[Primary cardiac tumours in infancy]. / Tumores cardíacos primarios en la infancia.

INTRODUCTION: Primary cardiac tumours are very rare in the paediatric age, their incidence varies from 0.0017 % to 0.28 %. More than 90 % are benign in nature. The most common variety is the rhabdomyoma, present in over 60 % of cases with tuberous sclerosis. MATERIAL AND METHODS: We performed a retrospective analysis of medical records with a diagnosis of primary cardiac tumor between March 1977 and March 2007, finding a total of 27 patients. RESULTS: The age of initial diagnosis is more prevalent in the neonatal period, beginning with the discovery of a heart murmur (11 cases). There was no difference in gender distribution. In 14 patients were found cardiomegaly on chest radiograph. According to the echocardiography characteristics there were diagnosed 20 rhabdomyomas, 2 fibromas, 2 pericardial teratomas and 3 non classifiable tumours. Most were located in the left ventricle. Echocardiography, cardiac catheterization was also performed in 3 cases and angioresonance in 5 cases. During their evolution, episodes of arrhythmias were observed in 11 patients, 5 patients required some sort of surgical procedure, which confirmed the histopathology diagnosis. In 3 patients the initial cause of death was cardiological. The 75 % of cases with rhabdomyomas presented or developed tuberous sclerosis. In most of the rhabdomyomas (13 cases), there was a spontaneous regression. CONCLUSIONS: Firstly, there is shown to be a low prevalence of this disorder in children. Rhabdomyoma is the most common primary cardiac tumour in our study and it was associated in 75 % of cases with tuberous sclerosis. The diagnosis is more common in the early neonatal period after auscultation of a cardiac murmur and echocardiography, the diagnostic technique of choice, other imaging techniques, such as angioMRI not being of much for diagnosis in children. The emergence of foetal echocardiography allows early detection. The course is benign in most tumours, rhabdomyomas tending to regress spontaneously. It must be monitored as the occurrence of arrhythmias during its evolution will require medical treatment. Surgery is needed in cases with severe symptoms, due to obstruction in the ventricular output tracts. One option is the surgical cardiac transplant in non-resectable life-threatening tumours.

[Ibuprofen versus indomethacin in the treatment of patent ductus arteriosus in preterm infants]. / Ibuprofeno frente a indometacina en el tratamiento del conducto arterioso persistente del prematuro.

INTRODUCTION: Persistent patent ductus arteriosus (PDA) is a common entity in preterm infants. The most commonly used pharmacological treatment to close the ductus is indomethacin but it can affect cerebral, renal and mesenteric blood flow. Ibuprofen has recently been shown to be effective in closing PDA with fewer hemodynamic effects. In this study we compared the safety and efficacy of ibuprofen and indomethacin in the treatment of PDA in preterm infants. MATERIAL AND METHODS: A randomized trial was performed. Premature infants with symptomatic PDA confirmed by echocardiography in the first week of life and who required respiratory support were included. The patients were randomly assigned to receive either intravenous indomethacin or ibuprofen. The rate of ductal closure, need for additional treatment, complications, and clinical course were evaluated. RESULTS: Twenty-four patients were treated with indomethacin and 23 with ibuprofen. The clinical characteristics before treatment were similar in both groups. Both treatments were effective in closing PDA (87.5% in the indomethacin group and 82.6% in the ibuprofen group). The two cohorts did not differ in the rate of reopening, need for a second pharmacologic treatment, or surgical ductal ligation. No patient in the ibuprofen group developed gastrointestinal adverse effects, but two infants in the indomethacin group had isolated bowel perforation and one had necrotizing enterocolitis. Transient renal dysfunction developed in seven patients (29%) in the indomethacin group versus two (9%) in the ibuprofen group. Transient renal insufficiency was found in one patient in the indomethacin group and in none in the ibuprofen group. The rate of other complications was similar in both groups. CONCLUSIONS: In our trial ibuprofen was as effective as indomethacin in closing PDA. No significant differences were found in the incidence of complications but fewer renal complications and no gastrointestinal complications were found in the ibuprofen group.

[Palliative arterial switch]. / Switch arterial paliativo.

We report the first case published in Spain of a palliative anatomic correction associated with aortic arch repair in a neonate with (S,D,L)-transposition of the great arteries, multiple ventricular septal defects, and severe hypoplasia of the right ventricle with subaortic obstruction and hypoplasic aortic arch with coarctation. A one stage palliative surgery on cardiopulmonary bypass was performed with reconstruction of the aortic arch and an arterial switch procedure which obtained a satisfactory result. The principle of this operation is to switch the subaortic obstruction into a subpulmonary obstruction and reconstruct a large natural aortic root from the principal ventricle. The right ventricle-pulmonary artery continuity may promote growth of the right ventricle with the possibility of a future biventricular repair. We conclude that this operation, when used by surgical teams experienced with arterial switch surgery, is the best treatment for the complex newborn group with single ventricles or severe ventricular disbalance, ventriculoarterial discordance and stablished subaortic stenosis.

