Surgical management of Wilms tumor with intravascular extension: a single-institution experience.

The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.4 years, range 2-8.1 years) were identified. At diagnosis, 6 of the 7 patients (85.7%) presented with tumor thrombus that reached the right atrium (RA) and 1 patient with infrahepatic inferior vena cava (IVC) thrombus. All patients received neoadjuvant chemotherapy (SIOP 2001 protocol) with vincristine, doxorubicin, and actinomycin D. Regression of the intravascular extension of the tumor was documented in all patients. Postchemotherapy level of extension was suprahepatic IVC in 1 patient, infrahepatic IVC in 2 patients, renal vein (RV) in 1 patient, and RA in 3 patients. Nephrectomy and thrombectomy were performed in all cases, requiring cardiopulmonary bypass for the 4 patients who presented with suprahepatic IVC and RA thrombus. The other 3 patients with infrahepatic IVC and RV involvement underwent cavotomy and thrombus extraction. Computed tomography, ultrasonography, and echocardiography were used for diagnosis and follow-up. All patients remain disease-free with a median follow-up of 6.3 years (range, 2-19 years). Neoadjuvant chemotherapy for WT with intravascular extension may facilitate the resection by decreasing the extent of the tumor thrombus. Cardiopulmonary bypass is indicated for suprahepatic IVC and RA involvement. Accurate diagnostic imaging is necessary.

[Postsurgical pseudoaneurysm of the right ventricular outflow tract]. / Seudoaneurisma posquirúrgico del tracto de salida ventricular derecho.

Cardiac pseudoaneurysm, a sealed rupture of the heart, is potentially fatal if not repaired surgically. The incidence in children is low, and it has been described in connection with surgery involving the right ventricular outflow tract. We describe a case diagnosed 20 months after complete repair of Tetralogy of Fallot with a single coronary artery. The diagnosis was made with two-dimensional echocardiography and confirmed with right ventriculography. The pseudoaneurysm was corrected by emergency surgery without complications. We emphasize the importance of a high index of suspicion of this entity in these patients, who are often asymptomatic.

[Aortopulmonary window: clinical assessment and surgical results]. / Ventana aortopulmonar: valoración clínica y resultados quirúrgicos.

INTRODUCTION AND OBJECTIVES: Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension. PATIENTS AND METHOD: Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1. Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization. Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics. RESULTS: No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months. CONCLUSIONS: Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.

Mid-term morbidity and mortality of patients after arterial switch operation in infancy for transposition of the great arteries.

INTRODUCTION AND OBJECTIVES: The arterial switch operation is currently the preferred surgical approach for complete transposition of the great arteries. We sought to determine the mid-term results of this intervention. METHODS: A single-institution retrospective review of clinical records of all consecutive patients who underwent the arterial switch surgery between 1985 and 2010. RESULTS: Overall, the operation was performed on 155 patients (68% boys) at a median age of 13 days: 64% with an intact septum, 46% with a ventricular septal defect, and 4.5% with associated aortic arch anomaly. The usual coronary pattern was found in 63%. Palliative surgery was performed prior to arterial switch in 6.5%. In all, 137 perioperative survivors were followed for a median of 6 years. Late mortality was 2.9%, of which 50% was due to coronary complications. Eighteen percent required surgical and/or percutaneous reintervention: 95.6% for right-sided obstruction and 4.3% for aortic regurgitation. At last follow-up, 92% had functional class I symptoms and 95% were free of arrhythmias. The left ventricular ejection fraction was greater than 55% in 95%, 28% had neoaortic regurgitation (78% mild regurgitation), and 31% had right ventricular outflow tract obstruction with a mean gradient according to echocardiography greater than 25mmHg. CONCLUSIONS: Mid-term survival of patients after arterial switch operation is excellent and their functional status is good. However, a few patients have residual lesions and a need for further intervention during follow-up, mostly for right-sided obstructions. Late mortality was uncommon and was related to coronary complications. Neoaortic root dilation and regurgitation are not major issues in early adulthood, but the long-term course of these lesions is still unknown.

Morbilidad y mortalidad de los pacientes con transposición completa de grandes arterias intervenidos mediante cirugía de corrección arterial / Mid-term Morbidity and Mortality of Patients After Arterial Switch Operation in Infancy for Transposition of the Great Arteries

Introducción y objetivos: La corrección anatómica es el tratamiento de elección para neonatos con transposición de grandes arterias. El objetivo es analizar resultados a medio plazo de esta intervención. Métodos: Estudio retrospectivo de los registros clínicos de todos los pacientes consecutivos operados mediante cirugía de intercambio arterial en el hospital infantil desde 1985 hasta 2010. Resultados: Se intervino a 155 pacientes (el 68% varones) a una mediana de edad de 13 días: el 64% con transposición completa con septo íntegro; el 46% con comunicación interventricular, y el 4,5% con enfermedad del arco aórtico asociada. El patrón coronario fue el usual en el 63%. Se realizó cirugía previa al 6,5%. De 137 supervivientes seguidos durante una mediana de 6 años, la mortalidad tardía fue del 2,9%, el 50% debido a complicaciones coronarias. El 18% requirió reintervención quirúrgica y/o angioplastia: el 95,6% por estenosis en el tronco y/o las ramas pulmonares y el 4,3% por insuficiencia aórtica. En la última revisión estaban en clase funcional I (92%) y sin arritmias (95%). La fracción de eyección del ventrículo izquierdo fue mayor del 55% en el 95%, el 28% tenía insuficiencia aórtica (el 78% de grado I) y el 31%, estenosis a la salida del ventrículo derecho (gradiente medio superior a 25 mmHg). Conclusiones: Los pacientes con corrección anatómica presentan buena supervivencia y buen estado funcional a medio plazo; las lesiones residuales del tronco o la arteria pulmonar son la complicación más frecuente y causa de reintervención. La muerte tardía de causa cardiaca no es un suceso frecuente, pero parece relacionado con complicaciones coronarias. Aunque hay casos de insuficiencia aórtica precoz, aún desconocemos la evolución a largo plazo en la vida adulta de la dilatación de la neorraíz de aorta e insuficiencia aórtica (AU)

