[Angioplasty with ductal stent: experience at Garrahan Hospital]. / Angioplastia con stent ductal: experiencia en el Hospital Garrahan.

Objective: To describe and evaluate the outcomes of ductal angioplasty with stent placement at a single high-complexity center during the period 2016-2022. Method: A retrospective descriptive cross-sectional study was conducted, including patients under 3 months of age who underwent ductal stent implantation as initial palliative treatment. Demographic, clinical, and anatomical data were collected before the intervention. Mortality, intra- and post-procedural complications, need for re-intervention, intensive care requirements, and hospital stay were recorded. The characteristics at the time of definitive surgery are described. Discrete variables are presented as percentages, and continuous variables are presented with their medians and respective interquartile ranges. Results: Twenty patients who underwent this treatment were reviewed, revealing a success rate of 80%. Complications due to stent dysfunction required surgical resolution. 95% of patients were dischargedfrom the institution after the procedure, and 17 patients reached a second definitive surgical stage. Three patients died afterthe procedure, but with no direct relation to it. Conclusions: Indications for ductal angioplasty with stent as an alternative treatment to systemic-pulmonary anastomosis by surgery are not yet fully defined; the strategy represents a valid alternative in appropriately selected patients. The presented experience shows results similar to international reference centers.

Objetivo: Describir y evaluar los resultados de la angioplastia con stent ductal en un único centro de alta complejidad durante el periodo 2016-2022. Método: Estudio descriptivo retrospectivo de corte transversal en el que se incluyeron pacientes menores de 3 meses a quienes se implantó un stent en el conducto arterioso como tratamiento paliativo inicial. Se recolectaron datos demográficos, clínicos y anatómicos previos a la intervención. Se registraron la mortalidad, las complicaciones intra- y posprocedimiento, la necesidad de reintervención, los requerimientos de cuidados intensivos y la estadía hospitalaria. Se describen las características al momento de la cirugía definitiva. Las variables discretas son presentadas con porcentajes, y las variables continuas con sus medianas y sus respectivos intervalos intercuartílicos. Resultados: Se revisaron 20 pacientes que habían recibido este tratamiento y se evidenció una tasa de éxito del 80%. Las complicaciones por disfunción del stent requirieron resolución quirúrgica. Egresaron de la institución el 95% de los pacientes luego del procedimiento y lograron arribar a un segundo estadio quirúrgico definitivo 17 pacientes. Fallecieron tres pacientes luego del procedimiento, pero sin relación directa con este. Conclusiones: Las indicaciones de angioplastia del conducto con stent como tratamiento alternativo a la realización de una anastomosis sistémico-pulmonar por cirugía todavía no están completamente definidas; la estrategia constituye una alternativa válida en pacientes adecuadamente seleccionados. La experiencia presentada muestra resultados similares a los de otros centros de referencia internacional.

Atención de enfermería en preescolar con atresia pulmonar en oxigenación con membrana extracorpórea: caso exitoso / Nursing Care in Kindergarten with Pulmonary Atresia in Extracorporeal Membrane Oxygenation: a Succesful Case / Cuidados de enfermagem em uma criança pré-escolar com atresia pulmonar em oxigenação por membrana extracorpórea: caso de sucesso

Introducción. La atresia pulmonar con comunicación interventricular es una cardiopatía compleja que enmarca grandes desafíos en su etapa pre y postquirúrgica; el uso del soporte vital extracorpóreo con membrana de oxigenación restablece la oxigenación y perfusión al organismo para permitir recuperación y complementar estudios. El objetivo de este caso clínico es determinar la atención de enfermería en la fase aguda post quirúrgica. Se expondrá usando el modelo teórico de Dorothea Orem: teorías de déficit de autocuidado y teoría de sistemas. Esta cardiopatía es la forma más severa de la tetralogía de Fallot. Tiene una incidencia del 2% entre todas las cardiopatías. Metodología. Es el caso de una preescolar con atresia pulmonar con comunicación interventricular, se describe los procesos realizados desde el diagnóstico, la intervención percutánea y reparo quirúrgico, así como el manejo de lesión residual en el marco del uso de diferentes tecnologías. Se resalta como elemento clave el uso del soporte con oxigenación con membrana extracorpórea usada como puente a decisión. Resultados. El plan de atención de enfermería en esta fase crítica logró los resultados planteados como la adecuada perfusión y oxigenación, recuperación de la función ventricular, estabilización hemodinámica para ser llevada al reparo de la lesión residual. Este novedoso soporte fue implementado dos veces durante la misma hospitalización y con resultados exitosos. Conclusiones. Caso de difícil manejo con terapias convencionales, pero con aplicación de una atención integral de enfermería; el uso de tecnología y de diversas especialidades permitió un egreso de la menor sin complicaciones. Palabras clave: Atresia Pulmonar; Oxigenación por membrana extracorpórea; Atención de Enfermería; Unidades de Cuidado Intensivo Pediátrico; Cardiopatías Congénitas.

