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Resumen Antecedentes: Las cardiopatías congénitas plantean un desafío terapéutico, específicamente la estenosis de la válvula pulmonar. Esta ha sido tratada durante muchos años con procedimientos invasivos e inserción de bioprótesis, que con el tiempo se vuelven disfuncionales y pueden reestenosarse por acumulación de tejido fibroso y calcificación. Debido a las complicaciones generadas por la injuria quirúrgica, se han descrito medidas menos invasivas para el manejo de la estenosis residual e inicial por medios endovasculares en adultos y más recientemente en población pediátrica. Objetivo: El objetivo de este reporte es describir la misma en el manejo endovascular del tracto de salida del ventrículo derecho, como el inicio de un trabajo continuo para la mejoría de los resultados pediátricos en países en vía de desarrollo. Métodos: Se presentan siete casos pediátricos de manejo endovascular del tracto de salida derecho; tres de ellos sometidos a valvuloplastia quirúrgica con persistencia de la estenosis pulmonar, por lo cual se decidió inserción percutánea de una válvula pulmonar (IVPP) transcatéter con válvula Melody utilizando la técnica valve-in-valve, con lo que se consiguió una resolución del 100% de la estenosis y no se presentó ningún tipo de complicación asociada al procedimiento. Resultados: En cuatro pacientes se logró una implantación exitosa de la válvula por vía percutánea en diferentes cardiopatías congénitas, siendo uno de ellos en tracto nativo; además, destaca el caso de un paciente en quien se realizó fractura intencional de la válvula pulmonar, procedimiento innovador en el manejo endovascular pediátrico en Colombia. Conclusiones: En estos pacientes el procedimiento resultó ser poco invasivo, seguro y efectivo. La técnica IVPP podría ser considerada una opción viable en Colombia (y en otros países en desarrollo) para el manejo de implantes valvulares primarios fallidos o incluso en tractos nativos.

Abstract Background: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. Objective: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries. Methods: Seven pediatric patients with endovascular management of the right outflow tract are presented. Three of them underwent surgical valvuloplasty with persistent pulmonary stenosis. They decided to insert a percutaneous transcatheter pulmonary valve (PPVI) with a Melody valve using the valve-in-valve technique, with 100% stenosis and no complications associated with the procedure. Results: Four patients with successful percutaneous valve implantation had different congenital heart diseases. In addition, the case of a patient in whom an intentional pulmonary valve fracture was performed, an innovative procedure in pediatric endovascular management in the country, is highlighted. Conclusions: The procedure was minimally invasive, safe, and effective. The IVPP technique could be a viable option in our country for managing failed primary valve implantations or even in native tracts.

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BACKGROUND: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. OBJECTIVE: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries. METHODS: Seven pediatric patients with endovascular management of the right outflow tract are presented. Three of them underwent surgical valvuloplasty with persistent pulmonary stenosis. They decided to insert a percutaneous transcatheter pulmonary valve (PPVI) with a Melody valve using the valve-in-valve technique, with 100% stenosis and no complications associated with the procedure. RESULTS: Four patients with successful percutaneous valve implantation had different congenital heart diseases. In addition, the case of a patient in whom an intentional pulmonary valve fracture was performed, an innovative procedure in pediatric endovascular management in the country, is highlighted. CONCLUSIONS: The procedure was minimally invasive, safe, and effective. The IVPP technique could be a viable option in our country for managing failed primary valve implantations or even in native tracts.