Introduction and objectives: The arterial switch operation is currently the preferred surgical approach for complete transposition of the great arteries. We sought to determine the mid-term results of this intervention. Methods: A single-institution retrospective review of clinical records of all consecutive patients who underwent the arterial switch surgery between 1985 and 2010. Results: Overall, the operation was performed on 155 patients (68% boys) at a median age of 13 days: 64% with an intact septum, 46% with a ventricular septal defect, and 4.5% with associated aortic arch anomaly. The usual coronary pattern was found in 63%. Palliative surgery was performed prior to arterial switch in 6.5%. In all, 137 perioperative survivors were followed for a median of 6 years. Late mortality was 2.9%, of which 50% was due to coronary complications. Eighteen percent required surgical and/or percutaneous reintervention: 95.6% for right-sided obstruction and 4.3% for aortic regurgitation. At last follow-up, 92% had functional class I symptoms and 95% were free of arrhythmias. The left ventricular ejection fraction was greater than 55% in 95%, 28% had neoaortic regurgitation (78% mild regurgitation), and 31% had right ventricular outflow tract obstruction with a mean gradient according to echocardiography greater than 25 mmHg. Conclusions: Mid-term survival of patients after arterial switch operation is excellent and their functional status is good. However, a few patients have residual lesions and a need for further intervention during follow-up, mostly for right-sided obstructions. Late mortality was uncommon and was related to coronary complications. Neoaortic root dilation and regurgitation are not major issues in early adulthood, but the long-term course of these lesions is still unknown (AU)

Seudoaneurisma posquirúrgico del tracto de salida ventricular derecho / Postsurgical pseudoaneurysm of the right ventricular outflow tract

El seudoaneurisma cardíaco es una rotura cardíaca contenida, potencialmente letal si no se corrige. Su incidencia en niños es muy rara y ha sido descrita en relación con la cirugía del tracto de salida ventricular derecho. Presentamos un caso diagnosticado 20 meses después de la reparación completa de una tetralogía de Fallot con arteria coronaria única. Los hallazgos ecocardiográficos fueron confirmados en la ventriculografía derecha y el seudoaneurisma corregido urgentemente sin complicaciones. Queremos resaltar la trascendencia del diagnóstico de sospecha en enfermos con frecuencia asintomáticos

Cardiac pseudoaneurysm, a sealed rupture of the heart, is potentially fatal if not repaired surgically. The incidence in children is low, and it has been described in connection with surgery involving the right ventricular out flow tract. We describe a case diagnosed 20 months after complete repair of Tetralogy of Fallot with a single coronary artery. The diagnosis was made with two-dimensional echocardiography and confirmed with right ventriculography. The pseudo-aneurysm was corrected by emergency surgery without complications. We emphasize the importance of a high index of suspicion of this entity in these patients, who are often asymptomatic

Ventana aortopulmonar: valoración clínica y resultados quirúrgicos / Aortopulmonary window: clinical assessment and surgical results

Introducción y objetivos. El defecto del septo aortopulmonar es una cardiopatía congénita rara. Hasta el momento se han publicado alrededor de 300 casos. Presentamos nuestra casuística, resaltando la importancia de su corrección precoz para evitar el desarrollo de enfermedad vascular pulmonar irreversible. Pacientes y método. Entre 1979 y 2000, 7 pacientes fueron intervenidos de dicha cardiopatía en nuestro centro. Dos pacientes presentaron el tipo I (proximal), cuatro el tipo II (distal) y uno el tipo III (defecto completo del septo aortopulmonar). En 4 casos (57 por ciento) se asociaron anomalías complejas: interrupción del istmo aórtico tipo A (n = 2), hipoplasia del istmo aórtico (n = 1) y transposición de los grandes vasos con comunicación intraventricular. El diagnóstico se realizó mediante ecocardiografía en 4 casos (57 por ciento) y en todos fue confirmado mediante estudio hemodinámico. Realizamos un estudio retrospectivo de sus historias clínicas, con especial atención a los datos clínicos, del diagnóstico ecocardiográfico y hemodinámico, así como a los aspectos quirúrgicos. Resultados. No hubo ningún fallecimiento durante la intervención quirúrgica. El enfermo portador de transposición de los grandes vasos con comunicación interventricular falleció 22 días después a causa de hipertensión pulmonar severa. Tras un seguimiento medio de 69 meses, los enfermos restantes se encuentran asintomáticos sin medicación. Conclusiones. A pesar de su rareza, se debe pensar en esta entidad ante toda cardiopatía compleja que curse con insuficiencia cardíaca e hipertensión pulmonar precoces. La corrección antes de los 6 meses evitará el desarrollo de enfermedad vascular pulmonar irreversible (AU)