Introduction. The pulmonary atresia with ventricular septal defect is a complex heart disseas that possess great challenges in pre and post-surgical stages; the use of vital support extracorporeal with membrane oxygenation restores oxygen and perfusion to the body to allow recovery and complement studies. The objetive of this case report is to determinate the nurse attention in the acute post quirulgical phase. It will be presented using Dorothea Orem's theoretical model: theories of self-care deficits and systems theory. This heart disease is the most severe form of tetralogy Fallot. It has an incidence of 2% among all heart diseases. Methodology. This is the case of a kindergarten with pulmonary atresia with ventricular septal defect, the processes carried out from diagnosis, percutaneous intervention and surgical repair are described, as well as the management of residual injury within the framework of the use of different technologies. The use of extracorporeal membrane oxygenation support used as a decision bridge is highlighted as a key element. Results. The nursing care plan in the critical phase achieved the results proposed as adequate perfusion and oxygenation, recovery of the ventricular function, hemodynamic stabilization to be carried out to repair the residual injury. This newfangled support was implemented twice during the same hospitalization with sucessful result. Conclusions. Case report with struggle managment with conventional therapies but with the application of comprehensive nursing care; the use of technology and the work of various specialities allowed the minor to be discharged without complications. Keywords: Pulmonary Atresia; Extracorporeal Membrane Oxygenation; Nursing Care; Intensive Care Units, Pediatric; Heart Defects, Congenital.

Introdução. A atresia pulmonar com comunicação interventricular é uma cardiopatia complexa que apresenta grandes desafios em sua fase pré e pós-cirúrgica. O uso de suporte de vida extracorpóreo com membrana de oxigenação restaura a oxigenação e a perfusão do corpo para permitir a recuperação e complementar os estudos. O objetivo deste caso clínico é determinar os cuidados de enfermagem na fase aguda pós-cirúrgica. Será apresentado utilizando o modelo teórico de Dorothea Orem: teorias do déficit de autocuidado e teoria de sistemas. Esta doença cardíaca é a forma mais grave de tetralogia de Fallot. Tem uma incidência de 2% entre todas as doenças cardíacas. Metodologia. É o caso de uma criança em idade pré-escolar com atresia pulmonar com comunicação interventricular, são descritos os processos realizados desde o diagnóstico, intervenção percutânea e reparação cirúrgica, bem como o manejo da lesão residual no âmbito da utilização de diferentes tecnologias. Destaca-se, como elemento-chave, a utilização de suporte com oxigenação por membrana extracorpórea como ponte para a decisão. Resultados. O plano de cuidados de enfermagem nesta fase crítica alcançou os resultados propostos como perfusão e oxigenação adequadas, recuperação da função ventricular, estabilização hemodinâmica a ser realizada para o reparo da lesão residual. Este novo suporte foi implementado duas vezes durante a mesma hospitalização e com resultados bem-sucedidos. Conclusões. Caso de difícil manejo com terapias convencionais, mas com aplicação de cuidados integrais de enfermagem, o uso da tecnologia e de diversas especialidades permitiu que a criança recebesse alta sem complicações. Palavras-chave: Atresia Pulmonar; Oxigenação por Membrana Extracorpórea; Cuidados de Enfermagem; Unidades de Terapia Intensiva Pediátrica; Cardiopatias Congênitas.

Frequency of chromosome 22q11.2 deletion among newborns with non-syndromic congenital heart defects from western Mexico.