ANTECEDENTES: Las cardiopatías congénitas plantean un desafío terapéutico, específicamente la estenosis de la válvula pulmonar. Esta ha sido tratada durante muchos años con procedimientos invasivos e inserción de bioprótesis, que con el tiempo se vuelven disfuncionales y pueden reestenosarse por acumulación de tejido fibroso y calcificación. Debido a las complicaciones generadas por la injuria quirúrgica, se han descrito medidas menos invasivas para el manejo de la estenosis residual e inicial por medios endovasculares en adultos y más recientemente en población pediátrica. OBJETIVO: El objetivo de este reporte es describir la misma en el manejo endovascular del tracto de salida del ventrículo derecho, como el inicio de un trabajo continuo para la mejoría de los resultados pediátricos en países en vía de desarrollo. MÉTODOS: Se presentan siete casos pediátricos de manejo endovascular del tracto de salida derecho; tres de ellos sometidos a valvuloplastia quirúrgica con persistencia de la estenosis pulmonar, por lo cual se decidió inserción percutánea de una válvula pulmonar (IVPP) transcatéter con válvula Melody utilizando la técnica valve-in-valve, con lo que se consiguió una resolución del 100% de la estenosis y no se presentó ningún tipo de complicación asociada al procedimiento. RESULTADOS: En cuatro pacientes se logró una implantación exitosa de la válvula por vía percutánea en diferentes cardiopatías congénitas, siendo uno de ellos en tracto nativo; además, destaca el caso de un paciente en quien se realizó fractura intencional de la válvula pulmonar, procedimiento innovador en el manejo endovascular pediátrico en Colombia. CONCLUSIONES: En estos pacientes el procedimiento resultó ser poco invasivo, seguro y efectivo. La técnica IVPP podría ser considerada una opción viable en Colombia (y en otros países en desarrollo) para el manejo de implantes valvulares primarios fallidos o incluso en tractos nativos.

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INTRODUCTION: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. METHODS: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. RESULTS: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. CONCLUSION: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

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ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

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Objective: To evaluate the effectiveness of the procedure and outcomes during follow-up. Methods: 80 patients with pulmonary valvular stenosis who underwent percutaneous balloon valvuloplasty between January 2014 and December 2019 are described. Demographic, echocardiographic, and hemodynamic characteristics of the procedure were evaluated. Follow-up included clinical, echocardiographic parameters, pulmonary regurgitation severity, and residual pulmonary gradient at each cutoff point. Results: The age range was 2 years (interquartile range: 10.5 months - 6 years), and the predominant sex was male with 56.2%. The transvalvular pulmonary gradient decreased from 61.7 mmHg +- 21.2 to 17 mmHg (interquartile range: 11-26 mmHg). The immediate success rate was 90%. Follow-up time showed a median of 21 months (interquartile range: 5-47.5 months). All patients at follow-up showed some degree of pulmonary insufficiency at each cutoff point; 17% of the cases at the end of their follow-up were found to have severe insufficiency. Three cases of long-term restenosis were found (3.8%), and 6 (7.5%) were admitted for valvuloplasty surgery or pulmonary valve replacement. The complications reported reached 10% of cases, two patients were admitted to surgery during the procedure for major complications. A significant association was found with severe pulmonary insufficiency at the end of follow-up and ring/balloon ratio. Conclusions: Percutaneous transluminal valvuloplasty with balloon is an effective technique in the treatment of pulmonary valvular stenosis, with reported complications but with good results during follow-up.

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Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

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Resumen Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.

Abstract Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

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Objetivo: Determinar los resultados del tratamiento percutáneo que se realizaron a pacientes con diagnostico de Cardiopatía Congénita (CC) en el Hospital Almanzor Aguinaga Asenjo (HNAAA) durante los años 2008 al 2015. Material y Métodos: Estudio cuantitativo de diseño descriptivo transversal, la población fue de 66 pacientes con diagnostico de CC sometidos a este tratamiento en el HNAA durante los años 2008 al 2015, los datos se obtuvieron del sistema de Gestión Hospitalaria y el registro medico solicitado en el área de hemodinámica del servicio de cardiología para la confección de la base de datos en Excel 2007. Resultados: Del registro de los 66 pacientes evaluados en este estudio, predomino el sexo femenino (74,2%). En el grupo etario menor de 10 años (59,1%) fue en los cuales se realizo con mayor frecuencia el tratamiento percutáneo. La cardiopatía congénita de mayor abordaje terapéutico fue el Conducto Arterioso Permeable (72,7%); seguidas de estenosis de la válvula pulmonar (EVP) (18,2%); Los resultados obtenidos por cardiopatía son: PCA (87,5%), EVP (100%), coartación aortica (CoA) (100%) y comunicación interauricular (CIA) (100%). Las complicaciones fueron: Embolizacion del dispositivo hacia la arteria pulmonar y hematoma inguinal pequeño. Conclusiones: El tratamiento percutáneo realizado en el hospital Almanzor Aguinaga Asenjo durante los años 2008 al 2015 presentó similitud a la literatura Internacional en cuanto a los resultados en pacientes con diagnostico de Cardiopatía Congénita, las complicaciones presentadas y el perfil clínico de los pacientes.

