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)

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Análisis demográfico de una clínica de cardiopatías congénitas del Instituto Mexicano del Seguro Social, con interés en el adulto / Demographic analysis of a congenital heart disease clinic of the Mexican Institute of Social Security, with special interest in the adult

Resumen Introducción: Las cardiopatías congénitas (CC) en México se presentan con una cifra estimada entre 18,000-20,000 nuevos casos por año. La mayor parte de los centros que atienden a estos enfermos son exclusivamente pediátricos y el Instituto Mexicano del Seguro Social (IMSS) cuenta con una clínica que atiende a todas las edades. Objetivo: Analizar los aspectos demográficos de una clínica de CC del niño y del adulto del IMSS. Métodos: De 2011 a 2016 se realizó un estudio transversal de la clínica de CC de un hospital de tercer nivel del IMSS, incluyendo todos los pacientes con cardiopatía estructural confirmada de reciente diagnóstico. Se registró el sexo, la edad, la entidad federativa de referencia, el antecedente de embarazo y el tratamiento. Se dividió a la población en rangos de edades (< 2 años, 2.1-6 años, 6.1-10 años, 10.1-17 años y > 17.1 años). Se empleó estadística descriptiva y prueba de y2 en las variables cualitativas. Resultados: Tres mil cuatrocientos ochenta y tres enfermos con CC (relación hombre:mujer,0.8:1.2), las cardiopatías acianógenas de flujo pulmonar aumentado son el grupo más grande (47.2%); 25.6% fueron adultos y 35% de las mujeres con antecedente de al menos una gesta. En general la cardiopatía más frecuente fue la comunicación interauricular. La entidad federativa con mayor frecuencia de adultos de reciente diagnóstico fue Chiapas (33.82%); el 7% no fueron candidatos a ningún tratamiento por complicaciones inherentes a la cardiopatía. Conclusiones: Existe un diagnóstico tardío de la atención de las CC en la etapa adulta. Es necesario crear un registro nacional para promover nuevas políticas de salud y distribución de recursos destinados a estos pacientes.

Abstract Introduction: Congenital heart disease (CHD) has an incidence of 8-10 cases per 1000 live births. In Mexico, there are 18,000-20,000 new cases per year. Most tertiary care centers for CHD attend only pediatric population; the Mexican Institute of Social Security (IMSS) has a clinic that attends pediatric and adult population. Objective: To analyze the demographic aspects of the CHD clinic of IMSS. Methods: From 2011 to 2016 a cross-sectional study of the CC clinic of a third level hospital of the IMSS, including all patients with confirmed structural heart disease of recent diagnosis was carried out. The sex, age, reference entity, antecedent of pregnancy and treatment were registered. The population was divided into age ranges (< 2 years, 2.1-6 years, 6.1-10 years, 10.1-17 years and > 17.1 years). Descriptive statistics and y2 test were used in qualitative variables. Results: 3,483 patients with CHD (male:female ratio, 0.8:1.2) were included. Increased pulmonary flow acyanogenic cardiopathies were the most frequent CHD group (47.2%), with atrial septal defect being the most frequent diagnosis overall; 25.6% were adults and 35% of women with a history of pregnancy. Chiapas was Federal entity with greater proportion of patients diagnosed in the adult stage (33.82%); 7% were not candidates for any treatment for complications of the disease. Conclusions: CHD is a late diagnosis; it is necessary to create a national register to promote new health policies and a rational distribution of resources for these patients.

Tratamento intervencionista das cardiopatias congênitas: estado da arte / Interventionist treatment of congenital heart defects: state of the art

Os avanços na tecnologia nas últimas décadas em relação ao tratamento intervencionista das cardiopatias congênitas têm permitindo excelentes resultados, em termos de morbimortalidade, a curto e longo prazo para este grupo especial de pacientes.Este artigo descreve os principais tratamentos disponíveis realizados com cateterismo intervencionista neste grupo de pacientes.

Technological advances in recent decades, in relation to interventionist treatment of congenital heart defects, have enabled excellent short- and long-term results for this group of patients, in terms of morbidity and mortality. This article describes the main treatmentsavailable involving interventionist catheterization in this group of patients.