BACKGROUND: Congenital heart defects (CHD) are among the most frequent manifestations of 22q11.2 deletion syndrome. Although we found relatively few studies aimed at specifically detecting 22q11.2 deletion in newborns (NB) with CHD, none of them has been performed in Mexico. METHODS: We conducted a prospective hospital-based study from January 2017 to March 2021 in the Genetics and Pediatric Cardiology Services of the Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, Mexico). All consecutive NBs identified with any non-syndromic major CHD confirmed by echocardiography were eligible to participate. A total of 98 NBs were included, 51 males and 47 females. Fluorescence in situ hybridization (FISH) analysis was conducted to search for deletion of chromosome 22q11.2 in interphase nuclei of standard lymphocyte cultures. RESULTS: We found eight patients (8.2%) with CHD and the 22q11.2 deletion, all of them with conotruncal defects, particularly of the truncus arteriosus (p = 0.013), tetralogy of Fallot (p = 0.024), and pulmonary atresia with ventricular septal defect (p = 0.031) subtypes. With de exception of one infant with hypocalcemia and another with hypocalcemia and thymic aplasia, the diagnosis of 22q11.2 deletion was not clinically suspected in the other patients. CONCLUSIONS: Our results confirm the importance of excluding the presence of the 22q11.2 deletion in every NB with CHDs, particularly of the conotruncal subtype, even in the absence of other manifestations.

INTRODUCCIÓN: Las cardiopatías congénitas (CC) son una de las manifestaciones más frecuentes del síndrome de deleción 22q11.2. A pesar de que existen relativamente pocos estudios dirigidos a detectar específicamente la deleción 22q11.2 en recién nacidos (RN) con CC, ninguno de ellos ha sido realizado en México. MÉTODOS: Se realizó un estudio prospectivo de base hospitalaria desde enero de 2017 hasta marzo de 2021 en los Servicios de Genética y Cardiología Pediátrica del Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, México). Todos los RN consecutivos identificados con cualquier tipo de CC mayor no sindrómica confirmada por ecocardiografía fueron elegibles para participar. Se incluyeron 98 recién nacidos, 51 de sexo masculino y 47 de sexo femenino. Mediante el análisis de hibridación fluorescente in situ (FISH, por sus siglas en inglés) se realizó la búsqueda de la deleción del cromosoma 22q11.2 en núcleos en interfase de cultivos de linfocitos estándar. RESULTADOS: Se encontraron ocho pacientes (8.2%) con CC y la deleción 22q11.2, todos ellos con defectos conotruncales, particularmente de los subtipos tronco arterioso (p = 0.013), tetralogía de Fallot (p = 0.024) y atresia pulmonar con comunicación interventricular (p = 0.031). Con excepción de un lactante con hipocalcemia y otro con hipocalcemia y aplasia tímica, el diagnóstico de deleción 22q11.2 no se sospechó clínicamente en los demás pacientes. CONCLUSIONES: Los resultados de este trabajo confirman la importancia de excluir la presencia de la deleción 22q11.2 en todos los RN con CC, particularmente del subtipo conotruncal, incluso en ausencia de otras manifestaciones.

Frequency of chromosome 22q11.2 deletion among newborns with non-syndromic congenital heart defects from western Mexico / Frecuencia de la deleción del cromosoma 22q11.2 en recién nacidos con cardiopatías congénitas no sindrómicas del occidente de México

Abstract Background: Congenital heart defects (CHD) are among the most frequent manifestations of 22q11.2 deletion syndrome. Although we found relatively few studies aimed at specifically detecting 22q11.2 deletion in newborns (NB) with CHD, none of them has been performed in Mexico. Methods: We conducted a prospective hospital-based study from January 2017 to March 2021 in the Genetics and Pediatric Cardiology Services of the Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, Mexico). All consecutive NBs identified with any non-syndromic major CHD confirmed by echocardiography were eligible to participate. A total of 98 NBs were included, 51 males and 47 females. Fluorescence in situ hybridization (FISH) analysis was conducted to search for deletion of chromosome 22q11.2 in interphase nuclei of standard lymphocyte cultures. Results: We found eight patients (8.2%) with CHD and the 22q11.2 deletion, all of them with conotruncal defects, particularly of the truncus arteriosus (p = 0.013), tetralogy of Fallot (p = 0.024), and pulmonary atresia with ventricular septal defect (p = 0.031) subtypes. With de exception of one infant with hypocalcemia and another with hypocalcemia and thymic aplasia, the diagnosis of 22q11.2 deletion was not clinically suspected in the other patients. Conclusions: Our results confirm the importance of excluding the presence of the 22q11.2 deletion in every NB with CHDs, particularly of the conotruncal subtype, even in the absence of other manifestations.

