[Chronic pulmonary insufficiency after tetralogy of Fallot and pulmonary stenosis intervention. Pulmonary valve replacement with mechanical prosthesis]. / Insuficiencia pulmonar crónica secundaria a tetralogía de Fallot y estenosis pulmonar intervenida. Recambio valvular pulmonar con prótesis metálica.

INTRODUCTION: Tetralogy of Fallot and pulmonary stenosis are a frequent cause of consultation in patients with congenital heart disease. Interventions performed in infancy can cause residual pulmonary insufficiency and may require pulmonary valve replacement when adults. METHODS: From 2003 to 2007, 37 patients underwent right ventricular outflow tract or pulmonary valve intervention (tetralogy of Fallot and pulmonary stenosis). Differences between echocardiographic parameters in both pathologies, indications for pulmonary valvular replacement and the type of prosthesis used, were studied. RESULTS: 25 (67.6 %) patients had tetralogy of Fallot and 12 (32.4 %) pulmonary stenosis. In the pulmonary stenosis group, 7 patients had pulmonary valvulotomy in infancy and 5 had percutaneous pulmonary valvuloplasty in adulthood. Fallot patients received a transannular patch. No significant differences were seen in echocardiographic parameters (right and left ventricular diameter, pulmonary insufficiency, transpulmonary gradient and left ventricular ejection fraction). Mechanical prosthesis valve replacement was performed in 5 (13.5 %) patients, with no morbidity-mortality and with a significant improvement of the functional class. CONCLUSIONS: Chronic pulmonary insufficiency is a frequent complication after intervention of tetralogy of Fallot and pulmonary stenosis. Mechanical prosthesis valve replacement has low morbidity-mortality and low risk of complications after short to medium-term follow-up.

Insuficiencia pulmonar crónica secundaria a tetralogía de Fallot y estenosis pulmonar intervenida. Recambio valvular pulmonar con prótesis metálica / Chronic pulmonary insufficiency after tetralogy of Fallot and pulmonary stenosis intervention. Pulmonary valve replacement with mechanical prosthesis

Introducción. La tetralogía de Fallot y la estenosis pulmonar intervenida son causas frecuentes de consulta en pacientes con cardiopatías congénitas. Las intervenciones realizadas en la infancia conllevan una insuficiencia pulmonar residual que puede requerir recambio valvular pulmonar en la edad adulta. Métodos. Entre 2003 y 2007 se controló a 37 pacientes con intervencionismo sobre el tracto de salida del ventrículo derecho y la válvula pulmonar (tetralogía de Fallot y estenosis pulmonar). Se realizó un estudio para evaluar la diferencia entre los parámetros ecocardiográficos de ambas patologías, las indicaciones de recambio valvular pulmonar y el tipo de prótesis implantada. Resultados. Del total de pacientes con intervención sobre la válvula pulmonar, 25 (67,6 %) tenían tetralogía de Fallot y 12 (32,4 %), estenosis pulmonar. De los pacientes con estenosis pulmonar, a 7 se les realizó valvulotomía en la infancia y a 5, valvuloplastia pulmonar en la edad adulta. A los pacientes con tetralogía de Fallot se les realizó una ampliación transanular con parche. La diferencia de medias de los parámetros ecocardiográficos estudiados (diámetros de los ventrículos derecho e izquierdo, grado de insuficiencia pulmonar, gradiente transpulmonar y fracción de eyección del ventrículo izquierdo) no fue significativa. Se realizó recambio valvular pulmonar con prótesis metálica en 5 (13,5 %) pacientes, sin morbimortalidad y con una mejoría significativa del grado funcional. Conclusiones. La insuficiencia pulmonar crónica es una complicación frecuente en los pacientes con tetralogía de Fallot y estenosis pulmonar intervenidas. El recambio pulmonar con prótesis metálica es un procedimiento de baja morbimortalidad y escaso riesgo de complicaciones a corto-medio plazo

Introduction. Tetralogy of Fallot and pulmonary stenosis are a frequent cause of consultation in patients with congenital heart disease. Interventions performed in infancy can cause residual pulmonary insufficiency and may require pulmonary valve replacement when adults. Methods. From 2003 to 2007, 37 patients underwent right ventricular outflow tract or pulmonary valve intervention (tetralogy of Fallot and pulmonary stenosis). Differences between echocardiographic parameters in both pathologies, indications for pulmonary valvular replacement and the type of prosthesis used, were studied. Results. 25 (67.6 %) patients had tetralogy of Fallot and 12 (32.4 %) pulmonary stenosis. In the pulmonary stenosis group, 7 patients had pulmonary valvulotomy in infancy and 5 had percutaneous pulmonary valvuloplasty in adulthood. Fallot patients received a transannular patch. No significant differences were seen in echocardiographic parameters (right and left ventricular diameter, pulmonary insufficiency, transpulmonary gradient and left ventricular ejection fraction). Mechanical prosthesis valve replacement was performed in 5 (13.5 %) patients, with no morbidity-mortality and with a significant improvement of the functional class. Conclusions. Chronic pulmonary insufficiency is a frequent complication after intervention of tetralogy of Fallot and pulmonary stenosis. Mechanical prosthesis valve replacement has low morbidity-mortality and low risk of complications after short to medium-term follow-up

[Aortic-left ventricular tunnel. Long-term surgical results]. / Túnel aorta-ventrículo izquierdo. Resultados quirúrgicos a largo plazo.

