Quadricuspid aortic valve: three cases report and literature review

Abstract Quadricuspid aortic valve (QAV) is a rare cardiac malformation. Many cases are incidentally diagnosed in aortic surgeries or autopsies and it usually appears as an isolated anomaly. The most widely classification used is the one by Hurwitz and Roberts[1], which divides 7 alphabetical subtypes based on the cusps size. The aim of this report is to describe three different anatomic presentations of this rare aortic valve anomaly.

Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Cardiopatías congénitas y nuevas técnicas de diagnóstico no invasivo: Tomografía computada multidetector (TCMD) / Congenital heart defects and new non-invasive diagnostic techniques: Multidetector computed tomography (MDCT)

Las cardiopatías congénitas presentan con frecuencia patrones anatómicos complejos y tradicionalmente se han estudiado con ecocardiografía y eventualmente con cateterismo cardíaco. Con los avances tecnológicos de las últimas décadas, la tomografía computada y la resonancia magnética nuclear han adquirido gran importancia en la evaluación cardíaca. Actualmente constituyen métodos utilizados en el estudio de variadas patologías cardiovasculares de la infancia de difícil diagnóstico ecográfico, que previamente eran evaluadas por angiografía convencional, lo cual implicaba una dosis de radiación mucho mayor (AU)

Congenital heart defects often have complex anatomical patterns and have traditionally been studied with echocardiography and eventually cardiac catheterization. With technological advances in recent decades, computed tomography and magnetic resonance imaging have become very important in cardiac evaluation. Currently, they are the methods of choice in the study of a wide range of childhood cardiovascular diseases that are difficult to diagnose with echocardiography, which were previously evaluated using conventional angiography, involving a much higher dose of radiation (AU)

Caracterización de las cardiopatías congénitas en la provincia de Matanzas. Estudio de trece años / Characterization of congenital heart diseases in Matanzas province. A thirteen years study

RESUMEN Introducción: el incremento de las cardiopatías congénitas en los últimos años y la relevancia que ha alcanzado en la provincia de Matanzas como una de las primeras causas de muerte infantil, es un tema que preocupa a los especialistas; sin embargo, la gestión en la búsqueda de nuevos conocimientos en esta área de interés aún no es suficiente. Objetivo: caracterizar la morbimortalidad por cardiopatías congénitas en la provincia de Matanzas en el período comprendido de enero del 2002 a diciembre del 2014. Materiales y métodos: se realizó un estudio descriptivo, transversal cuyo universo de trabajo estuvo conformado por todos los pacientes de edad pediátrica a los cuales se les diagnosticó cardiopatía congénita y fueron atendidos por el servicio de Cardiología del Hospital Pediátrico Docente "Eliseo Noel Caamaño". Se incluyeron todos los niños diagnosticados por ultrasonografía durante el período embriofetal. Resultados: la comunicación interventricular resultó ser la más frecuente. Hubo predominio de cardiopatías en pacientes masculinos y procedencia urbana; siendo diagnosticadas la mayoría en la etapa neonatal. La tasa de prevalencia por año presenta una tendencia creciente, mientras que la de mortalidad resultó decreciente. El Síndrome de Down fue la entidad genética más frecuente asociada con las cardiopatías congénitas al igual que las malformaciones craneofaciales. Conclusiones: estos resultados muestran el verdadero comportamiento de esta entidad en esta provincia (AU).

ABSTRACT Introduction: the increase of congenital heart diseases in recent years and the relevance it has achieved in the province of Matanzas as one of the main causes of infantile death is a matter of concern for specialists; however, management of search for new knowledge in this area of interest is still not enough. Objective: to characterize morbidity and mortality due to congenital heart disease in the province of Matanzas in the period from January 2002 until December 2014. Materials and methods: A descriptive, cross-sectional study was carried out in a universe formed by all the patients of pediatric age, diagnosed with congenital heart disease and treated by the Cardiology service of the Teaching Pediatric Hospital "Eliseo Noel Caamaño". All the children diagnosed by ultrasonography during the embryofetal period were included. Results: intraventricular communication was the most frequent. There it was a general predominance of heart disease in male patients, and from urban origins, most of them diagnosed at the neonatal stage. The prevalence rate per year shows an increasing tendency, while mortality decreased. Down syndrome was the most common genetic entity associated to congenital heart disease as well as craniofacial malformations. Conclusions: these results show the true behavior of this entity in our province (AU).

