Coração em Criss-Cross: um relato de caso / Criss-Cross Heart: a case report

Coração em criss-cross (ou coração entrecruzado) foi descrito pela primeira vez em 1974. Trata-se de uma malformação cardíaca congênita, rara, ocorrendo 8 casos a cada 1.000.000 de crianças, e representando somente 0,1% das malformações congênitas. Os métodos diagnósticos de escolha são o ecocardiograma transtorácico, a ressonância magnética cardíaca (RMC), a angiotomografia (TC) e, eventualmente, o cateterismo cardíaco. Neste relato, descreve-se o caso de um recém-nascido com coração em criss-cross somado à dupla via de saída do ventrículo direito (VD), com vasos mal posicionados, além de comunicação interatrial (CIA), comunicação interventricular (CIV), displasia de valva tricúspide e veia cava superior esquerda persistente. Não se sabe a etiologia exata dessa malformação, mas parece ocorrer pela rotação dos ventrículos em seu eixo longitudinal, não acompanhada das rotações atrial e das valvas atrioventriculares (AV). Esse movimento produz uma alteração das vias de entrada dos ventrículos, determinando que o VD se posicione em plano superior e o esquerdo em plano inferior. Apesar de ainda não se saber a exata causa dessa anomalia, acredita-se que uma alteração genética possa estar levando a esses casos: a mutação do gene Cx43. O diagnóstico do caso em questão foi dado pela ecocardiografia transtorácica e da TC de aorta e artérias pulmonares, que mostraram, além do criss-cross, outras alterações, como dupla via de saída do VD, CIA e CIV amplas.(AU)

Criss-cross heart was first described in 1974. It is a rare congenital heart malformation that occurs in 8 cases per 1,000,000 children, and represents only 0.1% of congenital malformations. The diagnostic methods of choice are transthoracic echocardiography, cardiac magnetic resonance (CMR), computed tomography angiography (CT) and, sometimes, cardiac catheterization. This report describes the case of a newborn with a criss-cross heart in addition to double-outlet right ventricle (RV), with poorly positioned vessels, in addition to atrial septal defect (ASD), interventricular septal defect, tricuspid valve dysplasia and persistent left superior vena cava. The exact etiology of this malformation is not known, but it seems to occur due to rotation of the ventricles in their longitudinal axis, not accompanied by rotation of the atrial and atrioventricular (AV) valves. This movement produces abnormal ventricular inlets, determining that the RV be positioned on a superior plane and the left ventricle on an inferior plane. Although the exact cause of this anomaly is still unknown, it is believed that a genetic abnormality may be leading to these cases: mutation of the Cx43 gene. Diagnosis of the case concerned was given by transthoracic echocardiography and computed CT of the aorta and pulmonary arteries, which showed, in addition to the criss-cross heart, other abnormalities, such as double-outlet RV, large ASD and ventricular septal defect (VSD).(AU)

Results of percutaneous closure with device in congenital heart disease of the atrial septal defect type / Resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular

Congenital Interatrial Septal Defects (TEA) are among the most common Congenital Heart Diseases in the population, where Atrial Septal Defect stands out, which is produced by a left-right short circuit that provides greater survival and less morbidity and mortality. The objective was to determine the results of Percutaneous Closure with a Device in Congenital Heart Disease of the Atrial Communication type at the Carlos Andrade Marín Specialty Hospital (Quito) in the Hemodynamics service from January 2009 to July 2019. It is a retrospective descriptive cross-sectional study . With a population of 122 cases from the AS400 registry. Thus, it was determined: there is a female predominance in a ratio of 2:1, the ranges for defect intervention were 5.5 mm to 35 mm, post-procedure pulmonary artery pressures (systolic 18-25 mmHg and diastolic 6-10 mmHg) in 95% of patients, ventricular overload in 76% in pre-procedure patients. Concluding that 96% of our population has a significant post-procedure improvement.

