Search

Antecedentes: Existe cierta variabilidad de práctica clínica en España sobre las intervenciones preventivas para evitar la infección respiratoria infantil por el virus respiratorio sincitial (VRS), entre las que se incluyen medidas higiénicas e inmunoprofilaxis intramuscular con palivizumab. Esta tarea implica varias especialidades pediátricas, que pueden tener distinta percepción de la magnitud del problema y distinto criterio profesional sobre las actuaciones apropiadas. Objetivos: Desarrollar un consenso multidisciplinar español sobre la prevención de la infección por VRS, con participación de las sociedades científicas pediátricas implicadas: Sociedad Española de Neonatología (SEN), Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas (SECPCC), Sociedad Española de Neumología Pediátrica (SENP) y Sociedad Española de Hematología y Oncología Pediátrica (SEHOP). Métodos: Consenso Delphi modificado en dos rondas. El estudio se efectuó en cuatro fases: 1) constitución de un comité científico multidisciplinar, a propuesta de las sociedades científicas participantes, para la revisión bibliográfica y formulación de las recomendaciones a debate; 2) constitución de un panel experto con 77 representantes de las especialidades; 3) encuesta postal en dos rondas con procesamiento intermedio de opiniones e informe a los panelistas, y 4) discusión de resultados en sesión presencial del comité científico. Resultados: Se consensúan 48 de las 57 recomendaciones preventivas analizadas. En las nueve cuestiones restantes no se consigue acuerdo, bien por disparidad de opinión profesional, bien por falta de criterio establecido en la mayoría de los expertos. Sólo en tres recomendaciones, la opinión de los expertos se asocia a la especialidad de procedencia. Conclusiones: Se presenta una lista de recomendaciones profilácticas anti-VRS, cualificadas según el grado de acuerdo profesional en que se sustentan, que pueden considerarse vigentes hasta la aparición de nueva información científica que indique su revisión. : Background: A certain degree of feasibility exists in Spanish clinical practice with respect to interventions performed to prevent paediatric respiratory infection by RSV, including hygienic measures and intramuscular immunoprophylaxis with palivizumab. This task involves different paediatric specialties that may have a different perception of the magnitude of the problem and different professional criteria regarding the most appropriate actions. Objectives: To develop Spanish multidisciplinary consensus on preventing infection by RSV with the participation of the paediatric scientific societies involved (SNS, SSPC, SSPP and SSHPO). Methods: Delphi Consensus modified in two rounds was used. The study was conducted in four phases: 1) constitution of a multidisciplinary Scientific Committee at the recommendation of the scientific entities participating in the study, for bibliographic review and submission of the recommendations to discussion; 2) constitution of an Expert Panel with 77 speciality representatives; 3) postal survey organised in two rounds and intermediate processing of opinions and issuing of a report for the panellists, and 4) discussion of the results in a meeting of the Scientific Committee. Results: Consensus was reached on 48 of the 57 preventive recommendations analysed. With respect to the 9 remaining issues, no consensus was reached, due to differences in professional opinion and the absence of established criteria among the majority of the experts. Only in 3 recommendations was the opinion of the experts associated with the speciality of origin. Conclusions: A list of anti-RSV prophylactic recommendations was submitted, rated in accordance with the degree of professional consensus on which they were based. These can be considered valid until such time as new scientific information emerges that warrants a review thereof.