Objetive: To determine the results of the percutaneous treatment that were performed in patients with a diagnosis of Congenital Heart Disease (CHD) in the Almanzor Aguinaga Asenjo Hospital (HNAAA) during the years 2008 t o 2015. Mat er i al and Met hods: A quantitative study with a cross-sectional descriptive design, the population was 66 patients with CC diagnosis undergoing this treatment in the HNAA during the years 2008 to 2015, the data was obtained from the Hospital Management system and the medical record requested in the hemodynamic area of the cardiology service for the preparation of the database in Excel 2007. Results: From the registry of the 66 patients evaluated in this study, the female sex predominated (74.2%). In the age group younger than 10 years (59.1%), percutaneous treatment was the most frequent. The congenital cardiopathy with the greatest therapeutic approach was the Permeable Arterial Conduit (72.7%); followed by pulmonary valve stenosis (PVS) (18.2%); The results obtained by heart disease are: PCA (87.5%), PVS (100%), aortic coarctation (CoA) (100%) and atrial septal defect (ASD) (100%). The complications were: Embolization of the device towards the pulmonary artery and small inguinal hematoma. Conclusions: The percutaneous treatment performed in the Almanzor Aguinaga Asenjo hospital during the years 2008 to 2015 presented similarity to the International literature in terms of the results in patients with a diagnosis of congenital heart disease, the complications presented and the clinical profile of the patients.

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Abstract Carcinoid heart disease is a rare but important cause of intrinsic right heart valve disorders leading to right heart failure. Occasionally, left-sided heart valves may also be involved. The characteristic cardiac pathological findings of carcinoid heart disease are endocardial thickening as a result of fibrous deposits on the endocardium. Echocardiographic examination and right heart catheterization are very useful for the diagnosis of the lesion. If more cardiac valves are affected, multiple valve replacement should be considered. The management of the pulmonary valve lesion depends on the extent of the diseased valve, either by valvulotomy, valvectomy, or valve replacement. Percutaneous valve implantations in the pulmonary and in the inferior vena cava positions have been advocated for high-risk patients.

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Introdução: Stress is associated with cardiovascular diseases.O implante percutâneo da válvula pulmonar é uma alternativa para condutos com disfunção. Descrevemos aqui a primeira experiência com o implante da válvula Melody® no Brasil. Métodos: Foram selecionados pacientes com estenose ou insuficiência pulmonar significativa em condutos de 16 a 22 mm. Foram empregadas técnicas padronizadas. Factibilidade, segurança e eficácia desse procedimento foram avaliadas. Resultados: Desde dezembro de 2013, dez pacientes (média de idade e peso de 16,5 anos e 49 kg, respectivamente) foram submetidos ao procedimento com intervalo médio de 11,9 ± 8,6 anos desde a última cirurgia. Insuficiência pulmonar foi indicação para o tratamento em três pacientes, estenose em dois e lesão mista em cinco. A válvula Melody® foi implantada com sucesso em todos os casos. A média da pressão sistólica do ventrículo direito e a relação ventrículo direito/ventrículo esquerdo diminuíram de 49,2 ± 15,9 para 35,8 ± 5,7 mmHg e de 0,55 ± 0,18 para 0,39 ± 0,08 mmHg (p < 0,01 para ambos). Não observamos estenose e nem insuficiência pulmonar residual significativa. Um paciente teve extravasamento contido requerendo um stent coberto e um segundo implante valvular. Todos os pacientes receberam alta do hospital em 72 horas. As válvulas funcionaram adequadamente ...

