[Cardiac Magnetic Resonance in long term follow-up of Tetralogy of Fallot]. / Resonancia magnética cardíaca en el seguimiento alejado de pacientes con tetralogía de Fallot.

INTRODUCTION: Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart disease. Pulmonary regurgitation (PR) and right ventricle (RV) enlargement and dysfunction are the most common long-term complications. Cardiac magnetic resonance (CMR) is the gold standard for RV evaluation. OBJECTIVE: To analyze CMR results in the follow-up of TOF patients. PATIENTS AND METHOD: All CMR performed between 2007 and 2012 in TOF patients with transannular patch (TAP) repair or infundibular widening, and without pulmonary valve replacement (PVR) were included. Pulmonary regurgitant fraction (PRF), ventricular end-diastolic (EDV) and end-systolic volume (ESV), and ejection fraction (EF) were examined. RESULTS: 122 CMR were performed in 114 patients. Average age at CMR was 15.4±7.4 years. 53.3% of them presented severe PR (> 40%). RVEDV was 157.3 ± 38.6 ml/m2, RVESV was 85.3 ± 27 ml/m2 and RVEF was 46.4 ± 7.1%. RVEDV was > 150 ml/ m2 in 48.4% and > 170 ml/m2 in 32.8% of patients. Patients with TAP showed larger RV volumes compared with those with infundibular widening. RVEDV > 170 ml/m2 showed worse RVEF that those with lower RVEDV (47.9 ± 7% vs 43.2 ± 6.4%, p < 0.01). CONCLUSION: Almost half of the pa tients showed significant RV enlargement, demonstrating that the indication of CMR is late in their follow-up. TAP was associated with higher RVEDV and RVESV, but no worse RVEF.

Resonancia magnética cardíaca en el seguimiento alejado de pacientes con tetralogía de Fallot / Cardiac Magnetic Resonance in long term follow-up of Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot (TF) es la cardiopatía congénita cianótica más frecuente. La insuficiencia pulmonar (IP) y dilatación del ventrículo derecho (VD) son las complicaciones más frecuentes a largo plazo. La resonancia magnética cardiaca (RMC) es el "gold standard" para la evaluación del VD. OBJETIVO: Analizar la información obtenida de las RMC en el seguimiento de pacientes con TF. PACIENTES Y MÉTODO: Se incluyeron RMC realizadas entre 2007 y 2012 a pacientes con TF, reparados con parche transanular (PTA) o ampliación infundibular (AInf) y sin recambio valvular pulmonar (RVP). La fracción de regurgitación pulmonar (FRP), el volumen y función ventricular fueron evaluados. RESULTADOS: Se realizaron 122 RMC a 114 pacientes. Edad promedio al examen 15,4 ± 7,4 años. 53,3% presentó IP severa (> 40%). La media del volumen de fin de diástole del VD (VFDVD) fue 157,3 ± 38,6 ml/m2, fin de sístole (VFSVD) de 85,3 ± 27 ml/m2 y fracción de eyección (FEVD) 46,4 ± 7,1%. 48,4% presentaba un VFDVD mayor de 150 ml/m2 y el 32,8% mayor a 170 ml/ m2. El PTA se relacionó con mayores volúmenes de VD que la AInf. VFDVD mayor a 170 ml/m2 mostraron peor FEVD (FEVD 47,9 ± 7% vs 43,2 ± 6,4%, p < 0,01). DISCUSIÓN: Casi la mitad mostró una significativa dilatación del VD demostrando que la indicación de RMC es tardía en el seguimiento. El PTA se asoció con mayores VFDVD y VFSVD pero no a peor FEVD.

