Luxación aislada de codo en niños menores de 10 años: reporte de dos casos / Isolated elbow dislocation in children under 10 years of age. Report of two cases

La luxación de codo aislada es muy infrecuente en esqueletos inmaduros. El manejo y el tratamiento no están estandarizados debido a la baja casuística. Las luxaciones aisladas en niños <10 años constituyen un cuadro descrito como evidencia compartida en estudios de niños más grandes. El objetivo de este artículo es comunicar dos casos de luxación aislada de codo en niños <10 años. Los niños consultan a la unidad de urgencias por dolor y deformidad en el codo a las pocas horas del trauma. En ambos casos, se realizaron evaluaciones clínicas y radiográficas en busca de lesiones asociadas antes de la reducción cerrada y después. Se descartó inestabilidad articular bajo anestesia y se inmovilizó por dos semanas. Los resultados funcionales fueron excelentes en ambos casos, a los tres meses de la lesión. Nivel de Evidencia: IV

Isolated elbow dislocation is extremely rare in immature skeletons. Due to the low casuistry, the management and treatment are not standardized. Isolated dislocations in children under 10 years of age constitute a pathology described as shared evidence in studies of older children. The objective of this report is to share the experience of two cases of isolated elbow dislocation in children under 10 years of age. The children consult the emergency unit about pain and elbow deformity a few hours after the trauma. For each case, a clinical and radiological evaluation was developed in search of associated lesions before and after closed reduction. Joint instability was ruled out under anesthesia and the patient is immobilized for two weeks. Excellent functional outcomes were obtained in both cases three months after the injury. Level of Evidence: IV

Experiencia con la prótesis de pericardio bovino Carpentier-Edwards® en posición pulmonar en pacientes con cardiopatías congénitas / Experience with Carpentier Edwards® bovine pericardium implantations in pulmonary position in patients with congenital heart diseases

Introducción. El número de pacientes con cardiopatías complejas corregidas en la infancia que necesitan una sustitución valvular pulmonar para restaurar la competencia o solucionar la estenosis del tracto de salida de ventrículo derecho ha aumentado en los últimos años. El injerto ideal continúa siendo motivo de controversia. En el servicio de cardiocirugía del Hospital Ramón y Cajal de España, se comenzó a utilizar prótesis de pericardio bovina de Carpentier-Edwards siendo el objetivo de este estudio su evaluación a corto y medio plazo. Materiales y Métodos. Entre enero de 2004 y mayo de 2010 fueron intervenidos 42 pacientes para sustitución valvular pulmonar mediante prótesis de pericardio bovino. El estudio fue ambispectivo con prospección durante los dos últimos años. Resultados. La mediana de la edad fue de 20,96 años (amplitud intercuartil 10,5 años). El número medio de cirugías previas fue de 1,9±0,9 siendo el tiempo medio entre la última cirugía y la implantación de la prótesis de 17,2±7 años. Las indicaciones quirúrgicas fueron: disfunción del ventrículo derecho (45%), su dilatación progresiva (38%), arritmias ventriculares (14%) y síncopes (3%). La mortalidad precoz de causa cardiológica fue de dos pacientes. El tiempo medio de seguimiento fue de 2,1±1,4 años (rango entre 0,1 y 6,3 años) estando el 94,3% de ellos en clase funcional I de la New York Heart Association. El gradiente Doppler pico transprotésico por ecocardiografía fue de 18,5±17 mm Hg. No se observaron cambios degenerativos ni ningún tipo de deterioro estructural de la prótesis. Conclusiones. La prótesis de pericardio bovino en posición pulmonar presenta excelentes resultados a corto y medio plazo. Sin embargo, es necesario un seguimiento mayor para confirmar lo resultados iniciales respecto a su durabilidad y hemodinamia a largo plazo...

