 39 (28%) overweight, and 53 (37%) obesity. The diastolic diameter of the left ventricular and interventricular septum, and diameter of the left atrium and LV mass were significantly higher in children with obesity compared to those with normal weight. No significant differences in the conventional indicators of LV systolic and diastolic function were found between groups. Significant differences in the regional myocardial deformation between the three groups were observed. Mean global longitudinal myocardial deformation was smaller in patients with obesity (-20.9% vs. -23.5%, p < 0.05) compared to children with normal weight. Conclusions: The childhood obesity was associated with altered myocardial deformation, even in the presence of normal ejection fraction. Myocardial deformation evaluation is relevant in the assessment of pediatric patients with obesity.

Objetivo: El propósito de este estudio fue evaluar los cambios tempranos en la función miocárdica en niños con sobrepeso y obesidad, sin hipertensión arterial. Métodos: Estudio transversal en el que se incluyeron 150 participantes de ambos sexos entre 6 y 15 años. Se realizaron evaluaciones antropométricas, bioquímicas y de función ventricular mediante métodos ecocardiográficos convencionales y análisis de deformación miocárdica con ecocardiografía bidimensional speckle tracking. La comparación global entre los grupos de estudio (niños con peso normal, sobrepeso y obesidad) se llevó a cabo con la prueba de análisis de varianza (ANOVA) de una vía y análisis post hoc con corrección de Bonferroni para las comparaciones múltiples, y se consideró a los niños con peso normal como grupo de referencia. Resultados: La muestra final fue de 142 participantes, 50 (35%) con peso normal, 39 (28%) con sobrepeso y 53 (37%) con obesidad. El diámetro diastólico del ventrículo izquierdo (VI) y el septum interventricular, y el diámetro de la aurícula izquierda (AI) y la masa del VI fueron significativamente más altos en el grupo con obesidad en comparación con el grupo con peso normal. No se observaron diferencias significativas en los indicadores convencionales de la función sistólica y diastólica ventricular izquierda. Se observaron diferencias significativas en la deformación miocárdica regional entre los tres grupos. La media de deformación miocárdica longitudinal global fue más baja en los pacientes con obesidad (−20.9% vs. −23.5%; p < 0.05) en comparación con los niños con peso normal. Conclusiones: La obesidad infantil se asoció a alteraciones en la deformación miocárdica, incluso en presencia de fracción de expulsión normal. La evaluación de la deformación miocárdica es relevante en los pacientes pediátricos con obesidad.

Outcomes of Delayed Sternal Closure in Pediatric Heart Surgery: Single-Center Experience.

BACKGROUND: Delayed sternal closure (DSC) after cardiac surgery is a therapeutic option in the treatment of the severely impaired heart in pediatric cardiac surgery. METHODS: A single-center retrospective review of all bypass surgeries performed over a 10-year period (2003-2012). RESULTS: Of a total of 2325 patients registered in our database, the DSC group included 259 cases (11%), and the remaining 2066 cases (89%) constituted the control group (PSC). RACHS-1 risk was higher for the DSC group (74% had a score of 3 or 4) than for the PSC group (82% had a score of 2 or 3). The most frequent diagnosis for the DSC group was transposition of the great arteries (28%). We found out that hemodynamic instability was the main indication observed in patients aged ≤ 8 years (63%), while bleeding was the principal indication for patients aged ≥ 8 years (94%) (p ≤ 0.001). The average time between surgery and sternal closure was 2.3 ± 1.4 days. Overall mortality rates were higher for patients of the DSC group (22%) than for the PSC group (8.7%) (OR: 0.4 (95% CI: 0.4 to 0.5), p < 0.05). There were six patients with DSC who developed mediastinitis (2.3%). The risk of mediastinitis was significantly higher when DSC was performed 4 days after the primary surgery. CONCLUSIONS: DSC is an important management strategy for congenital cardiac surgery in infants and children. The prolonged sternal closure time is associated with an increased rate of postoperative mediastinitis.

[Risk factors related with pediatric accidents in a children's hospital in Bogotá]. / Factores de riesgo relacionados con accidentes pediátricos en un hospital infantil de Bogotá.

OBJECTIVE: To describe the biophysical and sociocultural risk factors related to the accident rate in pediatric patients. METHOD: Cross-sectional descriptive study. Data collection through the direct application of surveys. RESULTS: 308 surveys were analyzed, obtained between the months of february and april, 2010. Adolescents were the most affected group (33.5 %); the male gender registered the highest number of traumas (64.7 %). The main family type was nuclear 52 %, 34.9 % of patients belonged to stratum 2 and 46,6 % were under the care of their mothers when the accident occurred. 84 % of caretakers had over 5 years study, 47 % considered that the most unsafe place for a child is a public road, and 76.8 % stated having never received information on accident prevention. The most frequent accident was a fall from a height (54.7 %) and home was the place with highest number of traumas (51 %). DISCUSSION: Demographic features and sociocultural factors of this study differ from reports from large studies of non-intentional lesions.

