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1.
Artículo en Inglés | MEDLINE | ID: mdl-38634941

RESUMEN

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

2.
Arch Cardiol Mex ; 94(1): 7-14, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38507314

RESUMEN

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.


Asunto(s)
Cardiopatías Congénitas , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Estenosis de la Válvula Pulmonar , Válvula Pulmonar , Humanos , Niño , Implantación de Prótesis de Válvulas Cardíacas/métodos , Colombia , Resultado del Tratamiento , Cateterismo Cardíaco/métodos , Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/cirugía , Cardiopatías Congénitas/cirugía , Diseño de Prótesis
3.
Arch Cardiol Mex ; 2024 Mar 11.
Artículo en Inglés | MEDLINE | ID: mdl-38467114

RESUMEN

Background: The decision to close patent ductus arteriosus should always be individualized and taken together with the child's family once the risks and benefits of both choices have been exposed. Objective: This study aims to report the experience and outcomes in patients undergoing endovascular closure of small to medium-size PDA with a Nit-Occlud® device in a tertiary referral hospital in Colombia. Methods: Longitudinal descriptive study, which included all patients under 18 years of age who underwent percutaneous ductal closure with Nit-Occlud® device between January 1, 2011, and February 1, 2023. Patients with associated complex congenital heart disease requiring surgical management, pregnant patients, and patients with incomplete data regarding studied variables were excluded from the study. Results: Eighty-seven patients were documented, with a mean age, weight, and height at closure of 51 months, 14 kg, and 95.83 cm, respectively. About 70% of the patients (n = 61) were female, 76% were under 6-years-old and only one patient was over 15. The average size of the ductus at the pulmonary end was 2 mm. Four of the total number of patients did not achieve PDA closure during the procedure. Of the remaining 83, complete immediate closure was achieved in 81 patients. A device exchange for a larger device was required during the same procedure in one of the cases. Two patients presented residual shunt of 0.5 mm during follow-up, and one required a new procedure for device closure 10 months later. Only one device presented repeatedly embolization to the aorta, requiring surgical removal. As a technical difficulty, one device presented repeated passage into the aorta, so it was decided to remove it before releasing it to avoid complications, and given the complex anatomy of the ductus, surgical closure was indicated. Among the complications, one patient presented a hematoma of the subcutaneous tissue in the right thigh, which improved with medical management, and no deaths related to the procedure were registered. Conclusions: Using the Nit-Occlud® device to close small to moderate-sized ductus remains a safe and effective strategy with successful closure rates at 1-year follow-up irrespective of age, weight, height, or whether it involves a small or medium-sized duct. Despite our limitations, results concerning adverse effects are comparable to those observed in multicentric studies conducted in other regions.


Antecdentes: La decisión de cerrar el conducto arterioso permeable (CAP) siempre debe ser individualizada y tomada en conjunto con la familia del niño una vez expuestos los riesgos y beneficios de ambas opciones. Objetivo: Este estudio tiene como objetivo informar la experiencia y los resultados en pacientes sometidos a cierre endovascular del CAP de tamaño pequeño a mediano con un dispositivo Nit-Occlud® en un hospital de tercer nivel de referencia en Colombia. Método: Estudio descriptivo longitudinal, que incluyó a todos los pacientes menores de 18 años a quienes se les realizó cierre ductal percutáneo con dispositivo Nit-Occlud® entre el 1 de enero de 2011 y el 1 de febrero de 2023. Se excluyeron: pacientes con cardiopatía congénita compleja asociada que requirieron manejo quirúrgico, pacientes embarazadas y pacientes con datos incompletos sobre las variables estudiadas. Resultados: Se documentaron 87 pacientes, con edad, peso y talla promedio al cierre de 51 meses, 14 kg y 95.83 cm, respectivamente. El 70% de los pacientes (n = 61) eran mujeres, el 76% tenían menos de seis años y solo un paciente tenía más de 15 años. El tamaño medio del conducto en el extremo pulmonar fue de 2 mm. Cuatro del total de pacientes no lograron el cierre del CAP durante el procedimiento. De los 83 restantes, se logró el cierre inmediato completo en 81 pacientes. En uno de los casos fue necesario cambiar el dispositivo por uno más grande durante el mismo procedimiento. Dos pacientes presentaron shunt residual de 0.5 mm durante el seguimiento y uno requirió un nuevo procedimiento para cierre del dispositivo diez meses después. Solo un dispositivo presentó embolización repetida en la aorta, requiriendo extracción quirúrgica. Como dificultad técnica, un dispositivo presentó paso repetido hacia la aorta, por lo que se decidió retirarlo antes de liberarlo para evitar complicaciones y dada la compleja anatomía del ductus se indicó cierre quirúrgico. Entre las complicaciones, un paciente presentó un hematoma del tejido subcutáneo en el muslo derecho, que mejoró con el manejo médico, y no se registraron muertes relacionadas con el procedimiento. Conclusiones: El uso del dispositivo Nit-Occlud® para cerrar conductos de tamaño pequeño a moderado sigue siendo una estrategia segura y eficaz con tasas de cierre exitoso al año de seguimiento, independientemente de la edad, el peso, la altura o si se trata de un conducto de tamaño pequeño o mediano. A pesar de nuestras limitaciones, los resultados sobre los efectos adversos son comparables a los observados en estudios multicéntricos realizados en otras regiones.

4.
Cardiol Young ; 33(8): 1378-1382, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-36205146

RESUMEN

INTRODUCTION: Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia. MATERIALS AND METHODS: A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro-Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described. RESULTS: Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3 years (interquartile range 4.3-6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12 mmHg (interquartile range 10-15), the Nakata index 272 mm2/m2 (interquartile range 204-327), and the McGoon index (interquartile range 1.86-2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19 days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%. CONCLUSION: The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Atresia Tricúspide , Corazón Univentricular , Niño , Preescolar , Femenino , Humanos , Masculino , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos , Centros de Atención Terciaria , Resultado del Tratamiento , Atresia Tricúspide/cirugía
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