INTRODUCCIÓN: Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart disease. Pulmonary regurgitation (PR) and right ventricle (RV) enlargement and dysfunction are the most common long-term complications. Cardiac magnetic resonance (CMR) is the gold standard for RV evaluation. OBJECTIVE: To analyze CMR results in the follow-up of TOF patients. PATIENTS AND METHOD: All CMR performed between 2007 and 2012 in TOF patients with transannular patch (TAP) repair or infundibular widening, and without pulmonary valve replacement (PVR) were included. Pulmonary regurgitant fraction (PRF), ventricular end-diastolic (EDV) and end-systolic volume (ESV), and ejection fraction (EF) were examined. RESULTS: 122 CMR were performed in 114 patients. Average age at CMR was 15.4±7.4 years. 53.3% of them presented severe PR (> 40%). RVEDV was 157.3 ± 38.6 ml/m2, RVESV was 85.3 ± 27 ml/m2 and RVEF was 46.4 ± 7.1%. RVEDV was > 150 ml/ m2 in 48.4% and > 170 ml/m2 in 32.8% of patients. Patients with TAP showed larger RV volumes compared with those with infundibular widening. RVEDV > 170 ml/m2 showed worse RVEF that those with lower RVEDV (47.9 ± 7% vs 43.2 ± 6.4%, p < 0.01). CONCLUSION: Almost half of the pa tients showed significant RV enlargement, demonstrating that the indication of CMR is late in their follow-up. TAP was associated with higher RVEDV and RVESV, but no worse RVEF.

Bloqueo cardiaco fetal completo: reporte de dos casos y revisión de la literatura / Fetal complete atrioventricular block: two case report and literature review

Presentamos 2 casos de embarazos controlados en nuestro servicio con el diagnóstico de bloqueo aurículo-ventricular fetal. Este es un tipo de arritmia poco frecuente, relacionado con la presencia de anticuerpos antiribonucleoproteínas (Ro y La). El manejo es expectante en la mayoría de los casos ya que no existe forma de revertir el bloqueo; en caso de evidenciar una descompensación hemodinámica fetal, se pueden administrar corticoides como medida terapéutica con un éxito limitado. No existe contraindicación del parto vaginal y el uso de pH de cuero cabelludo y oximetría de pulso parecen ser métodos adecuados para la evaluación de la condición fetal intraparto. Recomendamos el enfoque multidisciplinario en esta patología para evitar intervenciones innecesarias, anticipar los riesgos fetales y obtener un mejor pronóstico postnatal.

Here we report the perinatal outcome of two patients with fetal complete atrioventricular (AV) block. This is an uncommon disease, related to the presence of autoantibodies against ribonucleoproteins (Ro and La). Management should be expectant in most cases because a treatment to revert the AV block is not available; when fetal hemodynamic problems are detected corticosteroids can be used, but with limited effectiveness. Vaginal delivery is allowed; fetal scalp pH and pulse oximetry are appropriate for intrapartum fetal surveillance. We recommend a multi disciplinary approach to avoid unnecessary interventions, anticipate fetal risk and obtain a better perinatal outcome.

[Cardiac tumors in children and adults: A Multicentric retrospective study]. / Comportamiento clínico de los tumores cardíacos desde el feto hasta el adulto: serie multicéntrica de 38 pacientes.

BACKGROUND: Cardiac tumors are very uncommon at all ages. There are important clinical differences between children and adults in the behavior of these tumors. AIM: To compare the behavior of primary and secondary cardiac tumors, from fetal age to adults. PATIENTS AND METHOD: Multicentric retrospective analysis of 38 children and adults with cardiac tumors, evaluated with echocardiography between January 1995 and August 2001. Medical records, echocardiographic and radiological examinations, surgical protocols and pathologic examinations were reviewed. Follow-up was obtained through data on medical records or calling patients by telephone. RESULTS: Tumors were diagnosed in 38 patients (13 children and 25 adults), from a total of 31.800 echocardiograms. In children the diagnosis was made by fetal, transthoracic or transesophageal echocardiography in 23.6% and 8% of cases, respectively. Eighty five percent were primary (10 benign and 1 malignant) and 15%, secondary tumors. Fifty four percent were rhabdomyomas and 75% regressed spontaneously. Seventy seven percent were symptomatic and 31% were treated with surgery. During a follow up of 44+/-35 months, 31% of patients died. In adults, 76% of tumors were diagnosed by transthoracic and 20% by transesophageal echocardiography. Seventy six percent were primary (18 benign and 1 malignant) and 24% secondary tumors. Fifty six percent were myxomas. Ninety two percent were symptomatic and 84% were treated surgically. Twenty percent of patients died in the early postoperative period. No adult patients had a follow-up. CONCLUSIONS: Rhabdomyomas were solely found in children. In adults, myxomas are the predominant cardiac tumors. Primary and metastasic malignant tumors are observed both in children and in adults.