[Scimitar syndrome: series of 12 cases]. / Síndrome de la cimitarra: serie de 12 casos.

Our experience in 12 cases of pediatric patients with scimitar syndrome is reported. Except for one, all of them presented cardiac or respiratory manifestations. The symptomatology was related to associated defects (3 atrial septal defects and 1 multiple peripheric pulmonary stenosis), degree of pulmonary hypoplasia, size of the right to left shunt and pulmonary hypertension. 3 patients underwent surgical treatment. One of them died during operation and the other two have had a good evolution. Nine patients with later respiratory manifestations have improved their condition progressively without surgical intervention. Therapeutic approach for patients with scimitar syndrome, respiratory manifestations and onset beyond the neonatal period, should be conservative.

[Immature ovarian tumour and dilated myocardiopathy]. / Teratoma inmaduro de ovario y miocardiopatía dilatada.

Asymptomatic 2 month-old infant referred for evaluation of a hard abdominal mass on the left side. The ultrasound examination showed a solid-cystic tumour above the left kidney. The alpha-fetoprotein level was 2000ng/ml. The meta-iodobenzylguanidine (123-I-MIBG) showed no tumour uptake. At 48h, she showed signs of hypovolemic shock. The chest X-ray showed cardiomegaly with a cardiothoracic ratio of 0.7 and pulmonary congestion. The echocardiogram showed a dilated left ventricle with ejection fraction 35-40%. Anaemia, hypertension, hypervolemia and hyper-secretion of catecholamines were ruled out. The virology and metabolic screens were normal. The highly vascularised retroperitoneal tumour was resected without incident and confirmed the diagnosis of an immature Norris grade 2 teratoma grade. At 3 months the outcome was satisfactory. Teratomas are rare tumours in childhood that generally have a benign course. Dilated cardiomyopathy (DCM) secondary to, chromaffin cell tumours (phaeochromocytoma, neuroblastoma, ganglioneuroblastoma), leukaemia infiltrates, and treatment with anthracyclines have been described, but there is no case reported in the literature regarding a teratoma with dilated cardiomyopathy. Various cytokines, such as INF-α, IL-1, IL-6 may be secreted by tumour, promoting fibroblast activity in the heart and inducing apoptosis and myocardial fibrosis. Thus, in the case presented resection of the tumour mass responsible for this production, enables the heart to return to normal.

[Exercise test in exercise-induced asthma in children: study of delayed response]. / El test de esfuerzo en el asma inducido por el ejercicio en niños: estudio de la respuesta tardía.

In this study we perform the exercise broncho-provocation test according to a protocol and involving 27 children with allergic asthma to study the prevalence and distinctive traits of the late response in exercise-induced asthma (EIA). At the conclusion of the study, 20 patients (74%) were diagnosed of EIA through some of the tests employed (ergometric bicycle, free run). In the cases where both tests were negative, we reproduced in a controlled manner the exercise reported as asthmogenic on the basis of anamnesis; no positivity was observed for the test in any case. Prevalence of late asthmatic response was 40% (8/20); mean beginning time to this response was 4-6 hours, lasting less than 1 hour and with a mean intensity rate of 26.8% (measured as the percentage of PEFR drop according to the preexercise basal value). The results are discussed, comparing them with those of other authors and with the allergen provocation response pattern; in addition the methods used and therapeutic implications in view of the results are dealt with.

[Repercussion of risk factors associated with the development of chronic bronchial pathology on pulmonary function in children]. / Estudio de la repercusión de los factores de riesgo asociados al desarrollo de patología bronquial crónica en el funcionalismo pulmonar en una población infantil.

Of a total of 1,566 children, 1,416 of both sexes and aged 7 to 14 were subjected to functional respiratory exploration with a Vitalograph dry spirometer. The impact of tobacco smoke--as active and/or passive smokers--on the spirometric variables (FVC, FEV1, FEF2572, FEF50) evaluated by variance analysis (ANOVA) revealed no significant reductions. Asthma was the single antecedent of respiratory morbidity showing significant reductions in FEF2575, FEF50 among males alone; no significant reductions were observed for any of the other spirometric variables. No significant decreases were found in the spirometric variables on comparing two areas of the same city with the greatest difference in air pollution level.