Investigation of the effect of gestational diabetes on fetal cardiac tissue in streptozotocin induced in rats

Abstract Purpose: To investigate the cause of congenital anomalies resulted from gestational diabetes on fetal cardiac tissue in experimental animal study model. Methods: Totally 12 female Wistar albino rats were divided into two groups, each consisting of 6 rats. Streptozotocin (60 mg/kg) was administered intraperitoneally to the study group by dissolving in citrate solution. The rats with a blood glucose level of 200 mg/dL and above were considered to be diabetic rats. Total antioxidant status (TAS), total oxidative stress (TOS) and oxidative stress index (OSI) values were calculated in the cardiac tissues and maternal serum samples of the fetuses delivered by cesarean section after the mating process. The cardiac tissues were also subjected to histopathological examination. Results: TOS and OSI values in fetal cardiac tissues of the diabetic rats were found to be significantly higher than that of the control group (p=0.026 and p=0.005). Histopathological examination revealed that the mitotic index was lower and the cell organization was found to be damaged in the fetuses of the study group rats. Conclusion: Increased levels of free oxygen radicals considered to be due to hyperglycemia may cause congenital anomalies, especially during organogenesis period, by disrupting cell homeostasis and adversely affecting mitosis.

Diagnóstico prenatal de cardiopatías congénitas / PRENATAL DIAGNOSIS OF CONGENITAL HEART DEFECTS FOR DETECTING CARDIAC MALFORMATIONS

Congenital heart defects or congenital cardiac malformations represent one of the major causes of death in children younger than 1 year. The incidence varies between 4 - 13 for each 1000 newborns. Represents the severe structural malformation of the higher prevalence worldwide. In the "Professor A. Posadas Hospital" during the year 2015, it was registered a frequency of congenital anomalies of 7,1 % , being in the first place the musculoskeletal malformations with 19,4 %, and in the second place we can find the cardiovascular manifestations with 18.1 %. It is important the prenatal diagnosis in order to carry out an assessment to their parents, to reach the birth in a high complexity center and in the possible case, to plan the possibility of performing a fetal therapy. There exists scientific evidence that proves that the prenatal diagnosis improves the neonatal survival, even being this anomalies the less diagnosticated during the prenatal period These considerations are discussed in the article.

Investigation of Copy Number Variation in Children with Conotruncal Heart Defects / Investigação da Variação no Número de Cópias Gênicas em Crianças com Defeito Cardíaco Conotruncal

Background: Congenital heart defects (CHD) are the most prevalent group of structural abnormalities at birth and one of the main causes of infant morbidity and mortality. Studies have shown a contribution of the copy number variation in the genesis of cardiac malformations. Objectives: Investigate gene copy number variation (CNV) in children with conotruncal heart defect. Methods: Multiplex ligation-dependent probe amplification (MLPA) was performed in 39 patients with conotruncal heart defect. Clinical and laboratory assessments were conducted in all patients. The parents of the probands who presented abnormal findings were also investigated. Results: Gene copy number variation was detected in 7/39 patients: 22q11.2 deletion, 22q11.2 duplication, 15q11.2 duplication, 20p12.2 duplication, 19p deletion, 15q and 8p23.2 duplication with 10p12.31 duplication. The clinical characteristics were consistent with those reported in the literature associated with the encountered microdeletion/microduplication. None of these changes was inherited from the parents. Conclusions: Our results demonstrate that the technique of MLPA is useful in the investigation of microdeletions and microduplications in conotruncal congenital heart defects. Early diagnosis of the copy number variation in patients with congenital heart defect assists in the prevention of morbidity and decreased mortality in these patients. .