Comportamiento clínico de los tumores cardíacos desde el feto hasta el adulto: serie multicéntrica de 38 pacientes / Cardiac tumors in children and adults: A retrospective study

Background: Cardiac tumors are very uncommon at all ages. There are important clinical differences between children and adults in the behavior of these tumors. Aim: To compare the behavior of primary and secondary cardiac tumors, from fetal age to adults. Patients and Method: Multicentric retrospective analysis of 38 children and adults with cardiac tumors, evaluated with echocardiography between January 1995 and August 2001. Medical records, echocardiographic and radiological examinations, surgical protocols and pathologic examinations were reviewed. Follow-up was obtained through data on medical records or calling patients by telephone. Results: Tumors were diagnosed in 38 patients (13 children and 25 adults), from a total of 31.800 echocardiograms. In children the diagnosis was made by fetal, transthoracic or transesophageal echocardiography in 23.6 percent and 8 percent of cases, respectively. Eighty five percent were primary (10 benign and 1 malignant) and 15 percent, secondary tumors. Fifty four percent were rhabdomyomas and 75 percent regressed spontaneously. Seventy seven percent were symptomatic and 31 percent were treated with surgery. During a follow up of 44±35 months, 31 percent of patients died. In adults, 76 percent of tumors were diagnosed by transthoracic and 20 percent by transesophageal echocardiography. Seventy six percent were primary (18 benign and 1 malignant) and 24 percent secondary tumors. Fifty six percent were myxomas. Ninety two percent were symptomatic and 84 percent were treated surgically. Twenty percent of patients died in the early postoperative period. No adult patients had a follow-up. Conclusions: Rhabdomyomas were solely found in children. In adults, myxomas are the predominat cardiac tumors. Primary and metastasic malignant tumors are observed both in children and in adults.

El diagnostico prenatal de cardiopatías congénitas mejora el pronostico neonatal

Las malformaciones congénitas son la principal causa de muerte neonatal precoz en nuestro medio; en la mayoría de los casos corresponden a cardiopatías congénitas mayores. Las cardiopatías congénitas tienen una incidencia de 8/1.000 recién nacidos vivos, correspondiendo la mitad de ellas a cardiopatías congénitas mayores. Una de las intervenciones recomendadas para reducir la mortalidad de este grupo de niños es evaluar rutinariamente la anatomía del corazón fetal mediante ecografía obstétrica, para planificar la atención neonatal, de aquellos fetos con cardiopatías congénitas, en el momento y lugar más oportuno. En objetivo de la presente revisión es comprobar si el diagnóstico prenatal de una cardiopatía congénita mejora el pronóstico perinatal respecto de aquellos casos que son diagnosticados post parto. Observamos que el diagnóstico antenatal de cardiopatía congénita, no mejora la sobrevida neonatal, excepto en ciertas cardiopatías congénitas ductus dependientes (transposición de grandes arterias, hipoplasia del corazón izquierdo y coartación de aorta), en que si se ha reportado una mayor probabilidad de sobrevida en el grupo de recién nacidos en los cuales se realizó el diagnóstico en el período prenatal. Recomendamos la evaluación rutinaria del corazón fetal en la ecografía obstétrica habitual, y efectuar ecocardiografía fetal especializada ante la sospecha de alteraciones o en aquellos grupos de mayor riesgo.

Congenital malformations are the main cause of neonatal death; in most of the cases they correspond to major congenital heart defects. Congenital heart defects have an incidence of 8/1,000 live newborns, corresponding half of them to major congenital heart defects. To reduce the mortality of this group of children routine evaluation of fetal heart anatomy by ultrasound is recommended, allowing neonatal care, of those fetuses with congenital heart defects, at the appropriate time and place. Here we attempt to verify if prenatal diagnosis of a congenital heart defect improves the perinatal outcome compare to postnatal diagnosis. We observed that the prenatal diagnosis of congenital heart defects, does not improve the neonatal outcome, except in certain congenital ductus dependent heart defects (transposition of great vessels, hypoplastic left heart syndrome and aortic coarctation), where a better outcome has been shown for those new born with prenatal diagnosis. We recommend routine evaluation of the fetal heart during prenatal ultrasound, and to carry out fetal echocardiography in high risk groups or when a defect is suspected.