Resumen Introducción: Las cardiopatías congénitas (CC) son una de las manifestaciones más frecuentes del síndrome de deleción 22q11.2. A pesar de que existen relativamente pocos estudios dirigidos a detectar específicamente la deleción 22q11.2 en recién nacidos (RN) con CC, ninguno de ellos ha sido realizado en México. Métodos: Se realizó un estudio prospectivo de base hospitalaria desde enero de 2017 hasta marzo de 2021 en los Servicios de Genética y Cardiología Pediátrica del Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, México). Todos los RN consecutivos identificados con cualquier tipo de CC mayor no sindrómica confirmada por ecocardiografía fueron elegibles para participar. Se incluyeron 98 recién nacidos, 51 de sexo masculino y 47 de sexo femenino. Mediante el análisis de hibridación fluorescente in situ (FISH, por sus siglas en inglés) se realizó la búsqueda de la deleción del cromosoma 22q11.2 en núcleos en interfase de cultivos de linfocitos estándar. Resultados: Se encontraron ocho pacientes (8.2%) con CC y la deleción 22q11.2, todos ellos con defectos conotruncales, particularmente de los subtipos tronco arterioso (p = 0.013), tetralogía de Fallot (p = 0.024) y atresia pulmonar con comunicación interventricular (p = 0.031). Con excepción de un lactante con hipocalcemia y otro con hipocalcemia y aplasia tímica, el diagnóstico de deleción 22q11.2 no se sospechó clínicamente en los demás pacientes. Conclusiones: Los resultados de este trabajo confirman la importancia de excluir la presencia de la deleción 22q11.2 en todos los RN con CC, particularmente del subtipo conotruncal, incluso en ausencia de otras manifestaciones.

Pulmonary atresia with intact ventricular septum: Intended strategies.

OBJECTIVE: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies. METHODS: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies. RESULTS: Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of -1.59 (interquartile range, -3.03 to 0.21) and -5.12 (interquartile range, -5.60 to -4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001). CONCLUSIONS: Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.

Surgical Strategies and Results for Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals: Experience of a Single Tertiary Center.

OBJECTIVE: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). METHODS: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. RESULTS: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. CONCLUSION: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Caso Clínico: Tetralogía de Fallot Extrema / Case Report: Extreme Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot representa la cardiopatía congénita cianógena más frecuente después del primer año de vida, ocurre aproximadamente en 1 de cada 3 500 nacimientos y representa el 7 al 10% de todas las Enfermedades Cardiacas Congénitas. Su variante extrema que se acompaña de atresia pulmonar representa el 1 a 2% de las cardiopatías congénitas. Presentamos una Tetralogía de Fallot Extrema Ductus Dependiente de manejo complejo. CASO CLÍNICO: Se trata de un neonato a término de 12 días de vida, adecuado para la edad gestacional, sin factores de riesgo prenatales, ni antecedentes patológicos familiares, el cual es diagnosticado de Tetralogía de Fallot Extrema por ecocardiograma. EVOLUCIÓN: Paciente inicialmente tratado con infusión de prostaglandina E1. Posteriormente mediante fístula Blalock-Taussig izquierda. CONCLUSIONES: Para mejorar el pronóstico y sobrevida de un paciente con Tetralogía de Fallot Extrema el diagnóstico prenatal oportuno y el tratamiento apropiado es de vital importancia, sin embargo el tratamiento definitivo con derivaciones pulmonares sistémicas representan complicaciones con riesgo de mortalidad. El presente caso tuvo un desenlace desfavorable.

BACKGROUND: The Tetralogy of Fallot represents the most common cyanogenic heart disease after the first year of life. It occurs in 1 of 3 500 live births and accounts as 7 to 10% of all Congenital Heart Diseases. Its extreme variant is accompanied by pulmonary atresia and accounts 1 to 2% of the Congenital Heart Diseases. We present an Extreme Tetralogy of Fallot­ Ductus Dependent of complex management. CLINICAL CASE: A full term 12 day neonate, adequate for gestational age, without prenatal risk factors or pathologic family antecedents, is diagnosed with Extreme Tetralogy of Fallot by echocardiogram. EVOLUTION: The patient was treated initially with an infusion of prostaglandin E1. Then, a left Blalock-Taussig shunt was performed. CONCLUSIONS: To improve the prognosis and survival rates of patients with Extreme Tetralogy of Fallot , a timely diagnosis and treatment are of vital importance. Definitive treatment with systemic pulmonary shunts carries high risk complications. The present case had an unfavorable evolution.

