A Truncus Bicaroticus and an Aberrant Right Subclavian Artery. A Rare Case and Literature Review / Un Tronco Bicarótico y una Arteria Subclavia Derecha Aberrante. Un Caso Raro y Revisión de la Literatura

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

Long-term outcomes after endovascular aortic treatment in patients with thoracic aortic diseases / Resultados a longo prazo após tratamento endovascular da aorta em pacientes com doenças da aorta torácica

Abstract Background Endovascular treatments for thoracic aortic diseases have been adopted rapidly, and long-term studies are relevant for durability evaluation. Objective To evaluate the long-term results of a prospective observational study of endovascular treatment in patients with thoracic aortic diseases who underwent percutaneous implantation of self-expandable endoprostheses. Methods Procedural success was defined as the absence of endoleak into the aneurysm or dissection-induced false lumen, no migration, and no conversion to open surgery. Intraoperative, postoperative, and late postoperative outcomes were evaluated in terms of complications, mortality, and evolution of the endoprosthesis over a follow-up of up to 179 months (median: 46 months). Results A total of 150 endoprostheses were implanted in 112 patients. Primary success was observed in 100 (82.14%) patients. Immediate mortality occurred in 7 patients (6.25%). Late mortality occurred in 31 patients (27.68%), 10 (8.93%) of whom died from cardiovascular causes, 12 (10.71%) from non-cardiovascular causes, and 2 (1.78%) from natural causes, while 7 (6.25%) had no diagnosis for cause of death. Types I, II, and IV endoleaks occurred during hospitalization in 4 (3.57%), 5 (4.46%), and 3 (2.68%) patients, respectively. Late types I and IV endoleaks occurred in 5 (4.46%) and 3 (2.68%) patients respectively. Twenty-two patients (19.64%) had clinical complications in the immediate postoperative period. Actuarial survival free from death from cardiovascular causes was 79.3% (95% confidence interval, 67.0-91.7%) at 132 months. Conclusions The low levels of intraoperative and postoperative complications demonstrate that endovascular treatment is safe and effective. The high rate of late survival for these critically ill patients indicates that the endovascular technique is beneficial for treatment of thoracic aortic diseases in terms of long-term outcomes.

Resumo Contexto Os tratamentos endovasculares para doenças da aorta torácica têm sido adotados rapidamente e estudos de longo prazo são relevantes para avaliação de durabilidade. Objetivo Avaliar através de estudo observacional e prospectivo, os resultados a longo prazo do tratamento endovascular em pacientes com doenças da aorta torácica submetidos a implante percutâneo de endoprótese autoexpansível. Métodos O sucesso do procedimento foi definido como ausência de vazamento interno para o aneurisma ou falso lúmen induzido por dissecção, ausência de migração e sem necessidade de conversão para cirurgia aberta. Os resultados intraoperatórios, pós-operatórios e tardios foram avaliados quanto a complicações, mortalidade e evolução da endoprótese em um seguimento de até 179 meses (mediana de 46 meses). Resultados Um total de 150 endopróteses foram implantadas em 112 pacientes. Sucesso primário foi observado em 100 (82,14%) pacientes. Mortalidade imediata ocorreu em sete pacientes (6,25%). A mortalidade tardia ocorreu em 31 pacientes (27,68%), dos quais 10 (8,93%) morreram por causas cardiovasculares; 12 (10,71%), por causas não cardiovasculares; dois (1,78%), por causas naturais e sete (6,25%) não tiveram a causa da morte diagnosticada. Vazamentos tipo I, II e IV ocorreram durante a internação em quatro (3,57%), cinco (4,46%) e três (2,68%) pacientes, respectivamente. Vazamentos tardios tipo I e IV ocorreram em cinco (4,46%) e três (2,68%) pacientes, respectivamente. Complicações clínicas no pós-operatório imediato foram observadas em 19,64% dos pacientes. A sobrevida atuarial por causas cardiovasculares foi de 79,3% em 132 meses. Conclusões Os baixos índices de complicações intra e pós-operatórias demonstram que o tratamento endovascular é seguro e eficaz. A alta taxa de sobrevida em 132 meses em pacientes críticos indica que a técnica é benéfica para o tratamento de doenças da aorta torácica em resultados a longo prazo.

Treatment-refractory heart failure as a manifestation of aortic arch atresia / Falla cardiaca refractaria a tratamiento como manifestación de atresia de arco aórtico

Abstract Background: Distal segment atresia (isthmus) is an extremely rare anatomical variant of obstructive aortic arch anomalies. Case report: We present the case of a newborn who, at 48 hours of life, presented a clinical picture of heart failure. The initial echocardiogram showed a congenital interrupted aortic arch type A, patent ductus arteriosus, and ventricular septal defect. Prostaglandins were initially indicated. Subsequently, a second echocardiogram showed the absence of ductus arteriosus; the CT angiography study confirmed this finding and revealed blood flow to the descending aorta through small intercostal blood vessels. The possibility of atresia of the distal segment (isthmus) of the aortic arch was considered and confirmed at the time of surgery. Conclusions: Aortic atresia should be considered a diagnostic possibility in the presence of type A interrupted aortic arch since the hemodynamic behavior between them is similar. Surgical medical treatment should be individualized since this condition is frequently an emergency in the neonatal period. However, this is not always the case, as other cases have been reported in schoolchildren and adults.

