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)

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Dextrocardia con doble salida del ventrículo derecho con comunicación interventricular no relacionada asociada a malposición de grandes vasos / Right ventricular double outlet dextrocardia with unrelated ventricular septal defect associated with great vessel malposition

Resumen En el siguiente reporte se expone un caso complejo en el que coexisten malformaciones cardiacas congénitas de presentación atípica con un doble tracto de salida del ventrículo derecho, en un paciente con múltiples anomalías menores en la exploración física y un cariotipo normal. La atipia de cada una de estas y su coexistencia hacen pensar en posibles alteraciones genéticas que aún son desconocidas. Lo anterior supone un reto terapéutico con el fin de restaurar una fisiología cardiaca compatible con la vida, lo cual se logra en este caso mediante un cerclaje exitoso de la arteria pulmonar.

Abstract The following report presents a complex case in which congenital cardiac malformations of atypical presentation coexist with a double outlet right ventricle, in a patient with multiple minor abnormalities on the physical examination and a normal karyotype. The atypia of these and their coexistence suggest possible genetic alterations that are still unknown. Therefore, a therapeutic challenge in order to restore a cardiac physiology compatible with life is proposed, which is achieved in this case through a successful banding of the pulmonary artery.

Éxitus materno tras cesárea en gestante con anomalía de Taussig-Bing y síndrome de Eisenmenger / Maternal Exitus after Cesarean Section in Pregnant Women with Taussig-Bing Anomaly and Eisenmenger Syndrome

La anomalía de Taussig-Bing es una cardiopatía congénita cianosante caracterizada por la dextrotransposición de grandes vasos. Esta produce una doble salida arterial desde el ventrículo derecho, asociado a una comunicación interventricular. Este cuadro puede generar una hipertensión pulmonar secundaria al aumento de las resistencias vasculares y un flujo reverso cardiaco, conocido como síndrome de Eisenmenger. Normalmente, se presenta antes de la pubertad, aunque en ocasiones, puede debutar en la vida adulta, progresando durante dicha etapa. Clínicamente, se caracteriza por rasgos crónicos, como las acropaquias, la disnea, la sensación de cansancio o la cianosis. El diagnóstico de este tipo de cardiopatías se basa en la clínica y en las pruebas de imagen, preferentemente en el estudio ecocardiográfico fetal o durante la edad pediátrica. El tratamiento de elección es la corrección quirúrgica de las malformaciones cardiacas, siendo preferente la rectificación de la salida de la aorta y el cierre de la comunicación interventricular. El pronóstico depende del grado de hipertensión pulmonar, del momento del diagnóstico y de la corrección quirúrgica precoz. A edades tempranas se obtiene un mejor resultado, aunque las tasas de mortalidad alcanzan 50 % en algunos casos, incluso tras una corrección quirúrgica óptima. La gestación no está recomendada en pacientes que padecen dicha patología, la cual se ha contraindicado, según algunos estudios, en ausencia de tratamiento adecuado. Así pues, describimos un caso en el que una gestante con una anomalía Taussig-Bing sufre una atonía uterina y un posterior paro cardiorrespiratoria tras el parto, realizado mediante cesárea electiva, tras la que la paciente falleció(AU)

The Taussig-Bing anomaly is a congenital cyanosis characterized by the dextrotransposition of large vessels. It produces a double arterial exit from the right ventricle, associated with an interventricular communication. This may lead to pulmonary hypertension secondary to increased vascular resistance and a cardiac reverse flow, known as Eisenmenger syndrome. Generally, it occurs before puberty, although occasionally, it can debut in adults, progressing during that stage. It is clinically characterized by chronic features, such as acropachies, dyspnea, tiredness or cyanosis. The diagnosis of this type of heart disease is based on clinical exam and imaging tests, if at all possible in the fetal echocardiographic study or during the pediatric age. The surgical correction of cardiac malformations is the treatment of choice, modifying the aortic exit and closing of ventricular septal defect. The prognosis depends on the degree of pulmonary hypertension, the time of diagnosis, and the early surgical correction. Better outcome is obtained at early ages, although mortality rates reach 50% in some cases, even after optimal surgical correction. Pregnancy is not recommended in patients suffering from this disease. Some studies contraindicate pregnancy in absence of proper treatment. Thus, we describe a case of a pregnant woman with a Taussig-Bing anomaly, who suffered uterine atony and a subsequent cardiorespiratory arrest after delivery. Elective cesarean section was performed. This patient died(AU)

Discordância atrioventricular associada à dupla via de saída do ventriculo direito: um raro caso com 28 anos e nenhuma cirurgia / Atrioventricular discordance and double outlet right ventricle: a rare case of a 28 year old man without surgery

Relatamos o raro caso de um paciente de 28 anos, com diagnóstico de discordância atrioventricular e dupla via de saída do ventrículo direito, obtido somente após o ecocardiograma transesofágico e sem cirurgia prévia onde a estenose pulmonar importante atuou como bandagem pulmonar natural, possibilitando essa evolução.