Plan de alta y seguimiento domiciliario a un preescolar con alteraciones funcionales y morfológicas del corazón / Discharge plan and follow a preschool house with functional and morphological alterations of the heart", "_e": "n

La sobrevida de las personas posoperadas de Fontan va en aumento. En los años 70 la sobrevivencia era del 75 al 80%, logrando en la actualidad una sobrevida hasta del 90% llegando a una edad promedio de 14 años. El objetivo del estudio de caso es elaborar un plan de cuidados especializado, individualizado e integral durante el alta y seguimiento domiciliario a un preescolar con alteraciones funcionales y morfológicas del corazón por ventrículo único. El presente estudio de caso es de un preescolar con atresia pulmonar y comunicación interventricular dependiente de conducto arterioso. Las etapas quirúrgicas por las cuales pasó el niño fue una fístula sistémico-pulmonar de Blalock-Taussig en el 2009 a los dos meses de vida y que al no estar funcional se realizó cirugía de Fontan a los cuatro años de edad; posterior a la cirugía se dio seguimiento domiciliario donde se aplicó el proceso de atención de enfermería basado en el modelo de Virginia Henderson, para brindar un cuidado especializado, que tiene como objetivo la reincorporación de la persona a sus actividades de la vida diaria con el apoyo del cuidador primario, mediante la elaboración de un plan de alta como herramienta para lograr una continuidad en el cuidado disminuyendo complicaciones a corto y largo plazo. Se llegó a la conclusión de la importancia del plan de alta para la continuidad de cuidados para evitar reingresos hospitalarios derivados del posoperatorio de Fontan, así mismo se detectaron de manera oportuna los signos de alarma que podían alterar el estado de salud del preescolar.

The survival of pos-operated Fontan people is increasing. In the 70’s the survival was about 75 to 80%, making today a survival rate to 90% reaching an average age of 14 years. The purpose of this case study is to develop a specialized, individualized and comprehensive care plan during the discharge and home monitoring to a preschool with functional and morphological alterations of the heart of unique ventricle. This case study is about a preschool with pulmonary atresia with ventricular septal dependent ductus arteriosus. The surgical stages that passed the child were a systemic pulmonary Blalock-Taussig fistula in 2009 at 2 months of life, and that not being functional a Fontan surgery was performed at 4 years old, after the surgery he was given a home monitoring where the nursing care process based on the model of Virginia Henderson was applied to provide specialized care that has the objective of return the person to their activities of daily life with the support of the primary caregiver through the development of a discharge plan as a tool to achieve a continuity of care to reduce complications at short and long term. Coming to the conclusion of the importance of the discharge plan for the continuity of care, avoiding hospital readmissions for the Fontan posoperative and also detect early warning signs that could alter the health status of the preschool.

Combined Rastelli and Bentall Procedures for Pulmonary Atresia With Ventricular Septal Defect and a Large Aortopulmonary Collateral Artery and Ascending Aorta Aneurysm.

We report a challenging case of a 32-year-old patient in New York Heart Association (NYHA) class IV with pulmonary atresia, ventricular septal defect, a left aortopulmonary collateral artery, a right modified Blalock-Taussing shunt, and a gigantic aneurysm of the ascending aorta with severe aortic valve insufficiency. A combined Rastelli and Bentall procedure was performed by a joint adult and pediatric cardiovascular surgery team. One year after the operation, the patient is in NYHA class I, working full time.