Reemplazo valvular mitral y aórtico en niños: resultados durante la última década con prótesis de última generación / Mitral and aortic valve replacement in children: last decade results with latest generation prothesis

Antecedentes: El reemplazo valvular mitral (RVM) y aórtico (RVA) es poco frecuente en pacientes pediátricos y las series publicadas reportan elevada morbi-mortalidad. Objetivo: Evaluar los resultados a mediano plazo en niños sometidos a RVM y RVA durante los últimos diez años, con el fin de determinar morbimortalidad y calidad de vida. Método: Análisis retrospectivo de 26 niños consecutivos (mediana de edad 5,6 años) sometidos a RVM y RVA, entre marzo 1992 y junio 2003. Diecisiete presentaban patología mitral y once aórtica. Resultados: Se realizaron 26 operaciones implantando 28 prótesis (26 mecánicas y 2 biológicas). Tres pacientes (12 por ciento) fallecieron durante el primer mes. La mediana de seguimiento fue de 6,2 años. Tres pacientes con RVM fallecieron alejadamente por causa no atribuible al reemplazo valvular con una sobrevida actuarial de 87 por ciento. Tres pacientes requirieron reoperación. Todos realizan actividades adecuadas a su edad, restringiéndose los deportes de contacto. Conclusión: Los resultados a mediano plazo del reemplazo valvular en niños con valvulopatía grave son satisfactorios.

[Progressive respiratory insufficiency secondary to pulmonary fibrosis in childhood. Report of one case]. / Insuficiencia respiratoria progresiva secundaria a fibrosis pulmonar en una escolar: Caso clínico.

Interstitial lung diseases are uncommon in children, and can be idiopathic or secondary to known causes, sharing common pathological findings. We report a girl with progressive respiratory insufficiency secondary to interstitial pneumonia and pulmonary fibrosis, with risk factors such as bronchopulmonary dysplasia and respiratory infections (respiratory syncytial virus and suspected Mycoplasma pneumoniae), that may have had an additive effect. Nasal bi-level Positive Airway Pressure was used in the last period of her disease. She died due to global respiratory failure at the age of 14 years.

Insuficiencia respiratoria progresiva secundaria a fibrosis pulmonar en una escolar: Caso clínico / Pulmonary fibrosis in childhood: Report of one case

Interstitial lung diseases are uncommon in children, and can be idiopathic or secondary to known causes, sharing common pathological findings. We report a girl with progressive respiratory insufficiency secondary to interstitial pneumonia and pulmonary fibrosis, with risk factors such as bronchopulmonary dysplasia and respiratory infections (respiratory syncytial virus and suspected Mycoplasma pneumoniae), that may have had an additive effect. Nasal bi-level Positive Airway Pressure was used in the last period of her disease. She died due to global respiratory failure at the age of 14 years.

Cirugía paliativa mediante operación de Norwood en distintas formas de corazón univentricular: resultados quirúrgicos inmediatos / Immediate results of palliative surgery for different forms of univentricular heart using the Norwood procedure

Background: Norwood procedure is used as the first stage in the palliative treatment of the hypoplastic heart syndrome and can be used, with some technical modifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. These patients have a high mortality (50 percent), derived from the procedure itself and from their abnormal physiological status. Aim: To report our experience with the Norwood procedure. Patients and methods: Retrospective analysis of all patients subjected to the Norwood procedure between February 2000 and June 2003. Results: Thirteen patients (9 females, age range 5-60 days and median weight of 3.3 kg) were operated. Eight had hypoplastic heart syndrome and five had a single ventricle with aortic arch hypoplasia. The diagnosis was done in utero in eight patients. All technical variations, according to the disposition and anatomy of the great vessels, are described. Cardiac arrest with profound hypothermia was used in all and regional cerebral perfusion was used in nine. Three patients died in the perioperative period and three died in the follow up (two, four and 10 months after the procedure). Gleen and Fontan procedures were completed in five and one patients, respectively. Conclusions: Our results with the Norwood procedure are similar to other series. There is an important mortality in the immediate operative period and prior to the Glenn procedure (Rev MÚd Chile 2004; 132: 556-63).