We report a rare case of a 28 years old patient with the diagnosis of atrioventricular discordance and double outlet right ventricle, obtained only after transesophageal echocardiogram and no surgery. Severe pulmonary stenosis was the natural banding allowing this outcome.

Doble salida de ventrículo derecho: Enfoque embriológico / Double outlet right ventricle: Embryological approach

Objetivo: Se presenta una explicación patogenética sobre la morfogénesis de la doble salida de ventrículo derecho que explica su origen. Método: Se describieron 35 corazones con doble salida de ventrículo derecho, 15 con grandes arterias ligeramente cruzadas, 10 con arterias lado a lado y 10 con aorta anterior y pulmonar posterior. Se comparó el plano de separación de las vías de salida y de las grandes arterias en los 3 tipos de esta cardiopatía, con el plano de la tabicación troncoconal normal del corazón embrionario. Se determinó el plano cefálico del tabique troncoconal y se comparó con la posición de su borde inferior, con lo cual se calculó el grado de torsión troncoconal en cada grupo de esta cardiopatía; esto permitió inferir el tipo de giro de ese tabique en las 3 formas anatómicas mencionadas. Resultados: En las grandes arterias ligeramente cruzadas el giro troncoconal fue de 135°,en las arterias lado a lado fue de 90°, en la aorta anterior derecha fue de 0° y en la aorta anterior izquierda fue de -90°. Conclusión: Embriológicamente esta cardiopatía se origina por persistencia de la continuidad entre el ventrículo derecho y el troncocono, que origina las vías de salida y las grandes arterias. Sus variantes anatómicas se originan por una detorsión progresiva del tabique troncoconal continuada con una torsión de -90°.

Objective: It is proposed a pathogenetic explanation that explains the morphogenesis of the anatomic variants of double outlet right ventricle. Method: An anatomic embryological correlation was made in which the plane separating the outlets and great arteries in the types of this cardiopathy was compared with the normal truncoconal septum in the embryonic heart. Thirty five hearts with double outlet right ventricle were described, fifteen with great arteries slightly crossed, ten with side by side great arteries and ten with anterior aorta and posterior pulmonary artery. The cephalic border of the truncoconal septum was compared with its inferior border in each group. With this procedure we calculated the type of torsion of the truncoconal septum. Results: In the slightly crossed great arteries the truncoconal twist was of 135° in side by side great arteries the twist was of 90° and in anterior right aorta the truncoconal septum was straight with 0° of rotation, and with left anterior aorta the rotation was of -90°. Conclusion: Embryologically double outlet right ventricle is originated by the persisting continuity between the right ventricle with the truncus and conus which form the great arteries and their outlets. The anatomic variations are the consequence of progressive detortion of the truncoconal septum followed by a torsion of -90°.

Arritmia ventricular derecha de alta densidad en pacientes sin cardiopatía estructural: características clínicas y electrocardiográficas / Right ventricular arrhythmia of high density in patients without structural heart disease: clinical characteristics and electrocardiographic

Las arritmias ventriculares originadas en el ventrículo derecho son un motivo frecuente de consulta en cardiología y suelen ser medicadas con antiarrítmicos. Objetivo. Describir las características clínicas y electrocardiográficas de pacientes con arritmia ventricular derecha de alta densidad. Métodos. presentamos 32 pacientes con arritmia ventricular de alta densidad (CVP ≥ 30/h), originada en el VD y con ecocardiografía normal. Resultados. la edad promedio de los pacientes fue 56 ± 18 años, la HTA y la dislipidemia fueron las comorbilidades más frecuentes, el 85% se encontraba sintomático en la primera evaluación y en su mayoría estaban medicados con fármacos antiarrítmicos. En la monitorización electrocardiográfica ambulatoria se encontró un promedio de 9350,09 ± 3455 CVP/ 24 h (casi un tercio tuvieron más de 10 000 CVP), los dos patrones ECG más frecuentes en V1 fueron el QS y el rS, con eje inferior derecho y onda R monofásica en D I. EL 45% de los pacientes se encontraron en tratamiento con betabloqueadores En el seguimiento de 6 y 12 meses los pacientes referían menos síntomas incluso sin tratamiento farmacológico, ninguno de ellos requirió del uso de amiodarona. Conclusión. Las arritmias ventriculares derechas originadas en el tracto de salida, de alta densidad y no asociadas a cardiopatía estructural, presentan una evolución y pronóstico favorable, incluso sin tratamiento farmacológico.