Atresia pulmonar con septo interventricular intacto y ausencia de desarrollo valvular tricúspide. Presentación de cuatro casos / Pulmonary Atresia with Intact Ventricular Septum and Absence of Tricuspid Valve. A Report of Four Cases

La atresia pulmonar con septo interventricular intacto sugiere una sencilla malformación congénita del corazón. Nada más alejado de la realidad que esta suposición. Dentro de las peculiaridades morfológicas de esta enfermedad, raramente se observa la existencia de orificio valvular tricúspide congénitamente desguarnecido. La presente comunicación posee el objetivo de describir una serie de cuatro pacientes, con ambas malformaciones, detectadas en el Cardiocentro Pediátrico William Soler durante el período comprendido de 1986 a 2012. El diagnóstico ecocardiográfico pudo ser efectuado con precisión en los últimos dos pacientes; la determinación de los casos iniciales correspondió a los hallazgos obtenidos de la necropsia. A todos se les practicó fístula quirúrgica de Blalock – Taussig como tratamiento paliativo inicial, en dos de ellos asociada a atrioseptostomía de balón. Solo uno de estos últimos niños sobrevivió. El pronóstico vital de los pacientes que padecen esta asociación de anomalías, radica en la identificación ecocardiográfica temprana.

Pulmonary atresia with intact ventricular septum suggests a simple congenital heart malformation. However, nothing could be further from the truth. Among the morphological peculiarities of this condition, the presence of congenitally unguarded tricuspid valve orifice is rarely observed. The present paper aims at describing a series of four patients with both malformations detected in the William Soler Children’s Heart Center from 1986 through 2012. Echocardiographic diagnosis could be accurately performed in the last two patients; identification of the initial cases was established by the findings at necropsy. All patients underwent Blalock – Taussig shunt as initial palliative treatment, which was associated with balloon atrioseptostomy in two cases. Only one of the last children mentioned survived. Vital prognosis of patients suffering from this association of malformations lies in early echocardiographic detection.

Oclusión de colateral aorto-pulmonar con dispositivo Amplatzer® Vascular Plug II en un paciente con atresia pulmonar y comunicación interventricular: Reporte de un caso / Occlusion of aortopulmonary collateral arteries with Amplatzer® Vascular Plug II device in a patient with pulmonary atresia and ventricular septal defect: Case report

Introducción. La atresia pulmonar con comunicación interventricular es una cardiopatía compleja con una incidencia aproximada de 2% entre todas las cardiopatías congénitas. Se asocia con frecuencia al síndrome de deleción 22q11. Tiene una amplia variabilidad anatómica que es necesario precisar con exactitud para poder establecer un plan médico quirúrgico individualizado. Caso clínico. Se presenta el caso de un paciente de 2 años con atresia pulmonar y comunicación interventricular asociadas a dos grandes colaterales aortopulmonares. Este paciente fue corregido mediante conexión de ventrículo derecho a arteria pulmonar, durante la cual fue posible ligar una de las colaterales; la restante fue embolizada mediante un dispositivo trascateterismo con oclusión total. Conclusiones. El manejo de las arterias colaterales asociadas a atresia pulmonar con comunicación interventricular es complejo pero accesible al tratamiento trascateterismo mediante el implante de dispositivos con mínima morbimortalidad.

Background. Pulmonary atresia with ventricular septal defect (VSD) is a complex heart disease with an incidence of ~2% of all congenital heart diseases. It is frequently associated with 22q11 deletion syndrome. Due to the extensive anatomic variability, it is necessary to accurately establish an individualized surgical/medical plan. Case report. We report the case of a 2-year-old patient with pulmonary atresia and two associated mayor aortopulmonary collateral arteries. This patient underwent right ventricular-pulmonary artery connection at which time it was possible to ligate one of the collaterals. The remaining were embolized by transcatheter device with total occlusion. Conclusions. Management of collateral arteries associated with pulmonary atresia with VSD is complex but is accessible with transcatheter treatment with device implantation with minimal morbidity.

Atresia pulmonar con septum interventricular intacto, relevancia clínica del tratamiento paliativo aplicado en nuestro medio / Pulmonary atresia with intact interventricular septum- the clinical relevance of the palliative treatment in our context

Introducción: la atresia pulmonar con septum interventricular intacto es una cardiopatía congénita poseedora de complejidad a la hora de tomar decisiones terapéuticas ante cada paciente; por esa razón el enfrentamiento exitoso de la entidad constituye una asignatura pendiente para la Cardiología Pediátrica. Objetivo: determinar el grado de relevancia clínica existente en las modalidades terapéuticas paliativas aplicadas ante la enfermedad en nuestro medio. Métodos: se evaluaron 43 niños con diagnóstico confirmado de la entidad remitidos al Cardiocentro Pediátrico William Soler, y sometidos a tratamiento paliativo inicial en el período comprendido entre enero de 1992 y noviembre de 2011. Para determinar la relevancia clínica del proceder aplicado se llevó a cabo un estudio de efectividad que comprendió el análisis de la incidencia/prevalencia de eventos bien definidos (mortalidad precoz y presencia de causa letal principal) en cada variante de tratamiento impuesta. Resultados: la realización de fístula quirúrgica asociada a atrioseptostomía revistió altos índices de relevancia clínica. Conclusiones: se documentó escasa incidencia/prevalencia de mortalidad precoz y reducida ocurrencia de insuficiencia respiratoria como complicación letal al ser aplicado este proceder híbrido, en comparación al resto de las modalidades terapéuticas estudiadas

Introduction: pulmonary atresia with intact interventricular septum is a complex congenital cardiopathy at the time of making therapeutic decisions in each case; this is the reason why the successful management of this disease is a problem to be solved by the pediatric cardiology. Objective: to determine the existing level of clinical relevance of palliative therapies applied in our context. Methods: evaluation of 43 children with confirmed diagnosis of the disease, who had been referred to William Soler pediatric cardiological center and had undergone initial palliative treatment in the period of January 1992 through November, 2011. With the objective of determining the clinical relevance of this procedure, an effectiveness study including the analysis of incidence/prevalence of well-defined events (early mortality and main lethality causes) in each therapeutic variant used in patients was conducted. Results: performance of surgical fistula associated with atrioseptostomy showed high indexes of clinical relevance. Conclusions: there were low incidences and prevalence indexes of early mortality and also reduced occurrence of respiratory failures as lethal complication from this combined procedure, compared with the rest of the therapeutic modalities under study

Morfología ecocardiográfica de la atresia pulmonar con septum interventricular intacto, estudio de dos decenios / Echocardiographic morphology of the pulmonary atresia with intact ventricular septum. A 20-year study

Introducción: la atresia pulmonar con septum interventricular intacto es una malformación cardiovascular que representa el 1 % de las cardiopatías congénitas observadas en vida extrauterina, y, dados sus resultados desfavorables, constituye un verdadero reto para la medicina contemporánea. Objetivo: la investigación condujo a la aplicación de pautas clasificatorias, a la caracterización del tracto de salida atrésico, al estudio morfológico ventricular derecho, a la valoración del anillo tricuspídeo y a la determinación de la presencia de anomalías en la circulación coronaria. Métodos: se estudiaron 43 pacientes con diagnóstico confirmado de la entidad remitidos al Cardiocentro Pediátrico "William Soler" entre enero de 1992 y noviembre de 2011. Se practicó a cada caso el examen ecocardiográfico bidimensional y doppler con codificación en colores. Resultados y conclusiones: se corroboró el predominio de la variante morfológica valvular de la entidad y la existencia de niveles moderados o severos de hipoplasia ventricular derecha en asociación con capacitancia volumétrica limitada de dicha cámara, con hipodesarrollo valvular tricuspídeo y con presencia de circulación coronaria anómala sinusoides dependiente. El foramen oval permeable constituyó el defecto septal interatrial más vinculado a la enfermedad, y se evidenciaron diversas anomalías estructurales del aparato tricuspídeo en conjunción o no con el hipodesarrollo anular imperante.

Introduction: pulmonary atresia with intact ventricular system is a cardiovascular malformation accounting for 1% of congenital cardiopathies seen in the extrauterine life; due to its unfavorable outcomes, this disease is a true challenge for the contemporary medicine. Objectives: the research study comprised the application of the classification guidelines, the characterization of the atresia outlet track, the morphological study of the right ventricle, the assessment of the tricuspid ring and the detection of anomalies in the coronary circulation. Methods: forty three patients diagnosed with this disease and referred to "William Soler" pediatric cardiocenter from January 1992 to November 2011 were studied. Each case was performed bidimensional echocardiography and color-coding Doppler. Results and conclusions: the study confirmed the predominance of the valvular morphological variant of the disease and the presence of moderate and severe levels of right ventricular hypoplexia associated with limited volumetric capacitance of this chamber, with tricuspid valvular hypodevelopment and with anomalous sinusoids-dependent coronary circulation. The permeable oral foramen was the interatrial septal defect that was most associated to this disease and several structural anomalies of the tricuspid system was evidenced in conjunction or not with the prevailing annular hypodevelopment.