Objetivos: Analizar los resultados del intervencionismo no quirúrgico sobre la recoartación y coartación de aorta, valorando los resultados de cada técnica, las complicaciones y su evolución Pacientes y métodos: Los tratamientos fueron: 51 dilatados con balón por recoartación (86,5%), dos en los que se dilató una coartación nativa (3,3 %) y seis en los que se ha implantado un tubo expansible (stent) por recoartación (10 %). Las edades oscilan entre los 2 y los 236 meses (109 ± 63,45 meses), con un tiempo de seguimiento entre 1 y 156 meses (38,87 ± 32,96 meses) Resultados: El gradiente predilatación en los niños recoartados fue de 34 ± 11,62 mmHg, que descendió a 11 ± 5,38 mmHg (p < 0,0001). Doce casos (20,3 %) no obtuvieron una dilatación eficaz. El tamaño de la estenosis fue de 6,7 ± 2,35mm predilatación y aumentó a 9,3 ± 3,10 mm (p < 0,0001) con un incremento porcentual del 50,97. Las complicaciones fueron escasas. Seis niños precisaron redilatación posterior. La experiencia con stent muestra un gradiente medio de 32,83 ± 10,62 mmHg que descendió a 7,3 ± 3,8 mmHg (p < 0,0001) con una relación globo-estenosis de 1,94 Conclusiones: La técnica intervencionista es muy eficaz, tanto en coartación nativa como en recoartación de aorta, en resultados inmediatos y durante la evolución, y con escasas complicaciones. Los stents en niños mayores obtienen igualmente excelentes resultados, abriendo un futuro esperanzador : Objectives: To analyze the results of nonsurgical treatment of aortic coarctation and recoarctation by evaluating the results of each technique, and its complications and outcome Patients and methods: The results were as follows: 51 children underwent balloon dilatation due to recoarctation (86.5 %); two underwent dilation of a native coarctation (3.3 %) and six underwent stent implantation for recoarctation (10 %). Age ranged from 2 to 236 months (109 ± 63.45 months) with a follow-up of between 1 and 156 months (38.87 ± 32.96 months) Results: The mean predilatation gradient in children with recoarctation was 34 ± 11.62 mmHg, which decreased to 11 ± 5.38 mm Hg (p < 0.0001). In 12 patients (20.3 %) effective dilatation was not achieved. The size of the stenosis was 6.7 ± 2.35 mm predilatation, which increased to 9.3 ± 3.10 mm (p < 0.0001) after dilatation with a percentage increase of 50.97. There were very few complications. Six children required subsequent redilatation. The experience with stent showed a mean gradient of 32.83 ± 10.62 mm Hg, which decreased to 7.3 ± 3.8 mm Hg (p < 0.0001) with a balloon/stenosis ratio of 1.94 Conclusions: We conclude that the interventionist technique is highly effective in both native coarctation and recoarctation in the short term, as well as subsequently, with very few complications. The stent technique produces equally good results in older children, which is promising for the future

Objetivos: Evaluar los resultados inmediatos obtenidos mediante valvuloplastia aórtica con balón en el período neonatal, así como la evolución ecocardiográfica a largo plazo, identificando variables predictivas en relación a la evolución. Identificar el grado de concordancia entre los estudios hemodinámicos y ecocardiográficos. Pacientes y métodos: Se analizaron los resultados obtenidos en 26 procedimientos realizados en una población compuesta por 18 varones y 8 mujeres (2,25:1) cuyas edades fueron de 2 a 49 días (16,1 ± 12,9 días). Todos fueron estudiados e intervenidos en el Servicio de Cardiología Pediátrica del Hospital Ramón y Cajal de Madrid, entre junio de 1989 y junio de 2001. El tiempo de seguimiento osciló entre 0 y 144 meses (39,5 ± 39,7 meses). Resultados: Los efectos inmediatos observados mediante el estudio ecocardiográfico fueron un descenso significativo en el gradiente Doppler máximo (de 77,8 a 32,4 mmHg; p < 0,0001) y del gradiente Doppler medio (de 41,7 a 18,5 mmHg, p < 0,05). Los estudios hemodinámicos reflejaron un descenso significativo de la presión sistólica del ventrículo izquierdo (de 119,8 a 82,8 mmHg, p < 0,0001) y un aumento de la presión sistólica aórtica (de 56,8 a 66,6 mmHg, p < 0,007). El gradiente pico hemodinámico descendió de 63,1 a 17,7 mmHg, p < 0,0001). La insuficiencia aórtica se incrementó de manera significativa en el 23 % de los casos.El seguimiento ecocardiográfico demostró un aumento significativo del tamaño telediastólico del ventrículo izquierdo (VIDD) y una reducción del gradiente Doppler máximo y medio. El porcentaje de éxito inicial fue del 68,7 % y el análisis de supervivencia libre de intervención sobre la válvula se mostró del 65,8 % a los 45 meses. El análisis de riesgos proporcionales demostró que el gradiente posvalvuloplastia resultante de la intervención era un factor predictivo de posteriores intervenciones sobre la válvula. : Objectives: To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study. Patients and methods: We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 ± 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 ± 39.7 months). Results: The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p < 0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p < 0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p < 0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p < 0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p < 0.0001. In 23 % of the patients, aortic insufficiency significantly increased.Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7% and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.