Los defectos del tabique interauricular congénitos (TEA) se encuentran entre las cardiopatías congénitas más comunes en la población donde resalta la comunicación interauricular que es producido por un cortocircuito izquierda derecha que brinda mayor sobrevida y menor morbimortalidad. El objetivo fue determinar los resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular en el hospital de especialidades Carlos Andrade Marín (Quito) en el servicio de hemodinamia en el período enero 2009 a julio 2019. Es un estudio transversal descriptivo retrospectivo. Con una población 122 casos del registro de AS400. Encontramos un predominio en sexo femenino en una proporción de 2:1, los rangos para intervención del defecto fueron de 5,5 mm a 35 mm, las presiones de la arteria pulmonar pos-procedimiento (sistólica de 18-25 mmHg y diastólica de 6-10 mmHg) en un 95% de los pacientes, sobrecarga ventricular en un 76% en los pacientes pre-procedimiento. Concluyendo que el 96% de nuestra población tiene una mejoría significativa post-procedimiento.

Triatriatum sinistrum em adulto assintomático: relato de caso / Cor triatriatum sinistrum in an asymptomatic adult: a case report

Cor triatriatum é um anomalia cardíaca congênita rara frequentemente diagnosticada na primeira infância. Este estudo de caso apresenta um adulto com um achado acidental de cor triatriatum sinistrum. Com base na apresentação clínica, o paciente foi tratado de forma conservadora. São apresentados achados de imagens ecocardiográficas de cor triatriatum sinistrum deste paciente juntamente de revisão narrativa da literatura sobre essa doença.(AU)

Cor triatriatum is a rare congenital heart anomaly often diagnosed in early childhood. This case study features an adult with an incidental finding of cor triatriatum sinistrum. Based on the clinical presentation, the patient was treated conservatively. Cor triatriatum sinistrum echocardiographic image findings of this patient are presented along with a narrative review of the literature about this disease. (AU)

Complicaciones a corto plazo del cierre percutáneo de comunicación interauricular / Short-term complications of percutaneous atrial septal defect closure

Resumen Introducción: El cierre percutáneo de la comunicación interauricular ostium secundum ha pasado a ser una alternativa a la cirugía convencional. En ese caso, los dispositivos de autocentrado son los más usados entre los cardiólogos intervencionistas. El tipo y la tasa de complicaciones difieren para los distintos dispositivos. Objetivo: Reportar las complicaciones a corto plazo del cierre percutáneo de la comunicación interauricular ostium secundum. Método: Entre abril de 2001 y diciembre de 2017, 129 pacientes (media de edad: 26 años; desviación estándar: 20,39 años) fueron sometidos a cierre percutáneo de comunicación interauricular ostium secundum. Las complicaciones fueron identificadas y clasificadas como mayores y menores. Resultados: 14 pacientes experimentaron complicaciones menores durante la hospitalización y en el primer año de seguimiento (10.85 %), y uno tuvo una complicación mayor que requirió cirugía electiva 4 meses después para la remoción del dispositivo mal posicionado. Entre los que presentaron complicaciones menores, dos tuvieron shunt residual leve, cinco presentaron arritmias interoperatorias, uno refirió dolor torácico y seis tuvieron migraña posimplante. Conclusiones: Esta serie de pacientes muestra que el cierre percutáneo de la comunicación interauricular ostium secundum es un procedimiento seguro y efectivo en casos bien seleccionados. Con el fin de reducir la tasa de complicaciones, están indicados el diagnóstico y el tratamiento tempranos, así como la observación de los criterios de selección de acuerdo con la morfología de la comunicación interauricular ostium secundum. La profilaxis antiagregante con clopidogrel para todos los pacientes requiere mayor investigación.