Aneurisma del tabique interauricular asociado a ictus / Atrial septal aneurysm associated with stroke

El aneurisma del tabique interauricular es una deformación de tipo sacular del tejido delgado situado en la zona del foramen oval, que se mueve libremente hacia la aurícula derecha, hacia la izquierda o ambas inclusive, con una excursión mayor de 10 mm. Se trata de una enfermedad rara, que cuenta con una fuerte asociación con el ictus. Se presentó una paciente que ingresó al centro con diagnóstico de ictus isquémico cardioembólico, en ausencia de factores de riesgo vascular relevantes y la ausencia de fibrilación auricular. El ecocardiograma transtorácico y transesofágico confirmaron el aneurisma del tabique interauricular asociado a foramen oval permeable. Se reconoció la asociación de esta entidad con el ictus cardioembólico, se explicaron los posibles mecanismos implicados en su génesis. Se recomienda tener en cuenta este diagnóstico en todo ictus criptogénico por las opciones terapéuticas de las que se dispone.

Atrial septal aneurysm is a sac-type deformation of thin tissue located in the area of the foramen ovale, which is free to move into the right atrium, left or inclusive, with greater excursion of 10 mm. It is a rare disease, which has a strong association with stroke. A patient who was admitted with a diagnosis of cardioembolic ischemic stroke, in the absence of relevant vascular risk factors and the absence of atrial fibrillation was presented. Transthoracic and transesophageal echocardiogram confirmed atrial septal aneurysm associated with patent foramen ovale. The association of this entity with cardioembolic stroke is recognized, the possible mechanisms involved in its genesis is explained. It is recommended to consider this diagnosis in all cryptogenic stroke by therapeutic options available.

Three-dimensional echocardiographic assessment of atrial septal defects.

Echocardiography provides a useful tool in the diagnosis of many congenital heart diseases, including atrial septal defects, and aids in further delineating treatment options. Although two-dimensional echocardiography has been the standard of care in this regard, technological advancements have made three-dimensional echocardiography possible, and the images obtained in this new imaging modality are able to accurately portray the morphology, location, dimensions, and dynamic changes of defects and many other heart structures during the cardiac cycle.

Comparison of Angiographic Outcomes of Side Branch Ostium at Bifurcation Coronary Lesion between Two-stent and One-stent Techniques

Although the favored strategy for coronary bifurcation intervention is stenting main vessel with provisional side branch (SB) stenting, we occasionally use two-stent strategy. The objective of this study was to investigate the angiographic outcome of SB ostium in two-stent group, compared with one-stent group. We analyzed 199 patients with bifurcation lesion who underwent percutaneous coronary intervention (PCI) with drug-eluting stent and follow up angiography. The patients were divided into one-stent group (167 lesions, 158 patients) and two-stent group (41 lesions, 41 patients). Prior to intervention, SB ostium minimal luminal diameter (MLD) was smaller in two-stent group than in one-stent group (1.08+/-0.55 mm vs. 1.39+/-0.60 mm; P=0.01). But, immediately after PCI, SB MLD of two-stent group became greater than that of one-stent group (2.41+/-0.40 mm vs. 1.18+/-0.68 mm; P<0.01). Six to nine months after PCI, this angiographic superiority in SB MLD of two-stent group persisted (1.56+/-0.71 mm vs. 1.13+/-0.53 mm; P<0.01), although there was larger late loss in two-stent group (0.85+/-0.74 mm vs. 0.05+/-0.57 mm; P<0.01). In terms of target lesion revascularization and target vessel revascularization rates, one-stent group showed better results than two-stent group. We could attain wider long term SB ostium after two-stent strategy than after one-stent strategy.