Recibido en febrero de 1999. Aceptado para su publicacion en febrero de 2000. INTRODUCCION Las cardiopatias congenitas complejas exigen, en su gran mayoria, un numero considerable de estudios hemodinamicos diagnosticos y terapeuticos durante su evolucion natural o modificada por diferentes intervenciones quirurgicas indispensables para paliar y/o corregir el/los defectos nativos o residuales. Asimismo, es frecuente en el postoperatorio, la necesidad de introducir y mantener cateteres venosos centrales durante prolongados periodos de tiempo. Una consecuencia habitual de tales procedimientos es la trombosis venosa y, por tanto, la progresiva limitacion de las rutas de acceso convencionales hacia el sistema venoso central; bajo estas condiciones es preciso considerar una via de abordaje alternativa. El acceso percutaneo de la vena cava inferior (VCI), tanto por via translumbar como transhepatica, se ha demostrado seguro y eficaz cuando se hace indispensable administrar medicacion, quimioterapia o nutricion parenteral. Recientemente, la via venosa transhepatica ha sido utilizada en enfermos pediatricos para la realizacion de cateterismo cardiaco diagnostico o intervencionista (abordaje percutaneo transhepatico [APT])1-4. El interes de esta comunicacion esta centrado en describir la novedosa tecnica de APT practicada en un enfermo pediatrico portador de cardiopatia congenita compleja.

A certain degree of feasibility exists in Spanish clinical practice with respect to interventions performed to prevent paediatric respiratory infection by RSV, including hygienic measures and intramuscular immunoprophylaxis with palivizumab. This task involves different paediatric specialties that may have a different perception of the magnitude of the problem and different professional criteria regarding the most appropriate actions.To develop Spanish multidisciplinary consensus on preventing infection by RSV with the participation of the paediatric scientific societies involved (SNS, SSPC, SSPP and SSHPO).Delphi Consensus modified in two rounds was used. The study was conducted in four phases: 1) constitution of a multidisciplinary Scientific Committee at the recommendation of the scientific entities participating in the study, for bibliographic review and submission of the recommendations to discussion; 2) constitution of an Expert Panel with 77 speciality representatives; 3) postal survey organised in two rounds and intermediate processing of opinions and issuing of a report for the panellists, and 4) discussion of the results in a meeting of the Scientific Committee.Consensus was reached on 48 of the 57 preventive recommendations analysed. With respect to the 9 remaining issues, no consensus was reached, due to differences in professional opinion and the absence of established criteria among the majority of the experts. Only in 3 recommendations was the opinion of the experts associated with the speciality of origin.A list of anti-RSV prophylactic recommendations was submitted, rated in accordance with the degree of professional consensus on which they were based. These can be considered valid until such time as new scientific information emerges that warrants a review thereof.

To analyze the results of nonsurgical treatment of aortic coarctation and recoarctation by evaluating the results of each technique, and its complications and outcome.The results were as follows: 51 children underwent balloon dilatation due to recoarctation (86.5 %); two underwent dilation of a native coarctation (3.3 %) and six underwent stent implantation for recoarctation (10 %). Age ranged from 2 to 236 months (109 +/- 63.45 months) with a follow-up of between 1 and 156 months (38.87 +/-32.96 months).The mean predilatation gradient in children with recoarctation was 34 +/- 11.62 mmHg, which decreased to 11 +/- 5.38 mm Hg (p0.0001). In 12 patients (20.3 %) effective dilatation was not achieved. The size of the stenosis was 6.7 +/- 2.35 mm predilatation, which increased to 9.3 +/- 3.10 mm (p0.0001) after dilatation with a percentage increase of 50.97. There were very few complications. Six children required subsequent redilatation. The experience with stent showed a mean gradient of 32.83 +/- 10.62 mm Hg, which decreased to 7.3 +/- 3.8 mm Hg (p0.0001) with a balloon/stenosis ratio of 1.94.We conclude that the interventionist technique is highly effective in both native coarctation and recoarctation in the short term, as well as subsequently, with very few complications. The stent technique produces equally good results in older children, which is promising for the future.