Abstract Introduction: Percutaneous closure of ostium secundum atrial septal defects has become an alternative to conventional surgery. Self-centering devices are the ones most used by interventional cardiologists in these cases. The type and rate of complications varies according to the various devices. Objective: To report the short-term complications of percutaneous closure of ostium secundum atrial septal defects. Method: Between April 2001 and December 2017, 129 patients (mean age: 26; standard deviation: 20.39 years) underwent percutaneous closure of ostium secundum atrial septal defects. Complications were identified and classified as major or minor. Two types of devices were used: the Amplatzer™ Septal Occluder in 98 patients, and the Figulla® Septal Occluder in 31 patients. Results: 14 patients had minor complications during hospitalization and the first year of follow up (10.85%), and one had a major complication which required elective surgery four months later to remove the misaligned device. Of those who experienced minor complications, two had mild residual shunting, five had intraoperative arrhythmias, one reported chest pain, and six had post-implant migraines. Conclusions: This series of patients shows that percutaneous closure of ostium secundum atrial septal defects is a safe and effective procedure in carefully selected cases. Early diagnosis and treatment are needed to decrease the rate of complications, along with adherence to the selection criteria according to the morphology of the ostium secundum atrial septal defect. Antiplatelet prophylaxis with clopidogrel for all patients requires further study.

Atrial Septal Aneurysm and Atrial Septal Defect Association - an Uncommon But Well-Recognized Association

Abstract Atrial septal aneurysm (ASA) is an uncommon but well-recognized cardiac abnormality. This educational text reviews the case of a 54-year-old female presenting an ASA related to a small ostium secundum atrial septal defect. The considerable signs and symptoms, interestingly, have not been justified by the clinical and hemodynamic investigations. So, we opted for a better imaging investigation with cardiac catheterization and transesophageal echocardiography. The surgical process was earlier indicated and performed with aid of cardiopulmonary bypass.

Caracterização clínico-epidemiológica de crianças e adolescentes portadores de cardiopatia congênita / Clinical-epidemiological characterization of children and adolescents with congenital heart disease / Caracterización clínico-epidemiológica de niños y adolescentes con cardiopatía congénita

Objetivo: Analisar características clínico-epidemiológicas de crianças portadoras de cardiopatia congênita residentes do município de Rio das Ostras referenciadas para unidades especializadas no estado do Rio de Janeiro. Método: Pesquisa descritiva, transversal realizada em duas unidades hospitalares no município do Rio de Janeiro, através da técnica documental retrospectiva por meio de dados nos prontuários. A coleta ocorreu entre setembro de 2018 a fevereiro de 2019. Resultados: Foram analisados 48 prontuários, com maior proporção entre os escolares (33,3%); do sexo masculino (58,3%); Em relação as cardiopatias, verificou-se com maior proporção as acianóticas (62,5%); destacando a comunicação interatrial com (14,6%); seguida da comunicação interventricular (12,5%) e coarctação da aorta (12,5%). Conclusão: A identificação dessas crianças no município de Rio das Ostras e a distribuição destas pelo território nacional são informações imprescindíveis para o planejamento e implementação de programas e políticas públicas que atendam as reais demandas deste segmento populacional

Objetivo: Analizar las características clínicas y epidemiológicas de los niños con cardiopatía congénita que viven en la ciudad de Rio das Ostras remitidos a unidades especializadas en el estado de Río de Janeiro. Método: Investigación descriptiva, transversal realizada en dos hospitales de la ciudad de Río de Janeiro, a través de una técnica documental retrospectiva a través de datos en registros médicos. La recolección se realizó entre septiembre de 2018 y febrero de 2019. Resultados: Se analizaron 48 registros médicos, con una mayor proporción entre los estudiantes (33.3%); hombre (58,3%); Con respecto a la enfermedad cardíaca, hubo una mayor proporción de acianóticos (62.5%); destacando la comunicación interauricular con (14,6%); seguido de comunicación interventricular (12.5%) y coartación aórtica (12.5%). Conclusión: La identificación de estos niños en la ciudad de Rio das Ostras y su distribución en todo el territorio nacional son información esencial para la planificación e implementación de programas y políticas públicas que satisfagan las demandas reales de este segmento de la población

Objective: To analyze the clinical and epidemiological characteristics of children with congenital heart disease living in the city of Rio das Ostras referred to specialized units in the state of Rio de Janeiro. Method: Descriptive, cross-sectional research conducted in two hospitals in the city of Rio de Janeiro, through retrospective documentary technique through data in medical records. The collection took place between September 2018 and February 2019. Results: 48 medical records were analyzed, with a higher proportion among students (33.3%); male (58.3%); Regarding heart disease, there was a greater proportion of acyanotic (62.5%); highlighting interatrial communication with (14.6%); followed by interventricular communication (12.5%) and aortic coarctation (12.5%). Conclusion: The identification of these children in the city of Rio das Ostras and their distribution throughout the national territory are essential information for the planning and implementation of programs and public policies that meet the real demands of this population segment