Relato preliminar sobre a concentração sanguínea de níquel após oclusão percutânea de comunicação interatrial com a prótese cocoon® / Preliminary report of serum nickel concentration after atrial septal defect occlusion with the cocoonTM device

Introdução: A possibilidade de ocorrer liberação de níquel na corrente sanguínea após implante de dispositivos oclusores de comunicação interatrial de última geração (Cocoon Septal Occluder®), cujo principal componente é o nitinol (55% de níquel e 45% de titânio), ainda permanece controversa, principalmente em determinados grupos de pacientes, como crianças e mulheres em idade fértil. Dessa maneira, o objetivo do presente estudo foi avaliar a correlação entre o implante da prótese e os níveis séricos de níquel. Métodos: Estudo prospectivo de coorte, longitudinal e observacional, realizado em um hospital público. Pacientes submetidos à oclusão percutânea de comunicação interatrial foram avaliados clinicamente, por meio de ecocardiograma transtorácico, e foi feita coleta de amostras de sangue em veia periférica, para a dosagem do níquel antes e após (1 dia, 1 e 3 meses) o implante. Resultados: O procedimento e os exames subsequentes foram realizados com sucesso em dez pacientes, com média de idade de 34,4 anos (variação de 5 a 60 anos). O ecocardiograma seriado comprovou a manutenção dos resultados adequados do implante dos dispositivos. Os pacientes não apresentaram manifestações que pudessem sugerir reação ao metal, como rash cutâneo, dispneia, desconforto torácico, palpitações ou migrânea. Níveis séricos de níquel não apresentaram variação significativa e se mantiveram dentro dos limites de normalidade populacional dos métodos de dosagem até os 3 meses decorridos do procedimento. Conclusões: Os resultados preliminares desta investigação com a prótese Cocoon demonstraram que, durante o período inicial de endotelização após o procedimento, não ocorreu liberação apreciável de níquel para a corrente sanguínea...

Background: The possibility of nickel release to the bloodstream after implantation of latest generation atrial septal defect occlusion devices (Cocoon Septal OccluderTM), whose main component is nitinol (55% nickel and 45% titanium), remains controversial, especially in certain groups of patients such as children and women of childbearing age. Thus, the aim of this study was to evaluate the correlation between the device implantation and serum levels of nickel. Methods: This was a prospective longitudinal observational study conducted at a public hospital. Patients undergoing percutaneous atrial septal defect occlusion were clinically evaluated using transthoracic echocardiography and peripheral vein blood sampling for serum nickel before and after (1 day, 1 and 3 months) implantation. Results: The procedure and subsequent examinations were successfully performed in ten patients, with mean age of 34.4 years (range 5 to 60 years). Serial echocardiography confirmed the maintenance of adequate results of the procedure. Patients did not show manifestations that might suggest a reaction to metal, such as skin rash, dyspnea, thoracic discomfort, palpitations or migraine. Serum nickel levels did not show any significant changes and remained within the normal range for the population, according to the dosing methods within 3 months of the procedure. Conclusions: Preliminary results of this investigation with the Cocoon device have shown that during the initial period of endothelization after the procedure there was no significant nickel release into the bloodstream...

Anomalías congénitas asociadas a la atresia esofágica / Esophageal atressia-associated congenital anomalies

Introducción: la atresia esofágica se presenta en 1 de cada 3 000 a 4 500 neonatos vivos. Existe un ligero predominio en los varones, aunque esto no es un hallazgo universal y tal vez no sea cierto para todas las variedades. Con el aumento en la supervivencia de estos niños operados de atresia esofágica, las anomalías asociadas han adquirido mayor significación; más de la mitad de estos tienen una o más anomalías acompañantes. Objetivo: caracterizar las anomalías asociadas a esta entidad. Métodos: se realizó un estudio observacional descriptivo transversal de todos los casos diagnosticados de atresia esofágica con o sin fístula traqueoesofágica, en el periodo comprendido desde enero de 2000 hasta diciembre de 2011, en el Hospital Pediátrico Docente Provincial José Luis Miranda de Santa Clara, Cuba. Resultados: predominaron las anomalías congénitas cardiovasculares, seguido de las malformaciones digestivas y respiratorias. La anomalía asociada más frecuente, junto a la comunicación interatrial, fue la malformación anorrectal. Hubo 1 caso de atresia duodenal, así como otro de agenesia diafragmática. No hubo asociaciones, y la única trisomía presentada fue una 21. Los pacientes con anomalías congénitas no cardiovasculares tienen 2,1 veces más probabilidades de morir, que los que no se asocian a este tipo de anomalías. La mortalidad global en esta serie fue del 46,9 por ciento. Conclusiones: la presencia de una anomalía congénita eleva la mortalidad en estos pacientes, sobre todo, las no cardiovasculares mayores y las cardiovasculares críticas

