Eye lens dose levels in interventional rooms using simple phantom simulation, dose management software and Monte Carlo method of uncertainty assessment.

This paper presents a fast method to estimate the annual eye lens dose levels for interventional practitioners applying the Monte Carlo method of uncertainty assessment. The estimation was performed by placing an anthropomorphic phantom in the typical working position, and applying the habitually employed protocol. No radiation protection devices were considered in the simulation. The results were compatible with the measurements performed during interventions by placing dosimeters in the vicinity of the eyes of two paediatric interventional cardiologists working with a fluoroscopic biplane system.

Patient doses in paediatric interventional cardiology: impact of 3D rotational angiography.

The aim of this study was to calculate the contribution of 3D rotational angiography to radiation doses received by paediatric patients in a cardiac catheterisation laboratory. The percentage increase in the median value of air kerma-area product due to cone beam CT was 33 and 16% for diagnostic and therapeutic procedures, respectively. Results are presented separately for five age groups and ten weight groups. Several methods for reducing radiation from 3D rotational angiography are suggested and patient doses are compared with previously published values.

Safety and tolerability of targeted therapies for pulmonary hypertension in children.

The objective of this study is to evaluate the safety and tolerability of the pharmacological treatment of pulmonary hypertension in pediatric patients. It is a retrospective, longitudinal, observational study on pediatric patients undergoing treatment with pulmonary targeted therapies. 63 patients were included (51% male), with a median age of 3.4 years (IQR, 3.6 months-10 years) and a median weight 13 kg (IQR, 6-30 kg). Congenital heart disease was the etiology of pulmonary hypertension in the majority of cases (n = 33) and 28 patients were in NYHA functional class III-IV. The most commonly used drug was sildenafil (n = 79, 56%), followed by bosentan (n = 27, 23%), and a combination of both (n = 14, 41%). 34 patients had adverse reactions (54%) with an incidence rate of 1.02 per patient per year. The most commonly reported reactions were gastrointestinal symptoms (22%) and spontaneous erections (22%) in males. Nine severe adverse reactions (10%) occurred, requiring eight treatment withdrawal and one hospital admission. Treatment with targeted therapies for pulmonary hypertension is safe in the pediatric population. Severe ADRs were uncommon both in monotherapy and in combination therapy. Combination therapy was associated with a higher rate of ADRs. We observed similar survival rates in children receiving sildenafil doses according to the European Medicines Agency (EMA) recommendations or higher.

Cardiología y cirugía cardíaca pediátricas / Pedaitric cardiology and cardiac surgery

Las cardiopatías congénitas representan uno de los aspectos de más complejo manejo. En el momento actual, la mayoría de ellas son diagnosticadas en el período fetal y, aunque la mayoría de ellas van a poder ser tratadas y cuadradas definitivamente, muchas van a requerir un seguimiento e intervenciones de distinto tipo durante toda la vida. Para ello se necesita que estos pacientes sean atendidos en centros, muy especializados con experiencia e infraestructura. El Hospital Infantil la Paz mantiene un compromiso, desde su inauguración hace más de cuarenta años, y dispone de personal altamente cualificado y de los medios físicos y tecnológicos adecuados, siendo sus unidades reconocidas a nivel ministerial (AU)

Congenital heart disease management is extremely delicate. Most of the patients are diagnosed in fetal life, and although the majority will be treated and discharge, many of them will require continuous follow-up and several kinds of interventions along their life. Therefore, these patients should be managed in high qualified centers, with experience and resources. The Hospital Infantil La Paz has been committed with these patients for more than forty years, with well trained personnel and adequate technology and is officially recognized (AU)

Utilidad de la terapia intracoronaria con células progenitoras en pacientes con miocardiopatía dilatada: ¿puente o alternativa al trasplante cardiaco? / Usefulness of intracoronary therapy with progenitor cells in patients with dilated cardiomyopathy: Bridge or alternative to heart transplantation?