OBJECTIVE: The aim of this work is to describe the different aspects of diagnosis and postoperative follow-up in four patients after surgery for aortic-left ventricular tunnel (ALVT). The surgical techniques and results have been published, in the literature but there are no reports about the progression of aortic regurgitation. METHOD: We reviewed the clinical features of four patients operated with ALVT. The clinical information (electrocar-diograms, echocardiograms and catheterilution) was studied in the preoperative period. Surgical technique and postoperative evolution were analysed. The follow-up period ranged between 4.7 and 13 years. RESULTS: In a 14 years period, four children (2 boys and 2 girls), were operated for ALVT. The mean age at the time of diagnosis was 8.5 years (ranged between 1 month and 14 years). A continuous murmurs was present in all cases, associated to cardiac failure in the youngest patient (1 month old). The clinical diagnosis was ALVT in the four cases but only in two the echocardiographic study showed correctly the anatomic aspects of the malformation. The angiohemodynamic study was correct in three patients and the differential diagnosis was made in last one with rupture of aortic sinuses of Valsalva to the left ventricle. All cases were operated; with direct closure of the aortic orifice (two cases), or with dacron patch (two cases). In one case aortic valve replacement was also performed. There was not mortality and the mean age at the time of surgery was 9 years (1.5 months and 14 years). The mean follow-up was 8.5 years (ranged between 4.7 and 13 years), three patients showed clinically and in the echocardiogram study an aortic valvular regurgitation without ALVT deshiscence. All cases are asymptomatic. No patient was reoperated. CONCLUSION: The surgical treatment of ALVT must be done as soon as the diagnosis is made to prevent left ventricular enlargement, ventriculo-aortic floor distortion and aortic valvular lesion. A precise echocardiographic study is necessary to avoid catheterization. This study provided a clear knowledge of anatomic structure of the ALVT for both clinician and surgeons. This information is important in order to obtain an optimal surgical success, particularly to prevent the valvar aortic regurgitation.

[Intracardiac mycetoma induced by central catheterization]. / Micetoma intracardíaco inducido por un catéter de vía central.

We report a case of an infective endocarditis presented as a right atrial mass which pathological study showed a big vegetation of candida albicans (mycetoma). We think its presence was related to a great central vein catheterization during the neonatal period. The clinical feature was completed with pulmonary fungal embolism and later tricuspid valve afectation. With this work we wish to remark the necessary careful management of patients with central vein catheters to avoid this severe complication. We review the pharmacological and surgical treatment of this uncommon entity.

[Aortico-left ventricular tunnel. Presentation of 2 new cases. Usefulness of non-invasive study]. / Túnel aorta-ventrículo izquierdo. Presentación de dos nuevos casos. Utilidad del estudio no invasivo.

In the last years the anatomic characteristics of the left aorto-ventricular tunnel and the clinical aspects of this uncommon malformation have been well described. Its evolution usually fatal without surgical procedure is in great contrast with the slight symptoms encountered by us in two new cases. This particular finding joined to the specific aspects found in the noninvasive color Doppler study, have lead us to present two new cases of left aorto-ventricular tunnel successfully operated.

Temas de ecocardiografía: III. Evaluación ecocardiográfica previa a la cirugía de los enfermos con conexión atrioventricular univentricular / Echocardiography: III. Early echocardiographic evaluation in surgery of patients with univentricular atrioventricular connection

Los pacientes con conexión atrioventricular univentricular pueden beneficiarse hoy día de diversos procedimientos quirúrgicos paliativos. La indicación quirúrgica óptima debe estar basada en un diagnóstico anatómico y funcional exactos. Para valorar hasta qué punto la ecocardiografía, puede contribuir al diagnóstico revisamos 45 pacientes operados con conexión atrioventricular tipo univentricular. Las técnicas quirúrgicas empleadas fueron las siguientes: banding de la arteria pulmonar en 10 pacientes, de los cuales en 8 se asoció la técnica de Blalock-Hanlon; fístula sistémico-pulmonar en 34 casos, de los que en 6 se realizó también atrioseptectomía de Blalock-Hanlong; anastomosis atriopulmonar modificada (técnica de Kreutzer) en 4 enfermos. La ecocardiografía, fue satisfactoria para el diagnóstico de la malformación y el estado del septo interatrial, el foramen interventrículo-cameral y las válvulas atrioventriculares y semilunares. También fue útil para conocer las características funcionales de las cámaras ventriculares. Sin embargo, la información que facilitó la ecocardiografía, en lo referente al arco aórtico y a las ramas de la arteria pulmonar, fue inexacta y no fiable. En conclusión: 1) El tratamiento quirúrgicos de los pacientes con conexión atrioventricular univentricular está justificado, pues con él se consigue mejorar la calidad de vida del enfermo y de su entorno y, a largo plazo, reducir los costos de la asistencia médica. 2) La ecocardiografía, proporciona una parte muy sustancial de la información que necesitamos para realizar un diagnóstico preoperatorio correcto