Introduction: esophageal atresia occurs in one out of 3 000 or 4 500 livebirths. Males are slightly predominant, but this is not a universal finding and maybe it is not valid for all varieties. With increasing survival rates of children operated on from esophageal atresia, the associated anomalies have become more significant since more than half of these children suffer one or more accompanying anomalies. Objective: to characterize the esophageal atresia-associated anomalies. Methods: an observational, cross-sectional and descriptive study of all the cases diagnosed with esophageal atresia, with or without tracheal esophageal fistula, from January 2000 to December 2011 was conducted in José Luis Miranda provincial pediatric teaching hospital in Santa Clara, Villa Clara province, Cuba. Resultados: congenital cardiovascular anomalies were prevailing, followed by digestive and respiratory malformations. The most frequent one, together with interatrial communication, was anorectal malformation. There was a case of duodenal atresia as well as another case of diaphragmatic agenesis. There was no association between them. The patients with non-cardiovascular congenital anomalies are 2.1 times more likely to die than those unrelated to this type of anomaly. The global mortality rate for this series was 46.9 percent. Conclusions: the existence of congenital anomaly raises the mortality likelihood for these patients, mainly in cases of major non-cardiovascular and critical cardiovascular anomalies

Pseudoaneurisma e CIV pós-IAM concomitantes: apresentação grave e rara / Pseudoaneurysm and VSD concomitant after AMI: severe and rare presentation

Relata-se caso raro de dupla-complicação mecânicapós-IAM anterior: pseudoaneurisma e comunicação interventricular (CIV). Paciente do sexo feminino,66 anos, tendo apresentado quadro de dor torácica típica, foi submetida à cineangiocoronariografia, que demonstrou oclusão total em terço médio de artéria descendente anterior (DA), com angioplastia realizada10 dias após o início do quadro. Evoluiu após três semanas com recidiva de dor precordial, dispneia,sopro sistólico novo, estável hemodinamicamente. Diagnóstico à cineangiocoronariografia e ao ecocardiograma de pseudoaneurisma de parede anterior de ventrículo esquerdo e CIV apical. Realizada correção cirúrgica, com ressecção do aneurisma e correção da CIV, obtendo-se ótimo resultado, apesar da gravidade do caso.

We report a rare case of double mechanical complication after anterior myocardial infarction:pseudoaneurysm and ventricular septal defect (VSD). Female patient, 66 years old, presented with typical chest pain and under went coronary angiography,which showed total occlusion in the middle third ofleft anterior descending artery (DA) with angioplasty performed 10 days after on set of symptoms. Evolved after three weeks with recurrence of chest pain, dyspnea, systolic murmur new, hemodynamically stable. Diagnostic coronary angiography and echocardiography pseudoaneurysm of the anterior wall of the left ventricle and apical VSD. Performed surgical repair with resection of the aneurysm and VSD correction, obtaining excellent results despite the gravity of the case.

Transesophageal echocardiography for diagnosis of inadvertant closure of Inferior Vena Cava opening during minimally invasive atrial septal defect closure.

Transesophageal echocardiography (TEE) is widely used in cardiac surgery. TEE provides important diagnostic and functional information before and after cardiopulmonary bypass thereby having a very important impact on perioperative clinical outcomes. We describe a case in which intraoperative TEE was instrumental in the timely diagnosis of inadvertant closure of the inferior vena cava (IVC) opening during minimally invasive surgical closure of atrial septal defect.