Fundamento: Os defeitos cardíacos congênitos são o grupo de anormalidades estruturais mais prevalentes ao nascimento e uma das principais causas de morbidade e mortalidade infantil. Estudos têm mostrado a contribuição da variação no número de cópias na gênese das malformações cardíacas. Objetivos: Investigar a variação no número de cópias gênicas em crianças com defeito cardíaco conotruncal. Métodos: Multiplex Ligation-dependent Probe Amplification (MLPA) foi realizado em 39 pacientes com defeito cardíaco conotruncal. Avaliação clínica e laboratorial foi realizada em todos os pacientes. Os pais dos probandos que apresentaram alterações também foram investigados. Resultados: Variação no número de cópias foi detectada em 7/39 pacientes: deleção 22q11.2, duplicação 22q11.2, duplicação 15q11.2, duplicação 20p12.2, deleção 19p, duplicação 15q e 8p23.2 com duplicação 10p12.31. As características clínicas foram compatíveis com o relatado na literatura associadas com microdeleção/microduplicação encontrada. Nenhuma dessas alterações foi herdada dos pais. Conclusões: Nossos resultados demonstram que a técnica de MLPA é útil na investigação de microdeleções e microduplicações em defeitos cardíacos congênitos conotruncais. O diagnóstico precoce das variações no número de cópias em pacientes com defeito cardíaco congênito auxilia na prevenção de morbidade e diminuição da mortalidade nesses pacientes. .

Comportamiento clínico epidemiológico de las cardiopatías congénitas en el primer año de vida / Clinical epidemiological behavior of congenital cardiopathies in first year children

Fundamento: los defectos congénitos del corazón son el grupo más común de anomalías congénitas y en su mayoría constituye, por su evolución e impacto fisiopatológico, en una enfermedad crónica de la infancia. Objetivo: describir algunas características clínicas de los pacientes con cardiopatías congénitas en la provincia de Sancti Spiritus. Metodología: se realizó un estudio descriptivo de corte longitudinal sobre el comportamiento de las cardiopatías congénitas en la provincia de Sancti Spiritus durante el período comprendido de enero del 2011 a diciembre del 2012 abarcó a todos los niños nacidos en la maternidades de toda la provincia, así como los atendidos en la consultas de cardiología pediátrica provincial. Las variables utilizadas fueron sexo, edad al diagnóstico, síntomas clínicos de presentación. Resultados: la incidencia de cardiopatía congénita fue de 3,3 por cada mil nacidos vivos, el diagnóstico de dicha afección ocurrió durante el 1er mes de nacido en 19 pacientes para un 56 %. Los signos clínicos de presentación fueron soplo asintomático 62 %, seguido de las dificultad cardiorespiratoria. Conclusiones: las malformaciones congénitas del aparato cardiovascular son un elemento importante a tener en cuenta al valorar a todos los pacientes al nacimiento en las maternidades.

Background: congenital heart defects are the most common group of congenital anomalies and the majority of them due to their evolution and pathophysiological impact are a chronic disease of childhood. Objective: to describe some clinical characteristics of patients with congenital heart disease in the province of Sancti Spíritus . Methodology: a descriptive longitudinal study on the behavior of congenital cardiopathies in the province of Sancti Spíritus from January 2011 to December 2012, it encompassed all children born in maternity hospitals across the province, as well as all cared patients at the provincial pediatric cardiology consultations. The variables used were sex, age at diagnosis and clinical presenting symptoms. Results: the incidence of congenital heart disease was 3.3 per thousand live births, the diagnosis of the condition occurred during the 1st month of age in 19 patients for a 56%. Clinical signs at presentation were asymptomatic heart murmur 62%, followed by cardiorespiratory difficulty. Conclusions: congenital malformations of the cardiovascular system are important to consider when assessing all patients at birth in maternity hospitals.

Soporte ventricular mecánico en el paciente pediátrico / Mechanical ventricular support in the pediatric patient", "_i": "

Para los pacientes pediátricos que presentan insuficiencia cardíaca aguda o crónica, ya sea por la evolución de la enfermedad o porque han sido sometidos a cirugía correctiva o paliativa, los dispositivos de soporte o asistencia ventricular son utilizados como opción de tratamiento cuando existe resistencia al manejo farmacológico; el advenimiento de estos dispositivos permiten al paciente la recuperación postquirúrgica o mantenerse con vida cuando el trasplante cardíaco es la única opción en los casos que no se tiene donante en puerta. Estos dispositivos pueden disminuir la mortalidad considerablemente; sin embargo, su uso es limitado debido a la anatomía y fisiología de los niños. Esta revisión tiene como objetivo dar a conocer los dispositivos de soporte ventricular disponibles más utilizados para pacientes pediátricos y los que se encuentran en fase de desarrollo.

For pediatric patients with heart disease who have acute or chronic heart failure either by the evolution of the disease or who have undergone corrective or palliative surgery, devices or VAD support are best used as a treatment option when there is resistance to pharmacological management, the advent of these devices allow patients to postoperative recovery or stay alive when heart transplantation is the only option and you do not have a donor available. These devices can reduce mortality considerably, however their use is limited because of the anatomy and physiology of pediatric patients. This review aims to raise awareness of available ventricular support devices commonly used for pediatric patients and those who are under development.

One and a half ventricular repair as an alternative for hypoplastic right ventricle / Operação de um ventrículo e meio como uma alternativa para o ventrículo direito hipoplásico

OBJECTIVE: Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. METHODS: From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). RESULTS: There was one (11.1 percent) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5 percent) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.

OBJETIVO: Pacientes com cardiopatia congênita complexa e caracterizada por hipoplasia do ventrículo direito têm uma opção de correção paliativa com a operação de um ventrículo e meio. MÉTODOS: De julho de 2001 a março de 2009, nove pacientes (idade média de 5,2 anos, variando de 3 a 9 anos), com hipoplasia do ventrículo direito, foram submetidos à correção com a técnica de um ventrículo e meio. Os diagnósticos pré-operatórios foram: atresia pulmonar com septo ventricular intacto, em seis casos, e anomalia de Ebstein, em três casos. Seis pacientes tiveram derivação cavo-pulmonar bidirecional (operação de Glenn) prévia. A abordagem cirúrgica foi realizada com circulação extracorpórea e os defeitos intracardíacos foram corrigidos: fechamento do defeito do septo atrial (nove casos), reconstrução da via de saída do ventrículo direito com prótese pulmonar porcina (sete casos); plástica da valva tricúspide (três casos). RESULTADOS: Houve um (11,1 por cento) óbito. Todos os pacientes receberam alta hospitalar em boas condições clínicas. Um paciente apresentou estenose pulmonar distal à prótese, sendo necessária a reintervenção cirúrgica. Houve um (12,5 por cento) óbito tardio, após a reoperação. Sete (77,8 por cento) pacientes foram seguidos por período médio de 39,8 meses (intervalo de 16 meses para 8,4 anos) e se encontram em boas condições clínicas. CONCLUSÕES: O tratamento cirúrgico de cardiopatias congênita, na presença de um ventrículo direito hipoplásico, por meio da operação de um ventrículo e meio, tem menor risco cirúrgico que a correção biventricular, mantendo o átrio direito com baixa pressão, fluxo sanguíneo pulmonar pulsátil e melhora da saturação sistêmica de oxigênio. Os resultados de curto e médio prazo são satisfatórios; porém será necessário um maior tempo de seguimento para provar a eficácia desta abordagem cirúrgica a longo prazo.