Introduction. In recent years the number of patients with complex congenital heart disease previously corrected in infancy who need a pulmonary valve replacement has increased dramatically. The ideal substitute remains a source of dispute. Nowadays, in the unit of heart surgery of the Hospital Ramon y Cajal in Spain, its being implanting in this position the bovine pericardium Carpentier-Edwards prosthesis. The aim of the study is its short and mediumtermassessment. Material and methods. Between January 2004 and May of 2010, 42 patients have been operated for pulmonary valve replacement with pericardium prosthesis. The study was ambispective, being prospective in its last two years. Results. The median age of the patients was 20.96 years (interquartile 10.5 years). The mean number of surgeries prior to the pulmonary valve replacement was 1.9±0.9, being the mean time between the “corrective” surgery and the prosthetic implantation 17.2±7 years. The main indications for this surgery were: right ventricle dysfunction (45%), progressive dilation of the same ventricle (38%), ventricular arrhythmias (14%) and syncopes (3%). Two patients died in the immediate postoperative due to cardiological causes. The mean follow-up time has been 2.1±1.4 years (0.1-6.3). The 94.3 % of the surviving patients are in functional class I, according to the New York Health Association. The peak Doppler transprosthetic gradient determined by echocardiography was 18.5±17 mm Hg. In the echocardiograpic follow-up there have been neither degenerative changes nor any type of structural deterioration of the prosthesis. Conclusions. The bovine pericardium prosthesis in pulmonary position presents excellent results in the short and medium-term. However, it is necessary a longer follow-up to confirm our initial results regarding its durability and haemodynamics long-term...

[Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant]. / Una forma rara de cardiopatía cianosante por obstrucción supravalvular tricuspídea en el lactante.

We present a case involving a rare form of cyanotic congenital heart disease in a 2-month-old neonate. The initial diagnosis was thought to be tricuspid dysplasia with right-to-left shunting through an interatrial communication. However, surgery showed the presence of a pedunculated mass that prolapsed into the tricuspid valve orifice, thereby causing severe obstruction to right ventricular filling. This mass was attached to the lower part of the atrial septum. Pathological examination showed fibrotic myxoid tissue. After the mass was resected surgically, the tricuspid valve appeared normal. We comment on the pathogenesis and the differential diagnosis of this rare entity.

The clinical profile of Ebstein's malformation as seen from the fetus to the adult in 52 patients.

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

[Single-stage Fontan procedure: early and late outcome in 124 patients]. / Operación modificada de Fontan o variantes efectuadas en un solo tiempo quirúrgico. Determinantes de la mortalidad.

INTRODUCTION AND OBJECTIVES: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period. MATERIAL AND METHODS: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified. RESULTS: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years. CONCLUSIONS: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.

Correccion quirurgica de la coartacion aortica en el neonato: Analisis de 35 casos / Surgical corrrection of the aortic coarctation in the newborn: Analysis of 35 cases

De 1978 a 1983 se operaron 35 neonatos con coartacion aortica, sobre un total de 200 ninos con la misma patologia, en la Unidad Medico-Quirurgica, servicio de remision nacional y dedicacion especifica a cardiopediatria en el Hospital Ramon y Cajal de Madrid, Espana. Las indicaciones para intervenirlos fueron: insuficiencia cardiaca rebelde al tratamiento medico y/o hipertension arterial. Como tecnicas se emplearon aortoplastia con parche de politetrafluoroetileno en 23 casos, aortoplastia con colgajo de subclavia en 11 (Wald-hausen), y reseccion del segmento coartado y anastomosis termino-terminal en 1. Destaca la gran incidencia de cardiopatias asociadas en el neonato. Hubo 8 muertes, 6 de ellas con severas anomalias cardiacas asociadas y otro con miocardiopatia. Tardiamente hubo 5 casos de reestenosis, y ninguna hipertension arterial. Los demas evolucionaron favorablemente, y otros esperan correccion definitiva de la cardiopatia asociada. El alto indice de mortalidad en este grupo, ademas de un estricto tratamiento medico previo, realizar la operacion en el momento y en las condiciones mas propicias y cuanto antes, pues estos casos se presentan casi siempre como emergencias que precisan intervencion quirurgica inmediata