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.

Interventional treatment and early-stage follow-up of pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.

Outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting / 中华儿科杂志

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.

Efficacy of transcatheter pulmonary valve perforation in neonates with pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the efficacy and safety of transcatheter pulmonary valve perforation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The clinical data on surgical treatment and follow-up in 16 patients with PA-IVS who underwent transcatheter pulmonary valve perforation in Women and Children's Hospital, Qingdao University from October 2018 to October 2021 were analyzed retrospectively. The right ventricular systolic pressure and percutaneous oxygen saturation (SpO2) were compared before and after operation. In addition, the SpO2 and echocardiographic data at preoperative and the last follow-up were compared. Comparisons between groups were performed using paired-samples t test. Results: Among the 16 patients (10 males and 6 females) with the age at operation of 19 (14, 26) days, 12 cases underwent transcatheter pulmonary valve perforation successfully, 2 cases were transferred to surgery department for open-heart pulmonary valvulotomy, and the remaining 2 cases were transmitted to surgery department for transthoracic pulmonary valve perforation. The age at operation of the 12 patients who underwent transcatheter pulmonary valve perforation was 18 (14, 27) days, and the weight was (3.6±0.4) kg. The immediate postoperative right ventricular systolic pressure decreased significantly ((57±16) vs. (95±19) mmHg (1 mmHg=0.133 kPa), t=7.49, P<0.001), and the postoperative SpO2 was improved effectively (0.90±0.48 vs.0.75±0.09, t=-5.61, P<0.001). The follow-up time was 22 (7, 33) months for 12 patients who underwent transcatheter pulmonary valve perforation successfully. At the last follow-up, the ratio of right to left ventricular transverse diameter was significantly higher than that before operative (0.55±0.05 vs. 0.45±0.05, t=-3.27,P=0.007). Furthermore, the Z-scores of pulmonary valvular diameter (-0.78±0.23 vs. -1.73±0.56, t=-8.52, P<0.001) and the tricuspid valvular diameter (-0.52±0.12 vs. -1.46±0.38, t=-10.40, P<0.001) were all significantly higher than preoperative data. At last, all the patients achieved biventricular circulation without death or major complications. Conclusion: Transcatheter pulmonary valve perforation is a safe and effective therapy for neonatal PA-IVS, and its curative effect has been confirmed by the medium follow-up data.

Atresia bronquial / Bronchial atresia

Introducción: La atresia bronquial es una patología poco frecuente, generalmente asintomática e incidental en las imágenes del tórax. La aproximación diagnóstica de esta entidad se puede hacer por radiografía y broncoscopia, donde se identifican algunos aspectos claves en el diagnóstico diferencial. De acuerdo las manifestaciones clínicas y posibles complicaciones el tratamiento puede ser desde conservador hasta quirúrgico con resección lobar o segmentaria. Objetivo: Presentar las características de un caso con atresia bronquial. Presentación de caso: Se presenta un caso de atresia bronquial en una paciente de 19 años de edad cuyo tratamiento definitivo fue bilobectomía media-inferior derecha. Conclusiones: La atresia bronquial es una entidad infrecuente que puede cursar de manera asintomática y ser detectada por un hallazgo radiológico en pacientes adultos de manera incidental. El diagnóstico se puede confirmar por broncoscopia y el tratamiento casi siempre es quirúrgico(AU)

Introduction: Bronchial atresia is a rare disease, generally asymptomatic and incidental in chest images. The diagnostic approach of this entity can be done by radiography and bronchoscopy, some key aspects are identified in the differential diagnosis. According to the clinical manifestations and possible complications, the treatment can range from conservative to surgical with lobar or segmental resection. Objective: To describe a case of bronchial atresia. Case report: A case of bronchial atresia is reported in a 19-year-old patient whose definitive treatment was a right-lower-middle bilobectomy. Conclusions: Bronchial atresia is a rare entity that can occur asymptomatically and be detected incidentally by a radiological finding in adult patients. The diagnosis can be confirmed by bronchoscopy, and treatment is almost always surgical(AU)

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.

Descripción anatómica, clínica y sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en el ductus arterioso persistente en el periodo 2009-2014 / Anatomical and clinical description and survival of patients with pulmonary atresia subjected to angioplasty with ductal stent in the period 2009-2014