Alteraciones cardíacas subclínicas detectadas por ecocardiografía en escolares Mexicanos con sobrepeso y obesidad / Subclinical cardiac alterations detected by echocardiography in Mexican schoolchildren with overweight and obesity

Resumen Objetivo: El propósito de este estudio fue evaluar los cambios tempranos en la función miocárdica en niños con sobrepeso y obesidad, sin hipertensión arterial. Métodos: Estudio transversal en el que se incluyeron 150 participantes de ambos sexos entre 6 y 15 años. Se realizaron evaluaciones antropométricas, bioquímicas y de función ventricular mediante métodos ecocardiográficos convencionales y análisis de deformación miocárdica con ecocardiografía bidimensional speckle tracking. La comparación global entre los grupos de estudio (niños con peso normal, sobrepeso y obesidad) se llevó a cabo con la prueba de análisis de varianza (ANOVA) de una vía y análisis post hoc con corrección de Bonferroni para las comparaciones múltiples, y se consideró a los niños con peso normal como grupo de referencia. Resultados: La muestra final fue de 142 participantes, 50 (35%) con peso normal, 39 (28%) con sobrepeso y 53 (37%) con obesidad. El diámetro diastólico del ventrículo izquierdo (VI) y el septum interventricular, y el diámetro de la aurícula izquierda (AI) y la masa del VI fueron significativamente más altos en el grupo con obesidad en comparación con el grupo con peso normal. No se observaron diferencias significativas en los indicadores convencionales de la función sistólica y diastólica ventricular izquierda. Se observaron diferencias significativas en la deformación miocárdica regional entre los tres grupos. La media de deformación miocárdica longitudinal global fue más baja en los pacientes con obesidad (−20.9% vs. −23.5%; p menor 0.05) en comparación con los niños con peso normal. Conclusiones: La obesidad infantil se asoció a alteraciones en la deformación miocárdica, incluso en presencia de fracción de expulsión normal. La evaluación de la deformación miocárdica es relevante en los pacientes pediátricos con obesidad.

Abstract Objective: The purpose of this study was to evaluate early changes in myocardial function in overweight and obese children without hypertension. Methods: Cross-sectional study involving 150 participants of both sexes between 6 and 15 years old. Anthropometric and biochemical evaluations were performed. Ventricular function was assessed by conventional echocardiographic methods and myocardial deformation analysis by two-dimensional speckle tracking echocardiography. One-way analysis of variance was employed for the global comparison of study variables between groups (children with normal weight, overweight and obesity), and post hoc analysis with Bonferroni correction was used for multiple comparison, considering normal-weight children as the reference category. Results: Overall, 142 participants were included, 50 (35%) with normal weight, 39 (28%) overweight and 53 (37%) obesity. Diastolic diameter of the left ventricular (LV) and interventricular septum, diameter of the left atrium and LV mass were significantly higher in children with obesity compared to those with normal weight. No significant differences in the conventional indicators of LV systolic and diastolic function were found between groups. Significant differences in the regional myocardial deformation between the three groups were observed. Mean global longitudinal myocardial deformation was smaller in patients with obesity (−20.9% vs. −23.5%, p less 0.05) compared to children with normal weight. Conclusions: The childhood obesity was associated with altered myocardial deformation, even in the presence of normal ejection fraction. Myocardial deformation evaluation is relevant in the assessment of pediatric patients with obesity.

Factores de riesgo relacionados con accidentes pediátricos en un hospital infantil de Bogotá / Risk factors related with pediatric accidents in a children's hospital in Bogotá

Objetivo Describir los factores de riesgo biofísicos y socioculturales relacionados con la accidentalidad del paciente pediátrico. Método Estudio descriptivo de corte transversal. Recolección de los datos a través de aplicación de encuestas en forma directa. Resultados Se analizaron 308 encuestas, diligenciadas entre los meses de febrero a abril del año 2010. Los adolescentes fueron el grupo más afectado (33,5 %), el género masculino registró el mayor número de traumas (64,7 %). El principal tipo de familia fue el nuclear 52 %, el 34,9 % de los pacientes pertenecían al estrato 2 y el 46,6 % se encontraba al cuidado de la madre al momento del accidente. El 84 % de los cuidadores tenían más de 5 años de estudio, el 47 % considero que el sitio más inseguro para un niño es la vía pública y el 76,8 % manifestó nunca haber recibido información sobre prevención de accidentes. El accidente más frecuente fue la caída de altura 54,7 % y el sitio con mayor número de traumas fue el hogar 51 %. Discusión Las características demográficas y factores socioculturales del presente estudio difieren de los reportes de grandes estudios de lesiones no intencionales.

Objective To describe the biophysical and sociocultural risk factors related to the accident rate in pediatric patients. Method Cross-sectional descriptive study. Data collection through the direct application of surveys. Results 308 surveys were analyzed, obtained between the months of february and april, 2010. Adolescents were the most affected group (33.5 %); the male gender registered the highest number of traumas (64.7 %). The main family type was nuclear 52 %, 34.9 % of patients belonged to stratum 2 and 46,6 % were under the care of their mothers when the accident occurred. 84 % of caretakers had over 5 years study, 47 % considered that the most unsafe place for a child is a public road, and 76.8 % stated having never received information on accident prevention. The most frequent accident was a fall from a height (54.7 %) and home was the place with highest number of traumas (51 %). Discussion Demographic features and sociocultural factors of this study differ from reports from large studies of non-intentional lesions.