Introducción y objetivos: Recientemente, algunas publicaciones pediátricas han planteado la utilidad de la terapia intracoronaria con células progenitoras autólogas (CPA) derivadas de la médula ósea en niños con miocardiopatía dilatada (MCD) e insuficiencia cardiaca. Describimos la utilidad de este tratamiento en dos lactantes con MCD e insuficiencia cardiaca grave, que habían sido trasladados a nuestro centro para valoración de trasplante cardiaco. Pacientes y métodos: El primer paciente es un varón de 3 meses de edad y 4 kg de peso. El segundo es un varón de 4 meses de edad y 5 kg de peso. En el momento del ingreso, ambos presentan mala situación clínica (NYHA IV), dilatación y disfunción sistólica grave (fracción de eyección [FE] <30%) del ventrículo izquierdo y marcada elevación de NT-pro BNP, precisando tratamiento con ventilación mecánica e inotrópicos en perfusión IV. Tras movilización con G-CSF durante 4 días, las CPA se obtienen desde la sangre periférica mediante leucocitoaféresis. Se administran por vía intracoronaria, con catéter-balón y técnica de stop-flow, 6,15 x 106 célulasCD34-positivas/kg en el primer paciente, y 10,55 x 106 células CD34-positivas/kg en el segundo. Resultados: Ya en la primera semana tras el procedimiento, la situación clínica de los pacientes mejora significativamente y en la ecocardiografía se objetiva una disminución importante de la dilatación del ventrículo izquierdo. Un mes después, también se evidencia mejoría importante en la FE (>40%) y de los niveles de NT-pro BNP, que se mantiene en el tiempo posteriormente. No obstante, en el primer paciente, a los cuatro meses del tratamiento, el ventrículo izquierdo se dilata de nuevo y empeora discretamente su función, aunque sin existir repercusión clínica significativa. Conclusiones: La terapia intracoronaria con CPA puede suponer una alternativa terapéutica en niños, especialmente de corta edad, con MCD y fallo cardiaco, pudiendo disminuir la mortalidad en lista, mejorar su situación clínica y ofrecer mayor tiempo de espera para recibir un órgano idóneo o, incluso, hacer innecesario el trasplante(AU)

Introduction and objectives: Some paediatric publications have recently raised the value of intracoronary therapy with autologous bone marrow-derived progenitor cells (APCs) in children with dilated cardiomyopathy (DCM) and heart failure. We describe the usefulness of this treatmen tin two infants with severe DCM and heart failure, who had been transferred to our hospital for cardiac transplant evaluation. Patients and methods: The first patient was a 3 months old male weighing 4 kg. The second was a 4 months old male weighing 5 kg. At the time of admission, both were in poor clinical condition (NYHA IV), with severe dilation and systolic dysfunction (ejection fraction [EF] <30%)of the left ventricle and marked elevation of NT-pro BNP, requiring treatment with mechanical ventilation and inotropic iv infusion. After mobilization with G-CSF for 4 days, APCs were obtained from peripheral blood by leukocytapheresis, administering them by a slow intracoronary bolus injection using a stop-flow technique (6.15x106 CD34-positive cells/Kg in the first patient, and 10.55x106 CD34-positive cells/Kg in the second). Results: Since the first week after the procedure, clinical status of patients improved and echocardiography showed a decrease in left ventricular dilation. A month later, there was a significant improvement in EF (> 40%) and NT-pro BNP levels, subsequently maintained throughout the follow-up. However, four months later in the first patient, the left ventricle dilated again and its function slightly worsened, but without any significant impact in his clinical status. Conclusions: Intracoronary therapy with APCs can be an alternative in children, especially infants, with DCM and heart failure. It can reduce the waiting list mortality, improve clinical status and provide more time on the waiting list to receive a suitable organ, or even to make transplantation unnecessary (AU)

[Usefulness of intracoronary therapy with progenitor cells in patients with dilated cardiomyopathy: Bridge or alternative to heart transplantation?]. / Utilidad de la terapia intracoronaria con células progenitoras en pacientes con miocardiopatía dilatada: ¿puente o alternativa al trasplante cardiaco?