Resumen Objetivo: describir las características anatómicas, la evolución clínica y la sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en ductus arterioso persistente y determinar si existen diferencias significativas en la saturación al inicio y final del procedimiento y a las 6, 12 y 24 horas posprocedimiento. Materiales y métodos: estudio analítico, descriptivo tipo cohorte. La población de estudio se tomó de las bases de datos del servicio de Cardiología pediátrica de una institución especializada en la ciudad de Bogotá, Colombia. Se incluyeron pacientes con diagnóstico de atresia pulmonar sometidos a angioplastia, en tanto que se excluyeron aquellos con cirugía cardiovascular previa. El análisis estadístico se realizó en STATA® 12,0, mediante estadística descriptiva, curvas de Kaplan-Meier y ANOVA de medidas repetidas. Resultados: se identificaron 237 pacientes con atresia pulmonar. Se seleccionaron 30 de los cuales se excluyeron 5. En total se construyó una cohorte de 25 pacientes que fueron llevados a angioplastia, 52% con atresia pulmonar con comunicación interventricular. Edad promedio 12,2 días, peso 2.698 g, diámetro del DAP 3,8 mm (DE = 1,1), índice de McGoon media 1,35. El implante del stent fue exitoso en todos los pacientes; sin embargo 2 fallecieron en la sala de procedimientos y uno durante las 6 horas siguientes. Las complicaciones más frecuentes fueron paro cardiorrespiratorio (n = 2), trombosis del stent (n = 2) y bacteriemia (n = 2). La mortalidad a 30 días fue del 28% (n = 7); durante el tiempo total fue del 36% (n = 9). Por medio del ANOVA no se encontraron diferencias significativas en los promedios de saturación en los cinco tiempos de interés. Conclusiones: la atresia pulmonar es una patología compleja que requiere intervención invasiva para el mantenimiento de la capacidad circulatoria de los pacientes. Este estudio muestra que a pesar de una intervención efectiva, la mortalidad a los 30 días es elevada. No se encontraron diferencias en los tiempos de saturación, lo que sugiere que mantiene la saturación de los pacientes de una forma no distinta que con la prostaglandina E (PGE1) pero garantiza la permeabilidad del ductus.

Abstract Objective: the aim of this study is to describe the anatomical characteristics, the clinical progression, and survival of patients with pulmonary atresia subjected to angioplasty with a stent in patent ductus arteriosus (PDA), and to determine if there are significant difference in the saturation at the beginning and the end of the procedure, and at 6, 12, and 24 hours after the procedure. Materials and methods: An analytical and descriptive cohort study was conducted on a population taken from the database of a Paediatric Cardiology Department of a specialised institution in the city of Bogota, Colombia. Patients with pulmonary atresia subjected to angioplasty were included, and those with previous cardiovascular were excluded. The statistical analysis was performed using STATA® 12.0, by means of descriptive statistics, Kaplan-Meier curves and repeated measurements ANOVA. Results: A total of 237 patients with pulmonary atresia were identified. Of the 30 that were selected, 5 were excluded, leaving a total of 25 patients that were subjected to angioplasty. Interventricular communication was observed in 52%. The mean age was 12.2 days, with a mean weight of 2.698 g, a mean PDA diameter of 3.8 mm (SD = 1.1), and a mean McGoon index of 1.35. Although the stent implant was successful in all patients, two died in the procedures room, and another one during the following 6 hours. The most common complications were, cardiorespiratory arrest in 2 patients, thrombosis of the stent in 2, and bacteraemia in two patients. The mortality at 30 days was 28% (7), and 36% (9) during the total time. Using ANOVA, no significant differences were found in the saturation means at the five times of interest. Conclusions: Pulmonary atresia is a complicated condition that requires invasive intervention in order to maintain the circulatory capacity of the patients. This study shows that, despite an effective intervention, the 30-day mortality is elevated. No differences were, found in the saturation times, which suggest that saturation is maintained in the patients in a way not unlike that with prostaglandin E (PGE1), but ensures the patency of the ductus.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization / 대한소아소화기영양학회지

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Linfoma primario de pulmón tipo malt: presentación de caso clínico / Primary lung lymphoma MALT Type: Clinical case presentation

Antecedentes:Background: Los Linfomas Pulmonares constituyen menos del 15 % de todos los linfomas. Los linfomas primarios en pulmón son una entidad de poca frecuencia, más aún el subtipo llamado MALT (linfoma de tejido linfoide asociado a mucosas) estos representan apenas al 1 % de todos los linfomas y 0,5 % de todos los tumores pulmonares. Se caracterizan por proli-feración clonal linfoide que afecta a los bronquios o al parénquima pulmonar de forma unilateral o bilateral sin que exista evidencia de afectación extra torácica al momento de su diagnóstico. Caso Clinico: paciente de 33 años atendido en servicio de Neumología del Instituto Nacional Cardiopulmonar de Tegucigalpa, Honduras por historia de dolor pleurítico en hemitórax derecho, tos con expecto-ración hemoptoica de 3 semanas de evolución acompañado de estertores crepitantes en hemitórax anterior derecho con diagnóstico histológico e inmunohistoquimico mediante biopsia de linfoma pulmonar de tipo Malt. Discusión: Es importante tener en cuenta a este tipo de tumores, dado que los síntomas, signos clínicos y los estudios de imágenes son inespecíficos. (5) teniendo en cuenta los diagnósticos diferenciales de causas no infecciosas, como la enfermedad metastásica, la granulomatosis de Wegener, los linfomas, las leucemias, las neumonías criptogénicas, la enfermedad tromboembólica y las enfermedades del colágeno...(AU)

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.

Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center / 소아과

PURPOSE: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. METHODS: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. RESULTS: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. CONCLUSION: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.

Platypnea-Orthodeoxia Syndrome Two Decades after Definitive Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum

A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.

Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals Associated with Left Pulmonary Artery Interruption

A multistage plan and multidisciplinary approach are the keys to successful repair in patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). In this article, we present a multidisciplinary approach adopted to treat a patient with PA with VSD and MAPCAs associated with left pulmonary artery interruption.

Atresia bronquial: Reporte de dos casos en población adulta / Bronchial Atresia. Report of Two Cases in Adults

La atresia bronquial (AB) es una anomalía congénita poco frecuente y de etiología desconocida. Se caracteriza por la falla en el desarrollo de una porción de un bronquio con acumulación de secreciones bronquiales y atrapamiento aéreo distal a la lesión. El conocimiento de esta patología permite su incorporación dentro de los diagnósticos diferenciales de masas pulmonares.

Bronchial atresia (BA) is an uncommon congenital anomaly of unknown etiology. It is characterized by the failure to develop a portion of the bronchus with accumulation of bronchial secretions and air trapping distal to the lesion. The knowledge of this pathology can be taken into account within the differential diagnosis of lung masses.

Off-pump bidirectional Glenn through right anterior thoracotomy / Glenn bidirecional sem uso de CEC via toracotomia anterior direita

AbstractThe Glenn operation involving anastomosis of the superior vena cava to the pulmonary artery has been performed for palliative operations of many cyanotic congenital heart diseases in addition to the single ventricle since the 1960s. The classic procedure is done via median sternotomy and cardiopulmonary bypass. The benefits of this procedure without the use of cardiopulmonary bypass remain mixed within reported series. Cases using this approach and off-pump technique together in Latin-America have not yet been reported in the scientific literature.

ResumoA operação de Glenn envolvendo anastomose da veia cava superior à artéria pulmonar foi realizada como procedimento paliativo de muitas doenças cardíacas congênitas cianóticas, além do ventrículo único desde os anos 1960. O procedimento clássico é feito por esternotomia mediana e circulação extracorpórea. Os benefícios deste procedimento sem o uso de circulação extracorpórea permanecem incertos dentro da série relatada. Casos utilizando conjuntamente esta abordagem e a técnica sem circulação extracorpórea na América Latina ainda não foram relatados na literatura científica.

Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review / 华中科技大学学报（医学）（英德文版）

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.

Initial experience with the new Pul-Stent in treating postoperative branch pulmonary artery stenosis / 中华儿科杂志

<p><b>OBJECTIVE</b>The Pul-stent is a new cobalt alloys stent, specially licensed for pulmonary artery stenosis. The aim of this study was to investigate the value of the stent implantation as the treatment of postoperative pulmonary artery stenosis.</p><p><b>METHOD</b>Clinical practice was carried out to evaluate effectiveness, safety and maneuverability of Pul-stent in the defined clinical setting. Transthoracic echocardiography, chest roentgenography and electrocardiography were carried out as follow-up studies at 1 and 3 months after procedures.</p><p><b>RESULT</b>Ten patients (7 males and 3 females) received Pul-stent implantation in left or right pulmonary arteries (9 stents in left and 1 stent in right). For 9 cases transcatheter stent implantation was performed and in I case hybrid procedure. The median age was 7. 9 years (range 3. 4 - 13. 7 years). The median weight was 22. 3 kg (range 13 - 32 kg). Among them 6 cases were post surgical repair of tetralogy of Fallot, 2 cases were after Fontan procedures, 1 patient was post surgical repair of pulmonary atresia with ventricular septal defect, and 1 patient had stenosis at Glenn pathway. The pressure gradient at the stenosis decreased from (31. 6 ± 10. 2) mmHg(1 mmHg =0. 133 kPa) to (7. 3 ± 10. 3) mmHg, and the diameter of the narrowest segment increased from (4. 0 ± 2. 3) mm to (9. 6 ± 2. 7) mm, the right ventricle to aortic pressure ratio decreased from 0. 54 to 0. 36, all of these improvements were statistically significant (t = 3. 9, -9. 7, 4. 5; P =0. 008, 0. 000, 0. 004). The total procedure time ranged from 55 to 220 min (median 117 min) , and the fluoroscopy time ranged from 9 to 67 min (median 26 min). There were 2 cases of post-stenting pneumorrhagia. No stent fracture, stent malposition and other severe complications were observed. Initial follow-up of 1 and 3-months showed good results with maintenance of improved caliber of the stented vessel, and the gradient across the stenosis area measured by echocardiography was (32. 0 ± 14. 6) mmHg after 3 months. Compared with before stentifng ((40.6 ± 15. 2) mmHg) and 1 month later ((30. 6 ± 13. 6) mmHg), the difference was not statistically significant (t =2. 2,1. 76; P =0. 07, 0. 10).</p><p><b>CONCLUSION</b>Pul-Stent tracking and delivery was excellent, the initial experience has shown that Pul-stent implantation was effective and safe in treating post-operative branch pulmonary artery stenosis. Further follow-up study should be conducted to make sure whether those good results would be kept constant.</p>