The ventricular arrhythmias originated in the rigth ventricle are one of the most common reasons of cardiology consulting and almost always medicated with antiarrhythmic drugs. Objective. To describe the clinical and electrocardiographic characteristicsin patients with high density right ventricular arrhythmia. Methods. we introduce 32 patients diagnosed with high density ventricular arrhythmia (VPCs ≥ 30/h), originated in the RV andnormal echocardiography. Results. The average age was 56 ± 18 years, hypertension and dyslipidemia were the most common comorbilities, the 85% ofpatients complained about symptoms in the first examination and the majority had been treated with antiarrhythmics drugs. During the ambulatory electrocardiographic monitorization wefound an average of de 9350,09 ± 3455 VPCs/ 24 hours (about a third presented more than 10000 VPCs), the two most common electrocardiographic patterns in VI were QS and rS, with right inferior axis and monophasic R wave in DI. The 45% of patients had been treated with beta-blockers. During the follow up at 6 and 12 months, the patients reported less symptoms des pite they do not receive any treatment, amiodarona was not required. Conclusion. The high-density right ventricular arrhythmias originated in the outflow tract and no associated with structural abnormalities, present a non complicated prognosis, even if they are not medicated.

Rol del ecocardiograma en el estudio de la hipertensión arterial pulmonar / Role of echocardiography in the study of pulmonary arterial hypertension

La presencia de una presión arterial pulmonar media >25 mmHg en reposo y >30 mmHg en ejercicio, con una presión en cuña igual o menor de 15 mmHg y una resistencia vascular pulmonar >3 mmHg /L/ min (Uds. Wood) condiciona una entidad clínica heterogenea denominada hipertensión arterial pulmonar que tiene un amplio rango de causas o enfermedades que llevan a cambios estructurales de las pequeñas arterias pulmonares ocasionando un aumento progresivo de la presión arterial pulmonar y de la resistensia vascular pulmonar, produciendo finalmente una sobrecarga del ventrículo derecho, insuficiencia cardíaca y muerte. Es una entidad clínica con síntomas muy inespecíficos en sus etapas más tempranas y se necesita un buen criterio clínico para llegar a su diagnóstico más rápidamente. En este sentido es que adquiere valor el uso de la ecocardiografía, método que permitirá o solo cuantificar los valores de presión arterial pulmonar, sino también determinar la causa del problema, una adecuada evaluación anatómica y funcional del lecho pulmonar y de las cavidades derechas del corazón, predecir el pronostico de estos pacientes y vigilar el efecto terapéutico de tratamientos muy específicos al ayudar a detectar estados preclínicos de la enfermedad. Revisamos la utilidad y el papel del ecocardiograma en el estudio de la hipertensión arterial pulmonar.

The presence of Medium Arterial Pulmonary Pressure >25 mmHg at rest or >30mmHg during exercise associated with a wedge pulmonary pressure equal or less than 15mmHg, and a Pulmonary Vascular Resistance > 3 mmHg/L/min (Wood units) entails a heterogeneous clinical entity known as Pulmonary Arterial Hipertension which has a very wide spectrum of causes or diseases which produce estructural changes in the walls of the small pulmonary arteries causing a progressive increase of the arterial pulmonary pressure as well as the pulmonary vascular resistence, ultimately producing right ventricular overload, heart failure and death. It is a clinical entitywith very unspecific symptoms at the early stages. Wich makes the physician´s good clinical criteria needed to diagnose itsooner. In this way comes to great value the use of echocardiography, method that would allow not only to measure the arterial pulmonary pressure but would help to carify the cause of the disease, getting an adequate anatomical and funtional evaluation of the pulmonary bed and right heart chambers, predict the outcome in these patients by detecting pre-clinic stages of the disease. We reviewed the utility and role of the Echocardiogram in the study of Pulmonary Arterial Hypertension.

Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares / Sequential segmental analysis in 100 cardiopulmonary specimens

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.

Tetralogía de Fallot, operación de Taussig-Blalock (18 casos operados) / Tetralogy of Fallot: surgical repair using the Taussig-Blalock procedure: revision of an article in this journal in 1948

Tetralogy of Fallot is the most common cause of cyanotic congenital heart disease in adults. This malformation consists of a ture anatomopathologic type represented by the following tetralogy: stenosis of the pulmonary artery... Surgical repair using the Taussig-Blalock procedure. Revision of an article published in this journal in 1948 with a revision of 18 cases