Cor triatriatum dexter y comunicación interauricular tipo seno venoso: Una asociación infrecuente / Cor triatriatum dexter and sinus venosus atrial septal defect: An infrequent association

RESUMEN El cor triatriatum es una malformación congénita poco frecuente, con una prevalecia de un 0,1% entre todas las anomalías congénitas cardíacas, donde el corazón queda dividido en tres atrios o aurículas. En el cor triatriatum dexter la aurícula derecha queda dividida, por una membrana fibromuscular, en dos partes, una proximal y otra distal, que se comunican o no entre sí. En la evolución natural de la enfemedad, los pacientes pueden permanecer asintomáticos hasta su diagnóstico o presentar manifectaciones cardiovasculares secundarias a insuficiencia cardíaca derecha y trastornos del ritmo. En el caso particular de aquellos que se encontraran sintomáticos, el tratamiento de elección sería la corrección quirúrgica del defecto y, en ocasiones, la rotura percutánea de la membrana; mientras que en los que se encuentran asintomáticos, el seguimiento y tratamiento oportuno de sus complicaciones parecería la alternativa más viable.

ABSTRACT Cor triatriatum is a rare congenital condition with a 0.1% prevalence among all cardiac congenital anomalies in which the heart is partitioned into three atria. In cor triatriatum dexter the right atrium is divided by a fibromuscular membrane into two parts, a proximal and a distal one, which may or may not communicate with each other. In the natural course of the condition, patients may remain asymptomatic until diagnosis or present with cardiovascular manifestations secondary to right heart failure and rhythm disorders. In the specific case of symptomatic patients, the treatment of choice would be surgical correction of the anomaly and sometimes percutaneous rupture of the membrane; while in asymptomatic patients, timely follow-up and treatment of their complications would seem to be the best alternatives.

Comparison of postoperative changes in inflammatory marker levels between transthoracic and transcatheter device closures of atrial septal defects in children

Abstract Objective: To explore the postoperative changes in inflammatory markers in children who underwent device closure of an atrial septal defect (ASD) via a transthoracic or transcatheter approach. Methods: The experimental and clinical data were retrospectively collected and analyzed for a total of 53 pediatric patients between September 2018 and December 2018. According to the different treatments, 19 patients who underwent transthoracic device closure were assigned to group A, and the remaining 34 patients who underwent a transcatheter approach were assigned to group B. Results: All patients were successfully occluded without any device-related severe complication. Compared with the preoperative levels, the postoperative levels of most inflammatory cytokines in both groups were significantly increased and reached a peak on the first day after the procedure. The level of postoperative inflammatory cytokines was significantly lower in group B than in group A. In addition, there was no significant difference in procalcitonin before and after the transcatheter approach. Conclusion: Systemic inflammatory reactions occurred after transthoracic or transcatheter device closure of ASDs in pediatric patients. However, these inflammatory reactions were more significant in patients who underwent a transthoracic approach than in patients who underwent a transcatheter approach.

Comparative study between surgical repair of atrial septal defect via median sternotomy, right submammary thoracotomy, and right vertical infra-axillary thoracotomy

Abstract Objective: To compare the results of surgical repair via median sternotomy, right submammary thoracotomy, and right vertical infra-axillary thoracotomy for atrial septal defect (ASD). Methods: This is a retrospective analysis of the relative perioperative and postoperative data of 136 patients who underwent surgical repair for ASD with the abovementioned three different treatments in our hospital from June 2014 to December 2017. Results: The results of the surgeries were all satisfactory in the three groups. No statistically significant difference was found in operative time, duration of cardiopulmonary bypass, blood transfusion amount, postoperative mechanical ventilation time, duration of intensive care unit, length of hospital stay, and hospital costs. However, the median sternotomy group had the longest incision. Meanwhile, there was no significant difference in postoperative complications. Conclusion: All three types of surgical incisions can be safely and effectively used to repair ASD. The treatments via right submammary thoracotomy and right vertical infra-axillary thoracotomy have advantages over the treatment via median sternotomy in cosmetic results and should be the recommended options.