Live/real time three-dimensional trans esophageal echocardiographic findings in amplatzer ASD closure devices in adults

Six female patients aged from 19 to 73 years, with ostium secundum atrial septal defect underwent closure procedure with Amplatzer septal occluder device. Three-dimensional Echocardiography [3D-TEE] was done during the procedure or one day after the procedure. 3D-TEE provides incremental value over Two- dimensional trans-esophageal echocardiography in measuring Amplatzer septal occluder disc sizes and correlates well with manufacture device size. 3D-TEE will surely prove to increase the technical efficiency and it will become an important tool for the interventionists for periprocedural evaluation of device closures

Anomalia de Ebstein com ventrículo esquerdo não compactado e comunicação interatrial do tipo seio coronário: um relato de caso / Ebstein's anomaly with left ventricular noncompaction and coronary sinus atrial septal defect: a case report

As cardiopatias congênitas envolvem um grupo de malformações que podem ser frequentes ou raras. Anomalias de Ebstein, ventrículo esquerdo não compactado e comunicação interatrial, tipo seio coronário, são condições incomuns na população e a ocorrência dos três defeitos, em um mesmo paciente, não possui relato prévio na literatura médica. Neste trabalho, é relatado o caso de um homem, com 37 anos de idade, que teve o diagnóstico feito em nosso serviço, por meio do ecocardiograma, dessa extremamente rara associação.

Experiência inicial no fechamento percutâneo da comunicação interatrial tipo ostium secundum com a prótese figulla / Initial experience in the percutaneous occlusion of ostium secundum atrial septal defect with the Figulla device

INTRODUÇÃO: O tratamento percutâneo da comunicação interatrial tipo ostium secundum é a modalidade terapêutica de escolha em mais de 80 por cento dos casos. Várias próteses encontram-se disponíveis pra tal finalidade. Relatamos nossa experiência com a oclusão percutânea da comunicação interatrial com a prótese Figulla (Occlutech, Praga, República Tcheca), avaliando a segurança e a eficácia do método. Método: De abril de 2008 a março de 2010, foram realizados 25 procedimentos em dois centros de referência em 25 pacientes não-consecutivos (mediana de idade de 22 anos e de peso de 60 kg) sob anestesia geral e monitoração pela ecocardiografia transesofagica. Do total de comunicações interatriais, 23 eram únicas, com diâmetro...

BACKGROUND: Percutaneous treatment of ostium secundum atrial septal defect is the preferred therapy in over 80% of cases. Several devices are available for this purpose. We report our experience with the percutaneous closure of atrial septal defect with the Figulla device (Occlutech, Prague, Czech Republic) assessing the safety and efficacy of the procedure. METHOD: From April 2008 to March 2010, 25 procedures were performed in 2 reference centers in 25 non-consecutive patients (median age 22 years and median weight 60 kg) under general anesthesia and transesophageal echocardiographic monitoring. Atrial septal defects were single in 23 cases and multiple in 2 cases and had a mean diameter of 17.5 ± 9.4 mm. In one patient it was necessary to use two devices in two distant atrial septal defects. The mean diameter of the devices was 20.8 ± 7.4 mm and they were implanted through 9-14 F long sheaths in the femoral vein. RESULTS: Successful implantation was observed in all cases. In one case, there was embolization immediately after device release. The device was retrieved from the descending aorta followed by implantation of a new device. The mean follow-up was 12 months and the occlusion rate was 100%. There were no late complications. CONCLUSION: In this initial experience, percutaneous occlusion of the atrial septal defect with the Figulla device was easy to perform, safe and highly effective.

Biatrial myxoma and atrial septal defect

Myxomas are rarely associated with congenital cardiac anomalies. We report a case of a young female presenting with symptoms of atrial myxoma but having co-existing ASD visualized on 64 slice multidetector computed tomography [MDCT] prior to cardiac surgery. Patient had a successful resection of biatrial myxoma and ASD repair. This case is unique as the myxoma originated from the inferior margin of the ASD straddling the inferior limbus. Over the years due to the left to right shunt at the ASD, the myxoma was initially prolapsing and oscillating between the two atria. As it grew larger it obliterated the ASD and got stuck in the right atrium [RA] and continued to grow giving false impression of a right atrial mass. Multi detector computed tomography is an alternative diagnostic modality to the gold standard transesophageal echo-cardiograph for detection of an ASD in the presence of atrial myxoma. Biatrial myxoma with associated atrial septal defect has optimal postoperative results