[Basal pulmonary function studies (FVC, FEV) in a selected child population]. / Estudio de la función pulmonar basal (FVC, FEV1) en una población infantil de referencia.

This is a study of the ventilatory function (FVC, FEV1) by dry spirometer Vitalograph in 1566 children of both sexes with ages ranging from 7 to 14; 1156 children (73.6 per 100) were selected as reference population. Height was the biometric parameter with the greatest correlation to the functional variables studied in both sexes. Significant differences were observed in functional variables between male and female subjects. Multiple and simple linear prediction equations and percentiles tables for each sex are presented. The results of the present study are compared with those of previously published of children.

El cateterismo cardiaco como tratamiento de las cardiopatías congénitas / Cardiac catheterization as treatment for congenital heart diseases

Como resultado de los numerosos avances tecnológicos, un mayor número de cardiopatías congénitas son susceptibles de tratamiento en el laboratorio de hemodinámica. Las técnicas de imagen han mejorado tanto que han facilitado la selección de pacientes y el desarrollo de una gran variedad de dispositivos diseñados específicamente para su uso en niños. Esto significa que muchos pacientes podrían evitar la cirugía, y otros con cardiopatías complicadas podrían requerir un menor número de procedimientos quirúrgicos complejos. Otras ventajas del tratamiento percutáneo serían la más rápida recuperación y el acortamiento de la estancia hospitalaria de estos pacientes, así como la mejora de la calidad de vida a corto y medio plazo, aunque, por otro lado, aún quedan muchas incógnitas sobre los efectos a largo plazo de este tipo de procedimientos, de los que debemos hacer posteriores estudios de seguimiento (AU)

As a result of recent technological advances, more types of congenital heart disease are amenable to treatment in the cardiac catheter laboratory than ever before. Improved imaging techniques allow for better selection of patients and the development of a wide range of devices specifically for use in children means that many patients can avoid surgery altogether, while those with complex congenital heart disease may require fewer or less complex surgical procedures. This allows for a quicker recovery and a shorter hospital stay, and gives many patients an improved quality of life in the short to medium term. However, the long-term outcome for many of the newer forms of intervention is still unknown (AU)

Tumores cardíacos primarios en la infancia / Primary cardiac tumours in infancy

Introducción: Los tumores cardíacos primarios son muy poco frecuentes en la edad pediátrica, y su incidencia varía entre el 0,0017 y el 0,28 %. Más del 90 % son de naturaleza benigna. La variedad más frecuente es el rabdomioma, asociado en más del 60 % de los casos con esclerosis tuberosa. Material y métodos: Se realizó un análisis retrospectivo de historias clínicas con diagnóstico de tumor cardíaco primario entre marzo de 1977 y marzo de 2007, y se encontró a un total de 27 pacientes. Resultados: La edad de diagnóstico inicial es más frecuente en el período neonatal, a partir del hallazgo de un soplo cardíaco (11 casos). No hubo diferencias en la distribución por sexos. En 14 pacientes se encontró cardiomegalia en la radiografía de tórax. De acuerdo con las características ecocardiográficas, se diagnosticaron 20 rabdomiomas, 2 fibromas, 2 teratomas pericárdicos y 3 tumores no clasificables. La mayor parte se localizaron en el ventrículo izquierdo. Además de la ecocardiografía, se realizó cateterismo cardíaco en 3 casos y angiorresonancia en 5 casos. Durante su evolución se constataron episodios de arritmias en 11 pacientes. En 5 se requirió algún tipo de procedimiento quirúrgico, en los que se obtuvo el diagnóstico anatomopatológico. La mortalidad inicial de causa cardiológica fue de 3 pacientes. El 75 % de casos con rabdomiomas presentaba o desarrolló esclerosis tuberosa. En la mayoría de los rabdomiomas (13 casos) se produjo regresión espontánea. Conclusiones: En primer lugar, se evidencia la baja prevalencia de esta patología en la infancia. El rabdomioma es el tumor cardíaco primario más frecuente y en nuestro estudio se asoció en el 75 % de los casos a esclerosis tuberosa. El diagnóstico es más frecuente en el período neonatal precoz tras la auscultación de un soplo cardíaco y la ecocardiografía, la técnica diagnóstica de elección, mientras que no se ha encontrado utilidad de otras técnicas de imagen, como la angiorresonancia, para su diagnóstico en la población infantil. La aparición de la ecocardiografía fetal permite descubrirlos precozmente. El curso es benigno en la mayoría de los tumores, con tendencia a la regresión espontánea en los rabdomiomas. Debe vigilarse la aparición de arritmias durante su evolución, que precisará tratamiento médico. La cirugía es necesaria en los casos con síntomas graves, debidos a obstrucción en los tractos de salida ventricular. Una opción quirúrgica es el transplante cardíaco en caso de tumores irresecables con riesgo vital (AU)