INTRODUCTION AND OBJECTIVES: Some paediatric publications have recently raised the value of intracoronary therapy with autologous bone marrow-derived progenitor cells (APCs) in children with dilated cardiomyopathy (DCM) and heart failure. We describe the usefulness of this treatment in two infants with severe DCM and heart failure, who had been transferred to our hospital for cardiac transplant evaluation. PATIENTS AND METHODS: The first patient was a 3 months old male weighing 4 kg. The second was a 4 months old male weighing 5 kg. At the time of admission, both were in poor clinical condition (NYHA IV), with severe dilation and systolic dysfunction (ejection fraction [EF]<30%) of the left ventricle and marked elevation of NT-proBNP, requiring treatment with mechanical ventilation and inotropic iv infusion. After mobilization with G-CSF for 4 days, APCs were obtained from peripheral blood by leukocytapheresis, administering them by a slow intracoronary bolus injection using a stop-flow technique (6.15x106 CD34-positive cells/Kg in the first patient, and 10.55x106 CD34-positive cells/Kg in the second). RESULTS: Since the first week after the procedure, clinical status of patients improved and echocardiography showed a decrease in left ventricular dilation. A month later, there was a significant improvement in EF (> 40%) and NT-proBNP levels, subsequently maintained throughout the follow-up. However, four months later in the first patient, the left ventricle dilated again and its function slightly worsened, but without any significant impact in his clinical status. CONCLUSIONS: Intracoronary therapy with APCs can be an alternative in children, especially infants, with DCM and heart failure. It can reduce the waiting list mortality, improve clinical status and provide more time on the waiting list to receive a suitable organ, or even to make transplantation unnecessary.

Clinical significance of persistent left superior vena cava diagnosed in fetal life.

OBJECTIVES: To determine the prevalence and clinical significance of persistent left superior vena cava (PLSVC) in fetuses with and without cardiac and extracardiac anomalies. METHODS: Charts and recorded images were reviewed from high-risk patients who underwent fetal echocardiography between January 2000 and December 2005. This retrospective study included 54 fetuses with confirmed PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Associated congenital heart defects (CHDs), extracardiac abnormalities, including first trimester nuchal translucency (NT) thickness, and fetal/postnatal outcome were analyzed. RESULTS: Of 5,737 referrals, 5,233 had a normal heart, and PLSVC was observed in 10 of these fetuses (0.2%; Group 1). CHDs were present in 504 and PLSVC was observed in 44 of these cases (9%). In the latter group, 18/44 (41%) fetuses had heterotaxy syndrome (Group 2) where the most common structural heart defects were atrioventricular septal defect and double-outlet right ventricle. Fetuses without heterotaxy syndrome (Group 3) accounted for 26/44 (59%) cases of CHDs associated with PLSVC. In this group of fetuses the most common CHDs were left outflow tract obstructive defects and conotruncal anomalies. Increased NT was observed in 29%, without differences among the three groups. The survival rates among fetuses in Groups 1, 2 and 3 were 100%, 44% and 50%, respectively. After excluding patients who underwent pregnancy termination, there were no significant differences in the survival rates among the groups, probably due to the small size of the samples. CONCLUSIONS PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD.

Migraine symptoms related to the percutaneous closure of an ostium secundum atrial septal defect: report of four paediatric cases and review of the literature.

Several publications have recently suggested that atrial septal defect may be associated with the physiopathology of headache with migraine-type characteristics. We describe four previously asymptomatic paediatric patients with atrial septal defect who underwent percutaneous Amplatzer septal occluder device implantation and who subsequently developed symptoms compatible with migraine headache. The cases had normal echocardiograms after the intervention and a benign course with headache improvement after several weeks or months. There are paediatric patients with atrial septal defect who may dramatically develop migraine symptoms with or without aura following percutaneous correction of their defect. Large paediatric studies are needed to offer accurate prognoses for children and their families. The possibility of using clopidogrel to treat this type of headache is subject to debate.

Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve.