Anatomic diagnosis of congenital heart disease. A practical approach based on the sequentiality principle / Diagnóstico anatómico de las cardiopatías congénitas

Based on the sequentiality principle, this review proposes a practical method that allows the systematization of the anatomic diagnosis of congenital heart disease. We emphasize the need to use sequential connection between the different cardiac segments: atria, ventricles and great arteries. Five ordered steps are defined, which include determination of atrial situs and of the connection features between the ventricles and the great arteries. Related lesions and some additional special features are a second stage in the sequential analysis of congenital heart disease, which is also important for the integral diagnosis.

En esta revisión se propone, en base al principio de secuencialidad, un método práctico que permite sistematizar el diagnóstico anatómico de las cardiopatías congénitas. Se enfatiza la necesidad de utilizar la conexión secuencial entre los distintos segmentos cardiacos que son los atrios, los ventrículos y las grandes arterias. Se definen cinco pasos ordenados que incluyen, en primera instancia, la determinación del situs atrial y de las características de la conexión entre los ventrículos y las grandes arterias. Las lesiones asociadas y algunas particularidades adicionales ocupan una segunda etapa en el análisis secuencial de las cardiopatías congénitas, que también tienen importancia en el diagnóstico integral.

Malformações cardíacas congênitas em necropsias pediátricas: características, associações e prevalência / Congenital cardiac malformations in pediatric necropsies: characteristics, associations and prevalence

FUNDAMENTO: No Brasil, desde 2001, as anomalias congênitas constituem a segunda causa de morte em crianças menores de um ano, respondendo os defeitos cardiovasculares por 39,4 por cento destes óbitos. OBJETIVOS: Conhecer a prevalência e as características das malformações cardíacas congênitas em necropsias pediátricas realizadas no Hospital Regional da Asa Sul, Brasília, DF, de janeiro de 1996 a dezembro de 2007. MÉTODOS: Estudo descritivo, transversal, sendo revisadas 1591 necropsias realizadas de janeiro de 1996 a dezembro de 2007 e encontradas 189 (11,9 por cento) com malformações cardíacas congênitas, incluídas neste trabalho. RESULTADOS: As anomalias cardíacas foram observadas principalmente no grupo dos neomortos (117/61,9 por cento), seguindo-se o grupo dos natimortos (35/18,5 por cento), o grupo dos lactentes (30/15,9 por cento) e o grupo dos pré-escolares (7/3,7 por cento), não havendo nenhum caso entre os escolares. As principais alterações detectadas nessa população foram: a comunicação interatrial em 96 pacientes (27 por cento), a comunicação interventricular em 66 (18,5 por cento) e a persistência do canal arterial em 51 (14,3 por cento), sem predomínio entre os sexos. Em 133 pacientes (70,4 por cento), as cardiopatias eram múltiplas e em 96 (50,8 por cento) estavam associadas a anomalias de outros órgãos e sistemas; dentre esses, 45 (23,8 por cento) apresentaram cardiopatias como componentes de síndromes, destacando-se a alta prevalência de doenças cromossômicas, especialmente as trissomias, em todas as faixas etárias. CONCLUSÕES: Os resultados deste trabalho mostram elevada prevalência de anomalias cardíacas congênitas em nosso meio e distribuição e associações semelhantes às observadas em países desenvolvidos. A elevada mortalidade associada a essas anomalias alerta para a necessidade de pesquisas mais abrangentes a fim de se conhecer os fatores de risco e buscar a prevenção primária de alguns desses defeitos.