Introduction: Primary cardiac tumours are very rare in the paediatric age, their incidence varies from 0.0017 % to 0.28 %. More than 90 % are benign in nature. The most common variety is the rhabdomyoma, present in over 60 % of cases with tuberous sclerosis. Material and methods: We performed a retrospective analysis of medical records with a diagnosis of primary cardiac tumor between March 1977 and March 2007, finding a total of 27 patients. Results: The age of initial diagnosis is more prevalent in the neonatal period, beginning with the discovery of a heart murmur (11 cases). There was no difference in gender distribution. In 14 patients were found cardiomegaly on chest radiograph. According to the echocardiography characteristics there were diagnosed 20 rhabdomyomas, 2 fibromas, 2 pericardial teratomas and 3 non classifiable tumours. Most were located in the left ventricle. Echocardiography, cardiac catheterization was also performed in 3 cases and angioresonance in 5 cases. During their evolution, episodes of arrhythmias were observed in 11 patients, 5 patients required some sort of surgical procedure, which confirmed the histopathology diagnosis. In 3 patients the initial cause of death was cardiological. The 75 % of cases with rhabdomyomas presented or developed tuberous sclerosis. In most of the rhabdomyomas (13 cases), there was a spontaneous regression. Conclusions: Firstly, there is shown to be a low prevalence of this disorder in children. Rhabdomyoma is the most common primary cardiac tumour in our study and it was associated in 75 % of cases with tuberous sclerosis. The diagnosis is more common in the early neonatal period after auscultation of a cardiac murmur and echocardiography, the diagnostic technique of choice, other imaging techniques, such as angioMRI not being of much for diagnosis in children. The emergence of foetal echocardiography allows early detection. The course is benign in most tumours, rhabdomyomas tending to regress spontaneously. It must be monitored as the occurrence of arrhythmias during its evolution will require medical treatment. Surgery is needed in cases with severe symptoms, due to obstruction in the ventricular output tracts. One option is the surgical cardiac transplant in non-resectable life-threatening tumours (AU)

Ductus arterioso persistente en el recién nacido pretérmino / Patent ductus arteriosus in preterm infants

El conducto arterioso persistente (DAP) complicala evolución de los niños prematuros. La incidenciade DAP es inversamente proporcional a la edad degestación. La clínica permite hacer un diagnósticode DAP pero por lo general de forma tardía, especialmenteen niños de peso extremadamente bajo alnacer, por lo que actualmente se utiliza la ecografíapara hacer un diagnóstico precoz. El tratamientoprecoz del ductus aumenta el porcentaje de éxitosde cierre y disminuye la morbilidad pulmonar. Tantoindometacina como ibuprofeno son válidos para eltratamiento médico. La cirugía está indicada en casode fracaso o contraindicación del tratamiento médico

A patent ductus arteriosus (PDA) complicates theclinical course of preterm infants. The incidence ofPDA is inversely proportional to the age of gestation.Symtomatic PDA is in general a late diagnosis, especiallyin infants of extremely low birth weight(ELBW), that is why nowadays we can use echocardiographicmarkers for an early diagnostic. Early treatmentimproves the outcome and decreases therisk of pulmonary morbidity. Indomethacin and ibuprofenboth are effective treatment to close a PDA.Surgical ligation of PDA is only preferred in case offailure or no possible medical treatment

Revisión de las técnicas de imagen en cardiología pediátrica. Un enfoque actualizado / Review of the imaging techniques in pediatric cardiology. An update approach

La cardiología pediátrica ha experimentado recientementegrandes avances en los campos dediagnóstico y tratamiento. Para planificar el tratamientoy la cirugía de las malformaciones cardiacascongénitas es esencial el conocimiento detallado desu anatomía y hemodinámica. Durante mucho tiempoel cateterismo cardiaco y la angiografía han sidolos únicos métodos para obtener dicha información.En los últimos 20 años se han desarrollado multitudde nuevos métodos de diagnóstico no invasivos. Elobjetivo de este artículo es describir la utilidad diagnósticade estas técnicas en la cardiología pediátrica

Pediatric cardiology has recently experienced highadvances in the diagnostic and therapeutical fields.To plan the treatment and surgery of congenital heartmalformations is essential a detailed knowledgeof anatomy and hemodynamics. For many years heartcatheterization and angiocardiography have beenthe only methods of obtaining this information. Duringthe past 20 years several non-invasive diagnosticmethods have been developed. This article aimsto describe the diagnostic utility of this tecniques inpediatric cardiology