Temas de ecocardiografía: III. Evaluación ecocardiográfica previa a la cirugía de los enfermos con conexión atrioventricular univentricular / Echocardiography: III. Early echocardiographic evaluation in surgery of patients with univentricular atrioventricular connection

Los pacientes con conexión atrioventricular univentricular pueden beneficiarse hoy día de diversos procedimientos quirúrgicos paliativos. La indicación quirúrgica óptima debe estar basada en un diagnóstico anatómico y funcional exactos. Para valorar hasta qué punto la ecocardiografía, puede contribuir al diagnóstico revisamos 45 pacientes operados con conexión atrioventricular tipo univentricular. Las técnicas quirúrgicas empleadas fueron las siguientes: banding de la arteria pulmonar en 10 pacientes, de los cuales en 8 se asoció la técnica de Blalock-Hanlon; fístula sistémico-pulmonar en 34 casos, de los que en 6 se realizó también atrioseptectomía de Blalock-Hanlong; anastomosis atriopulmonar modificada (técnica de Kreutzer) en 4 enfermos. La ecocardiografía, fue satisfactoria para el diagnóstico de la malformación y el estado del septo interatrial, el foramen interventrículo-cameral y las válvulas atrioventriculares y semilunares. También fue útil para conocer las características funcionales de las cámaras ventriculares. Sin embargo, la información que facilitó la ecocardiografía, en lo referente al arco aórtico y a las ramas de la arteria pulmonar, fue inexacta y no fiable. En conclusión: 1) El tratamiento quirúrgicos de los pacientes con conexión atrioventricular univentricular está justificado, pues con él se consigue mejorar la calidad de vida del enfermo y de su entorno y, a largo plazo, reducir los costos de la asistencia médica. 2) La ecocardiografía, proporciona una parte muy sustancial de la información que necesitamos para realizar un diagnóstico preoperatorio correcto (AU)

[Problems related to tricuspid atresia during the first year of life (author's transl)]. / Problemas específicos planteados por la atresia tricúspide en el primer año de la vida.

Sixty six per cent of cases need surgical treatment during the first months of life. The high mortality rate (23 to 40%) in those children which need pulmonary banding or sistemic pulmonary shunts authors believe is due to: 1) The existence of coartation of the aorta in 83% of the cases with trasposition of the great arteries and high pulmonary blood flow. 2) The small diameter of pulmonary arteries in cases with low pulmonary blood flow (smaller than those presenting Fallot's Tetralogy). 3) The association, in 25% of the cases with normally related great arteries, with a subaortic myocardiopathy. These findings require before use of surgical methods: a) Practice of aortography in cases with transposition of great arteries. b) Proper evaluation of pulmonary artery branches diameter performing, if needed, a contrast injection in pulmonary vein. c) Angiographic and echocardiographic studies of left ventricle to rule out association with a hypertrophic myocardiopathy.

[Ventricular septal defect in the first year of life (author's transl)]. / Comunicación interventricular en el primer año de la vida.

Thirty cases of CIV in the first year of life are presented. Association with prematurity and other malformations anticipate cardiac failure. Left ventricular hypertrophy is more frequent in conal defects. Repolarization disturbances were found in 73% of the cases in left precordial derivation with significative relief after surgical repair (banding). Conduction disturbances after surgical closure of ventricular septal defect was found in 15% of the cases. Surgical treatment during the first year was needed in 86% of patients. Only one case had good evolution without surgery. After four months of life total correction of septal defect as prefered. In patients with previous pulmonary artery banding, correction must be made one or two years later.

[Aortic coarctation in childhood. Surgical experience in 120 cases]. / Coartación de aorta en la infancia Experiencia quirúrgica en 120 casos

112 cases of coarctation of the aorta and 8 cases of tubular hypoplasia of the aortic isthmus operated upon in the Children's Hospital "La Paz" from Madrid, are reviewed. All children were under 7 years of age. 64.2% of the cases of aortic coarctation were in the first year of life, 47.3% of them had associated lesions, being the most frequently present persistent ductus arteriosus and ventricular septal defect. Hospital mortality was 14.2%, what is considered as very acceptable. All the children operated upon for the correction of tubular hypoplasia of the aortic isthmus were in the first year of age. 75% of them had associated ductus arteriosus and ventricular septal defect, being hospital mortality of 62.5%. Most frequent postoperative complications and cause of death were due to broncopulmonary disorders secondary to the existence of a previous pulmonary hypertension.