OBJECTIVES: To analyze fetal echocardiographic findings of absent pulmonary valve syndrome (APVS), its association with chromosomal and extracardiac anomalies including nuchal translucency (NT) and the outcome after diagnosis. METHODS: Data of 14 fetuses with confirmed APVS retrospectively collected in two tertiary referral centers between 1998 and 2004 were analyzed. The variables examined were: reason for referral, gestational age at diagnosis and associated abnormalities, including first trimester NT thickness. Cardiac evaluation included measurement of cardiothoracic ratio, diameter of pulmonary arteries and Doppler flow in the pulmonary trunk. Information was retrieved from clinical files, recorded videotapes and stored images. Karyotyping including examination for the 22q11 deletion was performed in all cases. RESULTS: Mean gestational age at diagnosis was 28 weeks, with 5/14 (36%) diagnosed before 22 weeks. In 13/14 (93%) there was an associated ventricular septal defect (subaortic in 12 fetuses and inlet-type in one) and all 13 had tetralogy of Fallot. Enlargement of the central pulmonary arteries and cardiomegaly were present in all cases diagnosed after 22 weeks. Of the five fetuses in which APVS was detected before 22 weeks, four (80%) had a normal pulmonary trunk diameter, two (40%) had normal pulmonary branches and three (60%) had normal cardiac size. The arterial duct was absent in 11/14 (79%). A correlation between presence of the arterial duct and the size of the central pulmonary arteries or cardiomegaly could not be established. Increased NT was observed in 4/10 cases (40%) for which this information was available. 22q11 microdeletion was diagnosed in three fetuses (21%). There were five terminations of pregnancy, one intrauterine death, five neonatal deaths and one infant death. Of the six neonates with respiratory distress, only one (17%) survived and of the eight babies in whom there was an intention to treat, two survived (25%). CONCLUSIONS: APVS can be accurately diagnosed by fetal echocardiography but screening ultrasound in the mid-second trimester is likely to have a low detection rate, probably due to the incomplete expression of the disease at this point. Many fetuses with APVS have an increased NT in the first trimester and this may help an earlier recognition of the defect. The most common associated karyotype anomaly is 22q11 microdeletion. Enlargement of the central pulmonary arteries is mainly related to the gestational age at diagnosis. Our results confirm that the outlook for these patients is extremely poor.

Pulmonary balloon valvuloplasty in a fetus with critical pulmonary stenosis/atresia with intact ventricular septum and heart failure.

Outcome of fetuses with critical pulmonary stenosis (critical PS) or atresia of the pulmonary valve (PA) with intact ventricular septum (IVS) is closely related with right ventricle hypoplasia and its consequent hemodynamics. Fetal echocardiography not only allows early detection of this condition but also monitors its normally unfavorable evolution. These cases may benefit from intrauterine intervention relieving outflow tract obstruction in order to achieve a biventricular circulation. Successful valvuloplasty of the pulmonary valve was performed in a fetus with critical PS-IVS and heart failure at 25 weeks. After the procedure there was a significant improvement in fetal hemodynamics. Follow-up scans at 34 weeks detected a significant restenosis with signs of circulatory failure leading to premature delivery of the baby. An immediate postnatal valvuloplasty successfully completed the ultimate objective of biventricular repair. Fetal pulmonary valvuloplasty is feasible and may change the natural history of the disease in fetuses with critical PS-IVS.

Cardiac defects in chromosomally normal fetuses with increased nuchal translucency at 10-14 weeks of gestation.

OBJECTIVE: To examine the prevalence, distribution and spectrum of cardiac defects in chromosomally normal fetuses with increased nuchal translucency thickness. PATIENTS AND METHODS: During a 4-year period, targeted fetal echocardiography was used in 353 chromosomally normal fetuses with increased nuchal translucency thickness at 10-14 weeks' gestation. The cardiac scan was performed at 18-22 weeks. In the last 138 cases enrolled, an additional scan at 12-16 weeks was carried out. The follow-up included the findings at necropsy or in the pediatric examination. A complete follow-up was achieved in 97%. RESULTS: Cardiac defects were present in 32 (9.1%) cases, increasing from 5.3% in those with a nuchal translucency thickness of > or = 95th centile (3.9 mm) to 24% when thickness > or = 6 mm (p < 0.001). In 31 cases (97%), the cardiac defect was diagnosed antenatally; in 24 cases (77%) this diagnosis was confirmed later. In the remaining seven cases, the autopsy examination was not available. A wide range of cardiac defects was observed, with the most common being atrioventricular septal defect and tricuspid atresia. CONCLUSIONS: Euploid fetuses with increased nuchal translucency thickness have a significantly increased risk of cardiac defects. This is a marker of different types of heart anomalies and constitutes an additional indication for targeted fetal echocardiography. Most of the cardiac defects can be detected by fetal echocardiography.

Diagnóstico prenatal de tumores cardíacos fetales: análisis retrospectivo de 18 casos / Prenatal diagnosis of fetal cardiac tumors: retrospective analysis of 18 cases

Objetivo: Analizar la repercusión, evolución perinatal y asociación con esclerosis tuberosa de los tumores cardíacos.Sujetos y método: Estudio retrospectivo de 18 casos diagnosticados prenatalmente (enero de 1991-diciembre de 2000) revisando características tumorales, repercusión y evolución posnatal (1-8 años).Resultados: Todos los tumores se detectaron en exámenes rutinarios en gestantes de bajo riesgo (edad gestacional media, 32 semanas); en 17 (94 per cent) eran aislados; en 10 (56 per cent) existían varias masas, y en 8 (44 per cent) sólo una. El tamaño osciló entre 1-46 mm, y la mayoría estaba en los ventrículos. En 16/18 fueron rabdomiomas (89 per cent). La repercusión más frecuente fue derrame pericárdico (8, 44 per cent). El manejo peri y posnatal fue conservador en la mayoría con reducción posnatal del tamaño en todos. La supervivencia ha sido del 67 per cent. Hubo manifestaciones extracardíacas de esclerosis tuberosa en 5/16 rabdomiomas (31 per cent). En todos había varias masas cardíacas.Conclusiones: Los tumores cardíacos son raros y habitualmente múltiples. Por su localización y tamaño pueden provocar complicaciones graves, incluso la muerte, pero la mayoría cursa sin repercusiones significativas. Por ello, puede proponerse un tratamiento conservador, ya que pueden comportarse de manera benigna. En cuanto a la esclerosis tuberosa, el pronóstico es más incierto, principalmente cuando existen varias masas cardíacas (AU)

Gestación gemelar monocorial-biamniótica con la misma cardiopatía congénita en ambos fetos / Monochorionic-diamniotic twin pregnancy with the same congenital heart detect in both fetuses

Se han descrito casos en la bibliografía en que ambos fetos de una gestación gemelar tienen una cardiopatía congénita, pero la concordancia malformativa es rara. Presentamos una gestación monocorial en la que los dos gemelos son portadores de la misma cardiopatía, concretamente un síndrome de ventrículo izquierdo hipoplásico, diagnosticado prenatalmente en el segundo trimestre. Por tanto, siempre que se diagnostique una cardiopatía congénita y, en general, cualquier malformación congénita en un gemelo, debe realizarse también una exploración pormenorizada del otro dada la posibilidad de concordancia malformativa (AU)

Early prenatal diagnosis of major cardiac anomalies in a high-risk population.