Effect of high-dose intravenous immunoglobulin in delayed pressure urticaria.

BACKGROUND: Delayed pressure urticaria (DPU) is difficult to treat. High-dose intravenous immunoglobulin (IVIG) has been found to be effective in treating patients with autoimmune chronic urticaria. OBJECTIVES: To report the effect of IVIG on eight patients with severe unremitting DPU. METHODS: IVIG was administered at a dose of 2 g kg-1 over 2-3 days on an in-patient basis. The response to treatment was assessed subjectively and recorded as remission, improved or unchanged. An autologous serum skin test (ASST) was performed in seven patients. RESULTS: Three of eight patients achieved remission; two after one infusion and one after three infusions. Two patients improved. Three patients remained unchanged; of these, two declined further treatment after two infusions, and one failed to improve after six infusions at monthly intervals. Four of seven patients had positive ASST; three responded to IVIG. Two developed delayed positive ASST; both responded to IVIG. Of three patients with negative ASST, two responded. CONCLUSIONS: IVIG induced remission or improved symptoms in five of eight patients with DPU with severe unremitting disease who had failed to respond to other therapies or were controlled only with systemic corticosteroids. Those who responded did so with three or fewer infusions. ASST is not a reliable predictor of response to IVIG.

Allergic contact dermatitis in venous leg ulcer patients.

Our aim was to determine the frequency and nature of contact sensitivity in venous leg ulcer patients in Lanarkshire. We performed patch testing with the European standard series, antimicrobials and medicaments on 200 patients referred to our leg ulcer clinics. Positive patch tests were found in 136 (68%) patients. Multiple allergies occurred in 102 (51%). The most frequent allergen groups were fragrances (30.5%), antimicrobials (19.5%), topical excipients (19.5%), rubber accelerators (13.5%) and topical corticosteroids (8%). We also found a high prevalence of positive patch tests to Intrasite gel (9.5%) and Hioxyl cream (8.5%), medicaments which are commonly used to treat leg ulcers in our area. Contact sensitivity is common in venous leg ulcer patients and has important implications for patient management. The allergens involved vary depending on local nursing practice. We suggest that all venous leg ulcer patients be patch tested with a locally relevant patch test series.

Erosive pustular dermatosis of the leg associated with compression bandaging and fungal infection.

BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) has been described in association with venous insufficiency and atrophy of the skin of the lower leg. Like erosive pustular dermatosis of the scalp, this disease has also been reported to be a non-infective condition. OBJECTIVES: To investigate the clinicopathological features and, where possible, the aetiology of clinical EPDL. METHODS: We identified a group of patients undergoing continuous compression bandaging for venous dermatitis of the legs and/or predominantly venous leg ulceration with clinical features described in patients with EPDL. They were investigated by skin biopsy, patch testing and microbiological tests for the presence of bacteria and fungi. RESULTS: Twenty-four of 400 (6%) patients were noted to have pustules on the leg(s). There was laboratory evidence of fungal infection in 13 of 24 patients (54%), with complete and sustained resolution of pustules after antifungal treatment. Pustulation in the other 11 patients (46%) was unresponsive to antibiotics for confirmed bacterial infection; some improvement was seen with potent topical corticosteroids but full clearance was achieved only after a switch from continuous four-layer compression bandaging to the use of intermittent long stretch compression. CONCLUSIONS: EPDL is a fairly common clinical picture seen in patients undergoing continuous compression bandaging. It may be produced by opportunistic, particularly fungal, infection. In almost half an infective aetiology cannot be demonstrated and a pyoderma gangrenosum-like process may be implicated.

Resonancia magnética en el estudio de cardiopatías congénitas: rol en la evaluación de las estructuras vasculares extracardiacas / Magnetic resonance in the study of congenital cardiopathies: role in the evaluation of the extracardiac vascular structures

En la actualidad la resonancia magnética ha adquirido cada vez más importancia en el diagnóstico y seguimiento de las cardiopatías congénitas. Entrega información anatómica y más recientemente información funcional. Se presenta la experiencia acumulada entre enero y agosto del año 2002, en cinco casos pediátricos en que la RM cardiaca fue utilizado como método complementario a la ecocardiografía y angiografía. En todos los casos aportó información anatómica precisa, en especial de la anatomía vascular extracardiaca y fue determinante en la conducta terapéutica. Se discute además las características de método e indicaciones.