BACKGROUND: In Brazil, since 2001, the congenital abnormalities have represented the second cause of death in children younger than one year, with cardiovascular defects being responsible for 39.4 percent of these deaths. OBJECTIVES: To establish the prevalence and the characteristics of the congenital cardiac malformations in pediatric necropsies performed in Hospital Regional da Asa Sul, Brasília, DF, Brazil, from January 1996 to December 2007. METHODS: This is a descriptive, cross-sectional study that reviewed 1,591 necropsies performed from January 1996 to December 2007 and found 189 (11.9 percent) with congenital cardiac malformations, included in this study. RESULTS: The cardiac abnormalities were observed mainly in the group of live births (117/61.9 percent), followed by the stillbirths (35/18.5 percent), the infant group (30/15.9 percent) and the preschoolers' group (7/3.7 percent), with no cases identified among school-aged children. The main alterations detected were: interatrial communication in 96 patients (27 percent), interventricular communication in 66 patients (18.5 percent) and patent ductus arteriosus in 51 (14.3 percent), with no predominance of either sex. In 133 patients (70.4 percent), the cardiopathies were multiple and in 96 (50.8 percent) they were associated with anomalies in other organs and systems; among these, 45 (23.8 percent) presented cardiopathies as syndrome components, especially trisomies, at all age ranges. CONCLUSIONS: The results of the present study show a high prevalence of congenital cardiac anomalies in our country and distribution and association that were similar to the ones observed in developed countries. The high mortality associated to such anomalies highlights the need for more comprehensive research in order to identify the risk factors and seek the primary prevention of some of these defects.

Surgical Management of Left Ventricular Outflow Tract Obstruction after Biventricular Repair of Double Outlet Right Ventricle

Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3+/-18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0+/-37.7 mmHg (15-140) after a mean follow-up of 9.5+/-6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2+/-11.4 mmHg (0-34) after a mean follow-up of 5.6+/-2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO.

Experiencia de 6 años en la evaluación de las cardiopatías congénitas con resonancia magnética / Evaluation of congenital heart disease using magnetic resonance imaging: a 6 year experience

Introducción: La Resonancia Magnética (RM) es de gran ayuda como método complementario en la evaluación de las cardiopatías congénitas debido a que provee información anatómica y funcional. Objetivo: Describir la utilidad, indicaciones y hallazgos más comunes en la evaluación de las cardiopatías congénitas por RM Método: Se realizó un análisis retrospectivo de las RM cardíacas efectuadas entre el año 2002 hasta el 2008 en el Hospital Clínico de la Pontificia Universidad Católica de Chile. Se incluyó la totalidad de los pacientes en edad pediátrica y aquellos adultos en los cuales la indicación del examen fue por cardiopatía congénita, tanto para evaluación preoperatoria como para control postquirúrgico. Se realizó estudio anatómico, funcional y se utilizó contraste paramagnético intravenoso en los casos necesarios. Resultados: Se realizaron 180 estudios. La indicación más frecuente fue la evaluación postoperatoria de la tetralogía de Fallot, seguida por drenaje venoso pulmonar anómalo, miocardiopatía no compactada y coartación de Aorta, entre otros. Conclusiones: La RM cardíaca es de gran utilidad en la evaluación de las cardiopatías congénitas, ya que demuestra detalles anatómicos con excelente resolución de contraste y provee además información funcional y de dinámicas de flujo.

Background: Magnetic resonance imaging (MRI) is helpful in the evaluation of congenital heart diseases because it provides anatomical and functional information. Aim: To describe the utility, indications and more commons findings in the evaluation of the congenital heart diseases using MRI.Method: A retrospective analysis of the cardiac MRI studies in patients with congenital heart diseases between 2002and 2008 was completed. Children and adults were included. Preoperative evaluation and or post surgical findingswere analyzed. Anatomical and functional studies were made and intravenous paramagnetic contrast agents were used in the selected cases.Results: 180 MRI studies were analyzed. The most frequent indication was corrected Fallot's Tetralogy followed by anomalous pulmonary venous connection, left ventricular non compaction and coarctation of the aorta. Flow dynamics studies were successfully performed in several cases. Conclusion: Cardiac MRI demonstrates anatomical details with excellent contrast resolution and allows functionaland flow dynamics studies.