To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study.We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 39.7 months).The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p0.0001. In 23 % of the patients, aortic insufficiency significantly increased. Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7 % and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.

To analyze the efficacy of balloon pulmonary valvuloplasty (BPV) as the elective treatment for neonatal critical pulmonary valvar stenosis (PVS).The results of clinical and echocardiographic features before and after the BPV were reviewed in 29 neonates (18+/-12 days of life). Different hemodynamic and 2-D color Doppler echocardiographic were evaluated. The BPV result was classified as favourable if no other balloon or surgical therapy was required to normalise pulmonary flow and achieve a sustained right ventricle-pulmonary artery (RV-PA) Doppler gradient below 40 mmHg. It was considered unfavourable if the neonate died, needed surgery or redilation and/or the RV-PA Doppler gradient wasor =40 mm Hg. The study developed in three phases: pre BPV immediate post BPV until the hospital discharge (14+/-11 days), and in the mid-term follow-up of between 8 and 96 months (51+/-31 months).Mortality was not registered with BPV. The RV/left ventricular systolic pressure decreased from 1.4+/-0.3 to 0.8+/-0.3 (p0.01) as a consequence of the dilation, and the the systemic oxygen saturation increased from 85 +/-12 to 92+/-6% (p0.01). The RV-PA Doppler gradient diminished from 86+/-18 to 28+/-16 mm Hg immediately after BPV (p0.01) and was registered at 13+/-6 mm Hg in the follow-up (n = 24). The RV-PA junction Z value grew from -1.25+/-0.9 before valvuloplasty to -0.51 +/-0.7 at the final echocardiogram (p0.01). No changes in the tricuspid diameter were detected between both periods of time. Five neonates obtained unsatisfactory results: 4 in the immediate post BPV (systemic-pulmonary artery shunt 2, transannular patch 2), and 1 in the mid-term follow-up (valvectomy + transannular patch). The actuarial curve reflects that 82,7% of the patients were free form reinterventions at 8 years.BPV is safe and effective to relief PVS in the neonate. The balloon promotes advantageous changes in both, pulmonary annulus and the right ventricle. In addition, the RV-PA Doppler gradient observations in the follow-up, support the expectation that the BPV is a "curative" therapy.

In spite of progressive improvement not only in myocardial protection but also in anesthetic and surgical techniques, the repair of complex congenital heart defects can still lead to cardiopulmonary compromise refractory to conventional treatment. We describe two patients in whom successful surgical repair of congenital heart defects was followed by severe heart failure refractory to medical treatment. Both patients were managed by extracorporeal membrane oxygenation (ECMO). We discuss the indications, contraindications and use of ECMO in the postoperative intensive care unit.

To study medium-term follow-up in children with pulmonary stenosis (PS) with percutaneous balloon pulmonary valvuloplasty (PBVP).Medium-term follow-up results of 106 consecutive PBVP procedures in 100 patients of the same health center are presented. The mean age of the patients was 61.3 +/- 5.1 months, 31 of them with severe PS.After PBVP, and once infundibular contraction ceased, the procedure was defined as successful in 85 children. PBVP was repeated in five patients, achieving satisfactory results in 3. Eight patients suffered from re-stenoses and surgery was performed in 7 of them. The other 12 patients in which PBVP failed underwent surgery. Follow-up of these 88 patients showed a cessation in clinical symptomatology at a mean of 10 +/- 0.7 months. Five-year actuarial probability of achieving a normal EKG axis was 61.7 +/- 9.1%. However, neither ECG nor chest radiology were useful in the diagnosis of high residual gradients. Pulmonary regurgitation was observed in 92% of the children, mild in all of them. The residual transpulmonary gradient obtained immediately after PBVP was the only variable that affected long-term results.Our results confirm the medium-term success of PBVP in the treatment of PS. The five year actuarial probability of restenosis is 12.4%. Mild pulmonary regurgitation is observed in more than 90% of patients.