Background: Transcatheter pulmonary valve implantation is an alternative for dysfunctional conduits. We report the first experience with the MelodyTM valve implantation in Brazil. Methods: Patients with significant pulmonary stenosis or significant pulmonary insufficiency in conduits measuring 16 to 22 mm were enrolled. Standardized techniques were employed. The feasibility, safety and efficacy of this procedure were assessed. Results: From December 2013, ten patients (mean age and weight of 16.5 years and 49 kg, respectively) have undergone the procedure with a mean interval of 11.9 ± 8.6 years since the last surgery. Pulmonary insufficiency was an indication for treatment in three patients, pulmonary stenosis in two, and mixed lesion in five. The MelodyTM valve was successfully implanted in all cases. Mean right ventricular systolic pressure and right ventricle/left ventricle ratio decreased from 49.2 ± 15.9 to 35.8 ± 5.7 mmHg and from 0.55 ± 0.18 to 0.39 ± 0.08 mmHg (p < 0.01 for both). Significant residual pulmonary stenosis or pulmonary insufficiency was not observed. One patient had a contained conduit tear requiring a covered stent and a second valve implantation. All patients were discharged within 72 hours. The valves were properly functioning in a mean follow-up of 4.1 ± 2.2 months with no complications. Conclusions: Transcatheter MelodyTM valve implantation was feasible, safe and effective ...

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BACKGROUND: Congenital heart disease with decreased pulmonary flow survival has increased due to systemic pulmonary fistulae (SPF); nevertheless, complications may occur. The purpose of this study was to identify mortality-related surgical and biochemical factors in pediatric patients with modified Blalock-Taussig shunt. METHODS: A cohort was formed with congenital heart disease newborns who survived Blalock-Taussig-type palliative systemic pulmonary fistulae surgery. At admission to the intensive care unit, surgical time, diameter polytetrafluoroethylene vascular graft, arterial blood gases and central venous saturation were recorded. The patients were followed until discharge or death. For statistical analysis, central tendency and dispersion measures, chi-square tests, odds-ratios, Cox regression model and hazard ratios (HR) were used, and for the most independent variable, Kaplan-Meier curves. RESULTS: Out of 33 newborns who underwent the surgical procedure, 9 died (23%). The most common heart diseases were pulmonary atresia with intact ventricular septum (8 patients, 25%) and hypoplastic right ventricle (8, 25%). The most independent mortality-associated variable was hyperlactatemia > 3 mmol/L (HR = 8 [1.9 - 32], p < 0.05). CONCLUSIONS: Lactate > 3 mmol/L was an independent predictor of mortality in patients who underwent Blalock-Taussig shunt.

INTRODUCCIÓN: la supervivencia de los pacientes con cardiopatía congénita con flujo pulmonar disminuido ha aumentado debido a las técnicas de anastomosis quirúrgica entre la circulación sistémica y la pulmonar, sin embargo, pueden presentarse complicaciones. El objetivo de este estudio fue determinar los factores quirúrgicos y bioquímicos relacionados con la muerte en pacientes pediátricos en quienes se realizó derivación de Blalock-Taussig modificada. MÉTODOS: se integró una cohorte de neonatos con cardiopatía congénita sometidos a derivación tipo Blalock-Taussig que sobrevivieron a la cirugía. Al ingreso a cuidados intensivos se documentó tiempo quirúrgico, diámetro del injerto vascular de politetrafluoroetileno, gasometría arterial y saturación venosa central de oxígeno. Se dio seguimiento hasta el egreso o defunción. Para el análisis estadístico se utilizaron medidas de tendencia central y dispersión, chi cuadrada, razón de momios, modelo de regresión de Cox, hazard ratio (HR) y curva de Kaplan-Meier, para la variable más independiente. RESULTADOS: de 33 neonatos sometidos a procedimiento quirúrgico, nueve murieron (23 %). Las cardiopatías más frecuentes fueron la atresia pulmonar con septo íntegro (ocho, 25 %) y ventrículo derecho hipoplásico (ocho, 25 %). La variable más independiente relacionada con mortalidad lo constituyó el nivel del lactato sérico > 3 mmol/L (HR = 8 [1.9-32], p < 0.05). CONCLUSIONES: el nivel del lactato sérico > 3 mmol/L fue un predictor independiente de mortalidad en los pacientes con derivación tipo Blalock-Taussig.