Atresia pulmonar con septum interventricular intacto / Pulmonary atresia with intact ventricular septum, a thematic review

La atresia pulmonar con septum interventricular intacto es una cardiopatía congénita cianótica aparentemente sencilla caracterizada por incorporar diversas variantes morfológicas y lesiones asociadas que conllevan a un diagnóstico y tratamiento más complejo del que inicialmente podría esperarse. La elevada mortalidad antes de los 6 meses de edad obliga a instaurar una conducta agresiva y precoz. Con esta revisión nos hemos propuesto efectuar la evaluación integral de la enfermedad desde sus elementos conceptuales hasta las variantes terapéuticas a adoptar, para lo cual se describen los aspectos más importantes en cuanto a morfología, fisiopatología, diagnóstico y tratamiento. Se discute, asimismo, el papel de la ecocardiografía, de la cirugía y de las técnicas de cardiología intervencionista. La estrategia fundamental del tratamiento es separar las circulaciones sistémica y pulmonar sin provocar disminución del gasto cardíaco ni aumentos en la presión venosa central. Se pretende, además, rescatar al ventrículo derecho para el circuito pulmonar siempre que la circulación coronaria no sea dependiente de aquel. Se resalta, mediante algoritmo, la importancia que reviste la integración de modalidades quirúrgicas, intervencionistas e híbridas en el acometimiento terapéutico de esta cardiopatía.

Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital cardiopathy, characterized by several morphological variants and associated lesions leading to a complex diagnosis and treatment. The high mortality rate recorded in under 6 months-old infants demands an early aggressive management to face this situation. This review was intended to make a comprehensive assessment of the disease from its basic conceptual elements to the therapeutic variants to be adopted. To this end, the most important elements of morphology, physiopathology, diagnosis and treatment were addressed, in addition to discussing the roles of echocardiography, surgery and interventional cardiology techniques. The fundamental strategy of treatment is to separate the systemic circulation from the pulmonary circulation, causing neither reduction of the cardiac output nor increase of the central venous pressure. It is also aimed to return the right ventricle to the pulmonary circuit, provided that the coronary circulation does not depend on it. The algorithm presented in this paper underlined the importance of the integration of the surgical, interventional and hybrid modalities for the therapeutic management of this cardiopathy.

Truncus arteriosus operado aos 28 anos: importância do diagnóstico diferencial / Atypical truncus arteriosus operated at 28 years of age: importance of differential diagnosis / Truncus arteriosus operado a los 28 años: importancia del diagnóstico diferencial

Descrevemos um caso de adulto de 28 anos com suspeita de cardiopatia congênita desde o nascimento, não tratada na infância por opção da família. Aos 27 anos, foi feito diagnóstico de atresia pulmonar com comunicação interventricular e colaterais sistêmico-pulmonares, sendo contraindicada a cirurgia. Uma nova reavaliação em nosso serviço demonstrou tratar-se de um truncus arteriosus atípico. O fato de um tronco arterial comum com shunt esquerda-direita ter sido visualizado ao ecocardiograma foi um dado crucial para a indicação de novo cateterismo, abrindo perspectiva de correção cirúrgica. No momento, o paciente encontra-se bem, com 7 anos de evolução pós-operatória.

This is the case of 28 year-old adult with suspected congenital heart disease since birth, not treated in childhood at the his family's choice. At 27 years old, he was diagnosed with pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals, where surgery was contraindicated. A new review in our department showed that it was an atypical form of truncus arteriosus. The fact that a common arterial trunk with left-right shunt was viewed by echocardiography was a crucial fact for the indication of new catheterization, opening the prospect of surgical correction. Currently, the patient is well, with 7 years of postoperative outcome.

Describimos un caso de adulto de 28 años con sospecha de cardiopatía congénita desde el nacimiento, no tratada en la niñez por opción de la familia. A los 27 años, se hizo diagnóstico de atresia pulmonar con comunicación interventricular y colaterales sistémico-pulmonares, y se contraindicó la cirugía. Una nueva reevaluación en nuestro servicio reveló tratarse de un truncus arteriosus atípico. El hecho de un tronco arterial común con shunt izquierdaderecha haber sido visualizado al ecocardiograma fue un dato crucial para la indicación de nuevo cateterismo, abriendo perspectiva de corrección quirúrgica. Al momento, el paciente se encuentra bien, con 7 años de evolución posoperatoria.