Pulse oximetry: a new tool to assess patients with leg ulcers.

The objective of the study was to investigate pulse oximetry as a guide to assessing patients with leg ulcers before treatment. Graduated elastic compression is the treatment of choice for uncomplicated venous leg ulcers, but is contra-indicated in patients with significant arterial disease. The standard assessment of arterial insufficiency by Doppler ultrasound ankle branchial pressure index (ABPI) has shortcomings which prompted this investigation of pulse oximetry as a possible additional, or alternative, method of assessment of patients with leg ulcers, prior to treatment with compression. The study, carried out on a population of patients attending hospital leg ulcer clinics, was designed to evaluate pulse oximetry assessment in the selection and monitoring of patients with venous leg ulceration leading to a prospective controlled study of ulcer healing in groups of patients with reduced and normal ABPI, selected for compression therapy by pulse oximetry criteria. Outcome measurement required follow-up of patients selected for compression therapy by pulse oximetry to record time to healing and rate of healing of leg ulcers. Results from the study show a fair correlation between the toe-finger oximetry index (TFOI) and Doppler ABPI. There is no difference between ulcer healing in patients with reduced and normal ABPI selected for treatment on the basis of pulse oximetry maximum compression pressure (MCP). In conclusion, pulse oximetry is an aid to the selection of patients who will benefit from compression therapy, but would be excluded on the basis of Doppler ABPI.

[Peracetic acid: alternative to the sterilization of bronchofibroscopes]. / Acido peracético: alternativa a la esterilización de broncofibroscopios.

The Steris system for cold sterilization with peracetic acid was evaluated by effecting a series of contaminations of a fiberoptic bronchoscope (FB) with specimens of Pseudomonas aeruginosa, Acinetobacter baumanii and Mycobacterium kansasi. The FB was contaminated 24 times, 8 times by each microorganism, using specimens containing more than 10(8) cfu/ml. After fixing the secretions on the FB and washing it with enzyme soap, the BF was sterilized. Specimens were taken for culturing after contamination of the FB, after washing, immediately after sterilization and 1 hour after sterilization. No microorganism growth of any of the samples was detected either immediately after sterilization or one hour later. Microbiological data confirmed contamination of the FB after aspiration and fixation of the inoculate. Chemical and biological tests with B. stearothermophilus spores as specified by the manufacturer were correct in all cases: 24 contaminations and 52 processes of prior training. The efficacy of washing with enzyme soap before sterilization stands out. In 14 of the 24 samples, culture was negative after washing and in 7 the concentration of microorganisms was less than 500 cfu/ml, which confirms the need for appropriate washing before any disinfection or sterilization process is begun. In conclusion, the Steris system based on peracetic acid is an alternative to other systems for cold sterilization or high level disinfection.

Fulguración por radiofrecuencia de las taquicardias supraventriculares en pediatría / Radiofrequency ablation of supraventricular tachycardias in pediatrics

Supraventricular tachycardias (SVT) are the most frequent cause of tachycardia in children. Its pharmacological treatment has adverse effects, is not curative and is not always effective. During the last few years radiofrequency ablation (RF-A) has changed the treatment. The purpose of this study is to evaluate our experience in RF-A in children with SVT. Between 1990 and 1995, 92 patients (1 mont to 17 years old) underwent electrophysiological study after the diagnosis of SVT. RF-A was attempted in 55 patients with accessory pathways (AP), slow-pathway of the atrioventricular node, or ectopic focus. The site of ablation was decided according to the electrical signals and the catheter position. The success of the RF-A was confirmed by the interruption of the tachychardia, the change in the sequence of activation of the intracardiac signals, the regression of the preexcitation and the inability to reinduce tachycardia. RF-A was succesful in 81 percent of the patients; 88 percent in those with a left AP 56 percent in those with a right arterial obstruction, one with a minimal pneumothorax and one with cardiac tamponade. During a follow up of 16.6 months there was no relapse nor late complications. We conclude that RF-A is a safe and effective procedure in pediatric patients with SVT