The stent has demonstrated to be a useful device in the prevention of postangioplasty coronary restenosis and it is expected to have a favourable effect as an alternative or complementary treatment of stenotic lesions in arteries or veins associated with congenital defects. The aim of this study is to analyze our experience in this setting.From February 1992 to March 1996, 28 stenting procedures were performed in 26 patients (mean age: 8.6 +/- 0.7 years; mean weight: 26.2 +/- 3 kg). In 12 patients, stenting was single, and a iliac Palmaz stents were always used. Stenting location was: pulmonary artery branches in 17 patients, right ventricular outflow in 2 patients, in the junction of right atrium with pulmonary artery in 2 patients, systemic veins in 2 patients and in post Mustard intratrial channel stenosis in 2 patients. 25 patients had previously undergone at least one surgical procedure.The stenotic diameter of the treated lesions increased significantly after the procedure (4.4 +/- 0.3 mm before stenting vs 11.6 +/- 0.3 mm after stenting, p0.0001) and the transtenotic gradient decreased from 38.1 +/- 5 to 12 +/- 3.8 mmHg. Those changes were associated with a diminution of right ventricular pressure (81.6 +/- 3 vs 56.7 +/- 6 mmHg, p0.0001) in patients with pulmonary branch stenosis without septal defects. There was no mortality among the percutaneously treated patients and only one patient needed surgery. Nevertheless, one patient died after bilateral intraoperative stenting.The treatment of proximal or distal stenotic lesions in the pulmonary tree, systemic veins, and obstructed intraatrial channels with stents, can replace or complement conventional balloon angioplasty. It also offers a useful and effective alternative to surgery, when it is impossible or carries a risk.

Aortico-left ventricular tunnel is an unusual cardiac anomaly. The main clinical feature is early, severe aortic regurgitation, and surgical management is mandatory. Exceptionally this defect is associated with pulmonary valve stenosis. A case of a newborn with aortico-left ventricular tunnel plus pulmonary valve stenosis is reported. Initially she underwent percutaneous pulmonary valvuloplasty during diagnostic cardiac catheterization and with surgical closure of the tunnel later.

A comprehensive review of all the present therapeutic possibilities in Fallot's tetralogy is presented: A) From the cardiological viewpoint: Pharmacological and Interventional procedures (Baloon dilatation, coil embolisation, pulmonary artery stenting and ablation procedures) are discussed. B) From the surgical viewpoint: Palliative and intracardiac corrective procedures are reviewed. Indications for surgery are evaluated, including primary versus two stage procedures. Postsurgical sequelae are described: both early and late and the need for reoperation. Finally, late post-op complications, such as arrhythmias and sudden death, are commented on among the long term follow-up.

Foreign body retrieval in the catheter room is a useful procedure at any age, but, although its interest, few reports of such technique have been reported in children.We review and present our experience in 8 children, aged 5 days to 11 years, five of them having congenital hearts defects.We retrieved 4 catheter fragments, 2 endocardial electrode catheter tips, 1 Rashkind 12 mm. PDA umbrella, and 1 detachable Jackson coil. All of them were placed in systemic veins, right heart chambers or pulmonary arteries. We used biplane fluoroscopy and percutaneous right femoral vein puncture in all cases. Goose-Neck (Microvena Corporation) snares were used in 5 patients, hand made snares in 2 and a Swan-Ganz catheter in one. In 4 cases, the snare was introduced trough a Mullins long sheath and the foreign body pulled into its distal end, in order to bring it out of the femoral vein. Six foreign bodies came off the femoral vein: 4 trough the puncture site and 2 needing a venous cut-down. The two remaining foreign bodies, stopped while pulling at the common iliac vein and a minor surgical procedure was needed for final extraction.Therapeutic catheterization is the technique of choice for intravascular foreign body retrieval in children.

We present our experience with four cases of unilateral pulmonary agenesis, 3 of the right artery and the other one of the left. Two of the patients had pulmonary hypertension and one of them died at two years of age. All cases were diagnosed by using pulmonary angiography. Aortography demonstrated the absence of circulatory supply to the lung and a contralateral aortic arch to the absent pulmonary artery. We have performed a review of the literature with an emphasis on the diagnostic aspects of this condition, and discuss the considerations for possible surgery in symptomatic patients.