Resumen Introducción: La atresia de segmento distal (istmo) de arco aórtico es una variante anatómica extremadamente rara de las anomalías obstructivas del arco aórtico. Caso clínico: Se presenta el caso de un recién nacido que a las 48 horas de vida presentó un cuadro clínico de insuficiencia cardiaca. El estudio de ecocardiograma inicial mostró una anomalía congénita de interrupción de arco aórtico tipo A, conducto arterioso y comunicación interventricular. De inicio se indicaron prostaglandinas. Posteriormente, el segundo ecocardiograma mostró la ausencia del conducto arterioso; el estudio de angiotomografía confirmó este hallazgo y también reveló flujo sanguíneo hacia aorta descendente a través de pequeños vasos sanguíneos intercostales. Se consideró la posibilidad de atresia del segmento distal (istmo) de arco aórtico y se confirmó al momento del acto quirúrgico. Conclusiones: La atresia aórtica debe ser considerada como posibilidad diagnóstica en presencia de interrupción de arco aórtico tipo A, ya que el comportamiento hemodinámico entre ellos es similar. El tratamiento médico quirúrgico debe individualizarse, ya que es frecuente que sea una urgencia en el periodo neonatal. Sin embargo, no sucede así siempre, ya que se han reportado casos en escolares y adultos.

Serie de casos de arco aórtico derecho: aproximación diagnóstica prenatal / Case series of right aortic arch: prenatal diagnostic approach

RESUMEN El arco aórtico derecho es producto de la involución anormal de los arcos vasculares embrionarios. En los últimos años, el diagnóstico fetal se ha hecho más frecuente con la utilización de la ultrasonografía rutinaria del corazón y los grandes vasos. El hallazgo de un arco aórtico derecho involucra muchos aspectos que pueden afectar el pronóstico del feto; por ello, el estudio exhaustivo debe ser sistematizado. A continuación, presentamos una serie de seis casos de diagnóstico prenatal y sugerimos un algoritmo de evaluación.

ABSTRACT The right aortic arch is a product of abnormal involution of the embryonic vascular arches. In recent years, fetal diagnosis has become more frequent with the use of routine ultrasonography of the heart and great vessels. The finding of a right aortic arch involves many aspects that may affect the prognosis of the fetus; therefore, the exhaustive study must be systematized. Below, we present a series of six cases of prenatal diagnosis and suggest an evaluation algorithm.

Diagnóstico prenatal de arco aórtico derecho fetal con anillo vascular / Prenatal diagnosis of fetal right aortic arch with a vascular ring

Resumen Las anomalías del arco aórtico se refieren a malformaciones congénitas de la posición o patrón de ramificación. Se pueden detectar mediante la visualización dirigida del corte de los tres vasos o de tres vasos y tráquea, con o sin Doppler de color. El diagnóstico prenatal debe conllevar un examen detallado de ultrasonido cardiaco y extra-cardiaco. La identificación de un arco aórtico derecho aislado es crucial para distinguir esta afección del arco aórtico doble. Se describe un caso de diagnóstico prenatal de arco aórtico derecho fetal con anillo vascular a las 29 semanas de gestación, quien fue referido por posible anomalía cardíaca en la ecografía de rutina. El ultrasonido demostró posición anormal de la aorta descendente, situada en el lado derecho de la columna vertebral en el plano tetracameral. La aorta se conectaba con la arteria pulmonar izquierda a través del ductus arterioso, configurando un anillo vascular. La detección prenatal de esta anomalía congénita vascular, ya sea de forma aislada o en asociación con otras malformaciones cardiacas o no cardiacas, podría conducir a un asesoramiento más adecuado.

Abstract Aortic arch anomalies refers to congenital malformations of the position or branching pattern. They can be detected by targeted visualization of the three-vessel slice or three-vessel and trachea, with or without color Doppler. Prenatal diagnosis should involve a detailed cardiac and extra-cardiac ultrasound examination. The identification of an isolated right aortic arch is crucial to distinguish this condition from a double aortic arch. We describe a case of prenatal diagnosis of fetal right aortic arch with vascular ring at 29 weeks gestation who was referred for possible cardiac anomaly on routine ultrasound. Sonography demonstrated abnormal position of the descending aorta, located on the right side of the spine in the tetra chamber plane. The aorta was connected to the left pulmonary artery through the ductus arteriosus, configuring a vascular ring. Prenatal detection of this congenital vascular anomaly, either in isolation or in association with other cardiac or noncardiac malformations, could lead to more appropriate counseling.

Arco aórtico: ramas y variaciones de diámetro interno en cadáveres frescos / Aortic arch: Branches and internal diameter variations in fresh corpses

Resumen Introducción: El conocimiento del patrón de las estructuras que emergen del arco aórtico es importante para las intervenciones en el cuello y la cabeza. Las variantes anatómicas y el área interna de estos vasos son cruciales para el enfoque de terapias endovasculares más seguras. