Gonzalo Urcelay M

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.Multicentric retrospective analysis of 38 children andadults with cardiac tumors, evaluated with echocardiography between January 1995 andAugust 2001. Medical records, echocardiographic and radiological examinations, surgicalprotocols and pathologic examinations were reviewed. Follow-up was obtained through data onmedical records or calling patients by telephone.

Operación de Fontan: resultados inmediatos y a mediano plazo con anastomosis cavopulmonar total

Background: During the last five years, 65 patients with univentricular heart have been treated surgically in our institution, according to a protocol of staged operations that have been previously reported. Aim: To evaluate the early and mid-term outcome of those patients that have completed their staging protocol by means of a Fontan procedure. Patients and Methods: Between April 1996 and June 2001, 23 patients (age 16 to 223 months) underwent a Fontan procedure, 15 with an intracardiac lateral tunnel technique and 8 with an extracardiac conduit. A retrospective review of their clinical, surgical, echocardiographic, angiographic and hemodynamic data was performed, trying to identify risk factors for both mortality and functional capacity (FC). Follow up was complete in all survivors. Results: Three patients died early after surgery (13.04%). Excessive pulmonary blood flow was a risk factor for early death (p= 0.03). One patient died at 14 months. Follow up was 29.9 months (1-63). For those who survived the operation, five years survival was 93.3%. The majority of patients are in FC I or II, with no related risk factors. Conclusions: Our current results are comparable with those of larger series. Patients reach good FC and mid-term survival, irrespective of type of single ventricle or the surgical strategy (Rev Med Chile 2002; 130: 1217-26).

EL DIAGNÓSTICO PRENATAL DE CARDIOPATÍAS CONGÉNITAS MEJORA EL PRONÓSTICO NEONATAL

SUMMARY 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.

Cirugía paliativa mediante operación de Norwood en distintas formas de corazón univentricular: resultados quirúrgicos inmediatos

: Norwood procedure is used as the first stage in thepalliative treatment of the hypoplastic heart syndrome and can be used, with some technicalmodifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. Thesepatients have a high mortality (50%), derived from the procedure itself and from their abnor-mal physiological status.

Insuficiencia respiratoria progresiva secundaria a fibrosis pulmonar en una escolar: Caso clínico

Interstitial lung diseases are uncommon in children, and can beidiopathic or secondary to known causes, sharing common pathological findings. We report a girlwith progressive respiratory insufficiency secondary to interstitial pneumonia and pulmonaryfibrosis, with risk factors such as bronchopulmonary dysplasia and respiratory infections(respiratory syncytial virus and suspected Mycoplasma pneumoniae), that may have had anadditive effect. Nasal bi-level Positive Airway Pressure was used in the last period of her disease. Shedied due to global respiratory failure at the age of 14 years (Rev Med Chile 2005; 133; 82-88).(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.

Bloqueo cardiaco fetal completo: Reporte de dos casos y revisión de la literatura

Presentamos 2 casos de embarazos controlados en nuestro servicio con el diagnostico de bloqueo auriculo-ventricular fetal. Este es un tipo de arritmia poco frecuente, relacionado con la presencia de anticuerpos antiribonucleoproteinas (Ro y La). El manejo es expectante en la mayoria de los casos ya que no existe forma de revertir el bloqueo; en caso de evidenciar una descompensacion hemodinamica fetal, se pueden administrar corticoides como medida terapeutica con un exito limitado. No existe contraindicacion del parto vaginal y el uso de pH de cuero cabelludo y oximetria de pulso parecen ser metodos adecuados para la evaluacion de la condicion fetal intraparto. Recomendamos el enfoque multidisciplinario en esta patologia para evitar intervenciones innecesarias, anticipar los riesgos fetales y obtener un mejor pronostico postnatal.

Resonancia magnética en el estudio de cardiopatías congénitas: rol en la evaluación de las estructuras vasculares extracardiacas

En la actualidad la resonancia magnetica ha adquirido cada vez mas importancia en el diagnostico y seguimiento de las cardiopatias congenitas. Entrega informacion anatomica y mas recientemente informacion funcional. Se presenta la experiencia acumulada entre enero y agosto del ano 2002, en cinco casos pediatricos en que la RM cardiaca fue utilizado como metodo complementario a la ecocardiografia y angiografia. En todos los casos aporto informacion anatomica precisa, en especial de la anatomia vascular extracardiaca y fue determinante en la conducta terapeutica. Se discute ademas las caracteristicas de metodo e indicaciones

Cierre transcatéter de ductus arterioso persistente con espirales de Gianturco

Objetivo: evaluar retrospectivamente el rendimiento del cierre del ductus arterioso persistente (DAP) con tecnica transcateter, utilizando espirales (coils) de Gianturco. Pacientes y Metodo: entre octubre de 1996 y septiembre de 2000, 22 pacientes de peso mayor de 10 kg (10,5 a 59), portadores de DAP de hasta 4 mm en su diametro menor, fueron sometidos a esta tecnica de cierre. Bajo sedacion con midazolam y ketamina, se efectuo sondeo cardiaco via arteria y vena femoral derechas, y aortografia. Por medio de un cateter multiproposito se avanzo espiral de Gianturco a traves del ductus desde su extremo aortico hasta posicionarlo en el DAP. Resultados: se logro cierre angiografico ductal en 22 pacientes (100%), observandose minima filtracion residual por ecocardiograma con Doppler color en 3 pacientes (14%), consignandose ausencia de filtracion a los 15 dias en dos de ellos. Todos los pacientes fueron dados de alta a las 24 horas. Cuatro pacientes presentaron embolizacion del espiral, los que fueron recuperados durante el procedimiento. Conclusion: el cierre transcateterismo de DAP con espirales de Gianturco es una tecnica segura y efectiva, constituyendo una muy buena alternativa al cierre quirurgico, con el beneficio adicional de no requerir anestesia general ni toracotomia.