Between february and december 1990, we attempted percutaneous closure of patent ductus arteriosus (PDA) in 26 patients and in one ostium secundum atrial septal defect (ASD). One of the PDA was right sided with right aortic arch and two were post surgical leaks. We used the "USCI-Rashkind PDA double disc occluder" and the newly designed "Lock Clamshell" occluder for the ASD. Twenty seven patients (20 females and 7 males), aged 1.3 to 16 years (M = 5.6) and weighing 9.5 to 56 kg (M = 21.3), were studied. Diagnosis was made clinically with the aid of Doppler colour flow echocardiography, with follow-up studies at 24 hours, 6 months and 1 year after the procedure. Diameter of the ductus varied from 1.6 to 8.2 mm (M = 2.6). Successful implant of the device was achieved in 25 cases (95%), of which 18 (72%) were totally occluded at 24 hours and 21 (84%) after 6 months, remaining small residual leaks in four (16%). In only one patient total occlusion was demonstrated 1 year after device implantation. We achieved 22 (88%) total occlusions. One case was not possible to occlude, due to its large size. Angiocardiography demonstrated adequate anatomy in 18, and difficult in 8 patients. Twelve and 17 mm devices were used in 16 and 9 patients, respectively. Successful second implant with subsequent total occlusion was achieved in one patient with a previous moderate residual leak. The ASD was central and single, with a diameter of 20 mm. Immediate total occlusion was achieved. We conclude that percutaneous PDA closure is a safe and effective procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Un garcon de 6 ans s'est presente avec une douleur epigastrique et des vomissements evoluant depuis 1 an. La radiographie thoracique et le transit œso-gastro-duodenal retrouvaient une masse mediastinale. L'angioscanner revela un anevrysme de l'aorte thoracique descendante. Les tests sanguins effectues etaient tous negatifs. L'anevrysme fut traite chirurgicalement en utilisant un patch en Dacron. L'etude anatomopathologique demontra des lesions d'atherosclerose avec des calcifications, compatibles avec un anevrysme atherosclereux. Les anevrysmes sont rares au sein de la population pediatrique. Habituellement, aucune etiologie n'est identifiee, et ces cas sont definis comme idiopathiques. Une chirurgie directe avec ou sans mise en place d'un patch est un choix therapeutique valable dans de telles situations et ne nuit pas a la croissance du diametre aortique.

Un nino de 6 anos de edad debuto con dolor epigastrico y vomitos de un ano de evolucion. La radiografia de torax y el transito baritado esofagogastrico demostraron la presencia de una masa mediastinica. La angiografia por tomografia computarizada toracica demostro un aneurisma de la aorta toracica descendente. Las determinaciones analiticas efectuadas fueron negativas. El aneurisma se corrigio quirurgicamente utilizando un injerto de dacron. En el estudio anatomopatologico se describian lesiones ateroscleroticas con calcificaciones, compatibles con una pared aneurismatica aterosclerotica. En la poblacion pediatrica los aneurismas son excepcionales. Habitualmente no puede determinarse la patogenia, y por tanto, dichos casos se agrupan como idiopaticos. La correccion directa con o sin un injerto es una alternativa terapeutica en estos aneurismas y permite el crecimiento de la circunferencia aortica. Los aneurismas son entidades poco frecuentes en la poblacion pediatrica. La mayoria afecta a la aorta toracica descendente (ATD), siendo los mas frecuentes los desarrollados despues de correcciones de coartacion aortica.

A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.

Clinical findings are reported in 5 cases of anomalous left coronary artery originating from pulmonary trunk, with reference of ventricular function evaluated through echocardiographic and angio-haemodynamic methods. All patients were catheterized between 7 and 8 months of age. Left atrial and ventricular dilatation, diminished shortening of minor diameter and free wall thickness and septum normal values were obtained by echocardiography. Quantitative angiography of left ventricle demonstrated an elevation of end-diastolic volumes (mean values: 270% of predicted normal). The ejection phase indexes are diminished. The left ventricular mass/end-diastolic volume ratio was low in all cases. Myocardial imaging with thallium-201 demonstrated perfusion defect in lateral area of left ventricle in one patient. The electrocardiographic signs of myocardial necrosis support the diagnosis, and the echocardiographic and isotopic studies may contribute to it. The angio-haemodynamic method corroborate the diagnosis, and the myocardial pump function evaluation may help to decide the most appropriate treatment for this anomaly.