OBJECTIVE: To examine the accuracy of early fetal echocardiography performed in a high-risk population combining transvaginal and transabdominal routes. METHODS: A series of 330 high-risk pregnancies were screened by transvaginal and transabdominal scan at 12-17 weeks' gestation in a prospective multicentre trial in Spain between September 1999 and May 2001. A total of 334 fetal heart examinations were performed, including four twin pregnancies. Maternal age ranged from 17 to 46 years (mean 33 years with 36% of women over 34 years). The median gestational age at scan was 14.2 weeks (range 12-17 weeks). For each fetus, visualization of the four-chamber view, the origin of the great arteries, aortic and ductal arches and systemic venous return was attempted in a segmental approach. B-mode and colour/pulsed Doppler flow imaging were used in all cases. The duration of complete heart examination was less than 30 minutes. The examinations were performed by three experienced operators. Reliability was assessed by conventional transabdominal echocardiography at 20-22 weeks, by postnatal follow-up in the first three months of life, and/or by autopsy in cases of termination of pregnancy. RESULTS: The rate of successful visualization of the fetal heart was 94.6% (316/334). In 48 out of 334 (14.4%) fetuses the final diagnosis was abnormal. In 38 out of 48 (79.2%) cases with heart defects the diagnosis was suspected at early echocardiography. In the group with congenital heart defects, 27 cases had an abnormal karyotype (56.3%) and 31 cases showed extracardiac anomalies (64.6%). There were 10 false-negative cases at early scan. There were no false-positive diagnoses. CONCLUSIONS: This experience stresses the usefulness of early fetal echocardiography when performed by expert operators on fetuses specifically at risk for cardiac disease. The high rate of successful visualization of the fetal heart provides a reliable diagnosis of major cardiac defects at this early stage of pregnancy.

Diagnóstico y manejo fetal del rabdomioma cardíaco y la esclerosis tuberosa / Fetal diagnosis and management of heart rhabdomyoma and tuberous sclerosis

Se comunica el diagnóstico prenatal de tumoración cardíaca en un caso de Esclerosis Tuberosa de Bourneville. A pesar del volumen de la masa tumoral, no hubo repercusión hemodinámica ni clínica cardiológica; aunque debutó con convulsiones neonatales. La Resonancia Magnética mostró la presencia de nódulos tuberosos cerebrales que ratifican el diagnóstico. Se discuten las posibilidades evolutivas y la pauta asistencial recomendada (AU)

[Cardiac hydatid cyst in a child]. / Quiste hidatídico cardíaco en un niño.

Cardiac hydatid cyst is an uncommon disease, especially in children. The interventricular septum region is the most frequent localization. The cysts usually involve other organs making the etiologic diagnosis easier. Our young patient presented a single hydatid cyst. The etiologic diagnosis was difficult due to the negativity of the serologic tests and there were no other organs affected. Surgical ablation of the cyst was performed and histopathological analysis achieved.

[Incessant fetal tachycardia coinciding with supraventricular tachycardia of the mother]. / Taquicardia incesante fetal coincidiendo con taquicardia supraventricular de la gestante.

The case of a gestant diagnosed fetal arrhythmia in the 35th week of gestation is presented. A fetal echocardiography indicates an aneurysm of the interauricular septum and a fetal supraventricular tachycardia. Also is obtained an ambulatory 24-hour Holter electrocardiograph of the gestant and fetus rhythm. The register makes evident a gestant supraventricular tachycardia and as well a fetal supraventricular tachycardia, occurring in both without clinical company. Following the birth, the tachycardia in the newborn is identified to be automatic and incessant, requiring digital for its control. In postpartum registers of the mother no significant arrhythmia of any type is observed. We conclude that the ambulatory monitoring with a Holter electrocardiograph could be a good method of diagnosis and follow-up of fetal and gestant arrhythmias.

[The curative treatment of incessant atrioventricular tachycardia by radiofrequency ablation]. / Curación de la taquicardia incesante auriculoventricular mediante ablación con radiofrecuencia.

The permanent form of junctional reciprocating tachycardia is due to accessory pathways with retrograde long conduction times. We report the localization of the atrial insertion of the accessory pathway and successful ablation with radiofrequency in 3 patients. The participation of an accessory pathway in the tachycardia was demonstrated by atrial capture without changes in the sequence, with ventricular stimuli during His bundle refractoriness. The atrial insertion was localized by mapping near the os of the coronary sinus. In all cases one to three radiofrequency pulses applied at this point interrupted the tachycardia. Retrograde conduction through the accessory pathway reappeared in 30 min or earlier in all, with displacement of the point of earliest atrial activation, and tachycardia recurred. Total abolition of accessory pathway conduction and cure of the tachycardia required new radiofrequency applications, that in one case were done in a second procedure.