Psicoterapia pais-bebê no contexto de malformação do bebê: repercussões no olhar da mãe acerca do desenvolvimento do bebê / Parent-infant psychotherapy in the context of malformation: implications on the mother’s perception about the baby’s development

Introdução: Diante de um bebê com malformação, as representações maternas podem fi car bastante distorcidas. Neste contexto, percebe-se a necessidade de intervenções psicológicas que ajudem os pais a adequar suas representações psíquicas, visando descontaminá-las de projeções e conflitos para facilitar o desenvolvimento físico e psíquico do bebê. Nesse sentido, o presente estudo investigou o impacto da psicoterapia breve mãe-bebê sobre as representações maternas a respeito do desenvolvimento do bebê quando este apresentava uma malformação cardíaca. Método: Relata-se o processo de psicoterapia breve (24 sessões) de uma díade composta por uma mãe de 22 anos e seu bebê de 11 meses. Mostra-se, ao longo do tratamento, como foram se dando algumas mudanças no tema vida e crescimento (Stern, 1997). Resultados: Os resultados revelam mudanças, tanto nas representações da mãe sobre o bebê, como na sua postura em relação à busca de informações sobre a malformação e sobre cuidados com o filho. Conclusões: Os resultados destacam o benefício da psicoterapia pais-bebê para o contexto de malformação do bebê e, especialmente, para as representações da mãe acerca do desenvolvimento do bebê.

Introduction: Maternal representations of the infant can be quite distorted when the infant has a malformation. Within this context, there is a need of psychological interventions aimed at helping parents to adapt their psychic representations with the purpose of avoiding projections and conflicts in order to promote the infant’s physical and psychic development. In this sense, the present study investigated the impact of a short-term motherinfant psychotherapy on the maternal representations of the infant’s development when the infant had a cardiac malformation. Method: The short psychotherapy process (24 sessions) of a mother-infant pair is reported. The authors describe how the issue of life and growth changes throughout treatment. Results: The results evidenced several changes in the maternal representations of the infant, as well as the mother’s attitude regarding the search of information on malformation and child care. Conclusions: The results emphasize the benefit of short mother-infant psychotherapy for the context of infant’s malformation, mainly for the maternal representations of the infant’s development.

Tectocerebellar dysraphia and occipital encephalocele: an unusual association with abdominal situs inversus and congenital heart disease.

Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.

Estudio morfopatológico de la conexión anómala total de venas pulmonares / Morphopathologic study of total anomalous pulmonary venous connection

To know the morphological features and the frequency of pulmonary anomalous connection types, 106 hearts from the collection of the Instituto Nacional de Cardiologia Ignacio Chávez were studied with the segmental sequential system. The atrial situs, connections of cardiac segments, morphology of cardiac chambers, interatrial septum, venous collector, sites of anomalous pulmonary venous connection and associated anomalies were determined. Darling's classification was used. The atrial situs was: solitus (73), dextroisomerism (30) and inversus (3). In the supradiaphragmatic level the supracardiac connection (44) was distributed into superior vena cava (24) and vertical vein (20). The cardiac level (39) was divided into venous coronary sinus (22) and to the right atrium (17). The infradiaphragmatic level was to the portal vein (10) and the mixed group (13) had different combinations. 100 hearts had atrial septal defect and 6 had patent foramen ovale. The hearts with dextroisomerism had several intracardiac malformations; 14 specimens had obstructed venous collectors. Three important aspects in diagnosis are: site of anomalous connection, size of atrial septal defect and obstruction in the venous collector; this information is valuable to understand the physiopathology, to establish the diagnosis and to help the surgeon to chose the best surgical strategy.

OEIS complex with major cardiac malformation: a case report.

OEIS Complex is a rare congenital multisystem defect that consists of omphalocele, exstrophy, imperforate anus and spinal defects. We report a case of such complex with additional major cardiac and other multisystem anomalies which are rarely described in literature. The authors give a review of literature on this infrequent complex along with a discussion on its pathogenesis, differential diagnosis and prenatal diagnosis.