Caso clínico de cor triatriatum en el adulto asintomático: diagnóstico ecocardiográfico / Clinical case of cor triatriatum in the asymptomatic adult: cardiac ultrasound diagnosis

Resumen El cor triatriatum es una anomalía congénita rara, que representa el 0,4% de todas las malformaciones cardíacas. En esta malformación la aurícula es separada en dos compartimientos por una membrana fibromuscular, que puede ser enteramente intacta o contener fenestraciones de diámetros variables. La aparición de la sintomatología está en relación con las propiedades obstructivas que mimetizan la estenosis mitral y la presencia de una comunicación interauricular. La ecocardiografía bidimensional y el Doppler color son las modalidades diagnósticas no invasivas de elección para el diagnóstico preciso del cor triatriatum y de su repercusión hemodinámica. Se reporta un caso de cor triatriatum en un paciente adulto mayor, en quien se hizo el diagnóstico tardío y casual de esta malformación.

Abstract Cor triatriatum is a rare congenital anomaly that represents 0.4% of all cardiac malformations. In this malformation the atrium is separated into two compartments by a fibrous-muscular membrane that may be completely intact or contain fenestrations with variable diameters. The appearance of symptoms is associated with obstruction-like properties that mimic mitral stenosis and an atrial septal defect. Two-dimensional cardiac ultrasound and colour Doppler are non-invasive methods of choice for the accurate diagnosis of cor triatriatum and its haemodynamic repercussions. A case of cor triatriatum is reported in an elderly patient, in whom a delayed and casual diagnosis was made of this malformation.

Rare association between atrioventricular septal defect and partial anomalous pulmonary venous connection

Abstract Clinical data: Infant, 7 months, female, referred to our department at one month of age, suspecting of congenital heart disease for further investigation. Chest radiography: Demonstrates cardiomegaly and prominent pulmonary vascular markings. Electrocardiography: Shows right ventricular hypertrophy and left anterior fascicular block. Echocardiography: Evidenced common atrioventricular valve with two orifices and the left superior pulmonary vein draining on the brachiocephalic vein. Computed tomography angiography: This complementary imaging exam was performed to confirm the diagnosis. Diagnosis: The patient presented an association between AVSD and PAPVC, a rare combination. The clinical picture of heart failure was preponderant, characterized by need for diuretics and complementary exams findings, and early surgical treatment was indicated. Operation: The operation was performed through a median sternotomy with 123 minutes of cardiopulmonary bypass and 89 minutes of cross-clamping time. The patient had no postoperative complications, remaining 10 days hospitalized.

Reparación de válvula mitral y cierre de comunicación interauricular, asistida por robot: experiencia inicial / Robotic mitral valve repair and closure of atrial septal defect: report of 13 procedures

Background Robot-assisted minimally invasive heart surgery is an effective alternative when compared with classical approaches. It has a low mortality and postoperative complications and its long-term durability is comparable with conventional techniques. Aim: To report short- and long-term results with the use of a robot-assisted transthoracic approach. Patients and Methods: Review of patients undergoing heart surgery between 2015 and 2019 using a robot assisted minimally invasive technique in a single center. We analyzed demographic characteristics, surgical and early ultrasound results. Results: Thirteen procedures were reviewed, nine mitral valve repairs (MVR) in patients aged 61 ± 21 years (seven males) and four atrial septal defect (ASD) closures in patients aged from 24 to 52 years (three men). For MVR, the average extracorporeal circulation and myocardial ischemia times were 120 ± 20.9 and 89 ± 21 minutes, respectively. The median hospitalization was four days. Two cases of MVR had postoperative complications. There was no mortality. All cases showed improvement in their symptoms. Ultrasound findings showed no postoperative mitral insufficiency except in one case. Conclusions: We report very good results in both complex mitral repair and CIA closure, comparable to centers with high standards in minimally invasive robot-assisted heart surgery.