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La estenosis valvular pulmonar aislada es poco frecuente. Se diagnostica y se trata casi siempre durante la infancia, razón por la cual es poco común encontrarla en la edad adulta. En esta presentación se describe el caso de una paciente de 40 años con disnea ante pequeños esfuerzos, cianosis central y uñas en vidrio de reloj, que presentaba una estenosis valvular pulmonar crítica con cortocircuito de derecha a izquierda a través del foramen oval. El gradiente transvalvular pulmonar era de 150 mm Hg. Se realizó una valvulotomía pulmonar percutánea con un catéter balón Nucleus de Numed Cardiac Diagnostics. El gradiente medido por cateterismo y ecocardiograma inmediatamente después del procedimiento fue de 23 mm Hg. Seis meses después había desaparecido la cianosis y el gradiente transvalvular pulmonar fue de 19 mm Hg. La paciente lleva una vida normal, sin limitaciones en su calidad y estilo de vida.

Isolated pulmonary valve stenosis is uncommon. As the diagnosis and treatment of this condition is made during childhood, it is a rare finding in adult patients. We describe the case of a 40-year old female patient with dyspnea during slight exertion, central cyanosis and nail clubbing with a critical pulmonary valve stenosis and right-to-left shunt through a patent foramen ovale. The pulmonary valve gradient was 150 mm Hg. Percutaneous pulmonary valvuloplasty was performed using a Nucleus balloon catheter (Numed Cardiac Diagnostics). Immediately after the procedure, the pulmonary gradient measured by cardiac catheterization and echocardiography decreased to 23 mm Hg. Six months later the cyanosis had disappeared and the gradient was 19 mm Hg. The patient lives a normal life with no limitations in her quality of life and lifestyle.

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La estenosis valvular pulmonar aislada es poco frecuente. Se diagnostica y se trata casi siempre durante la infancia, razón por la cual es poco común encontrarla en la edad adulta. En esta presentación se describe el caso de una paciente de 40 años con disnea ante pequeños esfuerzos, cianosis central y uñas en vidrio de reloj, que presentaba una estenosis valvular pulmonar crítica con cortocircuito de derecha a izquierda a través del foramen oval. El gradiente transvalvular pulmonar era de 150 mm Hg. Se realizó una valvulotomía pulmonar percutánea con un catéter balón Nucleus de Numed Cardiac Diagnostics. El gradiente medido por cateterismo y ecocardiograma inmediatamente después del procedimiento fue de 23 mm Hg. Seis meses después había desaparecido la cianosis y el gradiente transvalvular pulmonar fue de 19 mm Hg. La paciente lleva una vida normal, sin limitaciones en su calidad y estilo de vida.(AU)

Isolated pulmonary valve stenosis is uncommon. As the diagnosis and treatment of this condition is made during childhood, it is a rare finding in adult patients. We describe the case of a 40-year old female patient with dyspnea during slight exertion, central cyanosis and nail clubbing with a critical pulmonary valve stenosis and right-to-left shunt through a patent foramen ovale. The pulmonary valve gradient was 150 mm Hg. Percutaneous pulmonary valvuloplasty was performed using a Nucleus balloon catheter (Numed Cardiac Diagnostics). Immediately after the procedure, the pulmonary gradient measured by cardiac catheterization and echocardiography decreased to 23 mm Hg. Six months later the cyanosis had disappeared and the gradient was 19 mm Hg. The patient lives a normal life with no limitations in her quality of life and lifestyle.(AU)

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La estenosis valvular pulmonar aislada es poco frecuente. Se diagnostica y se trata casi siempre durante la infancia, razón por la cual es poco común encontrarla en la edad adulta. En esta presentación se describe el caso de una paciente de 40 años con disnea ante pequeños esfuerzos, cianosis central y uñas en vidrio de reloj, que presentaba una estenosis valvular pulmonar crítica con cortocircuito de derecha a izquierda a través del foramen oval. El gradiente transvalvular pulmonar era de 150 mm Hg. Se realizó una valvulotomía pulmonar percutánea con un catéter balón Nucleus de Numed Cardiac Diagnostics. El gradiente medido por cateterismo y ecocardiograma inmediatamente después del procedimiento fue de 23 mm Hg. Seis meses después había desaparecido la cianosis y el gradiente transvalvular pulmonar fue de 19 mm Hg. La paciente lleva una vida normal, sin limitaciones en su calidad y estilo de vida.(AU)

Isolated pulmonary valve stenosis is uncommon. As the diagnosis and treatment of this condition is made during childhood, it is a rare finding in adult patients. We describe the case of a 40-year old female patient with dyspnea during slight exertion, central cyanosis and nail clubbing with a critical pulmonary valve stenosis and right-to-left shunt through a patent foramen ovale. The pulmonary valve gradient was 150 mm Hg. Percutaneous pulmonary valvuloplasty was performed using a Nucleus balloon catheter (Numed Cardiac Diagnostics). Immediately after the procedure, the pulmonary gradient measured by cardiac catheterization and echocardiography decreased to 23 mm Hg. Six months later the cyanosis had disappeared and the gradient was 19 mm Hg. The patient lives a normal life with no limitations in her quality of life and lifestyle.(AU)

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INTRODUÇÃO: Nos pacientes portadores de cardiopatias congênitas que foram submetidos à valvotomia pulmonar ou cirurgias de ampliação com o uso do anel da válvula pulmonar, a insuficiência ou estenose pulmonar residual pode acarretar falência cardíaca direita com deterioração clínica. Essas crianças necessitam de múltiplas intervenções ao longo de suas vidas, que impõem uma elevada taxa de morbidade e mortalidade. OBJETIVO: Desenvolvimento de uma técnica menos invasiva para implante de uma prótese valvada através do ventrículo direito. MÉTODOS: A prótese valvada consiste em um anel metálico construído com nitinol autoexpansível, revestida de poliéster, onde três cúspides de pericárdio bovino foram montadas. Doze porcos foram utilizados para a realização dos implantes. Foi realizado controle ecocardiográfico imediatamente após o implante e com uma, quatro, oito e 12 semanas. RESULTADOS: Um animal apresentou refluxo de grau moderado a importante e outros três refluxo de grau leve. Os gradientes transvalvares medidos antes do implante variavam entre 3 a 6 mmHg, sendo que, logo após o implante, houve aumento dos gradientes que variaram de 7 a 45 mmHg. Houve queda desses gradientes, sendo que em apenas quatro dos 12 animais os gradientes estavam acima de 20 mmHg. Em seis animais, ocorreu a formação de trombo na prótese, sendo esta a complicação mais frequente. CONCLUSÃO: Esses achados evidenciam a necessidade da realização de estudos com a utilização de anticoagulantes e ou antiagregantes plaquetários na tentativa de diminuir esse evento. O estudo pretende apresentar subsídios para o início do emprego de próteses valvares que poderiam ser implantadas através de técnicas menos invasivas.

INTRODUCTION: Patients with congenital heart disease who underwent pulmonary valvotomy or surgery to open the pulmonary valve ring are prone to develop residual pulmonary insufficiency or stenosis that may lead to right heart failure with clinical deterioration. These children require multiple interventions throughout their lives, which impose a high rate of morbidity and mortality. OBJECTIVE: To develop a less invasive technique for implantation of a valved prosthesis through the right ventricle. METHODS: The valved prosthesis consists of an auto expanding metal stent built with nitinol, surrounded with polyester, where the three leaflets of bovine pericardium were mounted. Twelve pigs were used to perform the implants. Echocardiographic control was performed immediately after implantation and one, four, eight and 12 weeks. RESULTS: One animal showed reflux of moderate to severe and three mild reflux. Transvalvular gradients measured before implantation ranged from 3 to 6 mmHg and that soon after the implant was increased, ranging from 7 to 45 mmHg. There was a decrease in these gradients during follow up and in only four of the twelve animals the gradients were above 20 mmHg. Thrombus formation occurred in the prosthesis of six animals, and this was the most frequent complication. CONCLUSION: These findings highlight the need for studies with the use of anticoagulants and antiplatelet, an attempt to reduce this event. The study aims to contribute for the start of the use of prosthetic heart valves that could be implanted through minimally invasive techniques without the use of cardiopulmonary bypass.

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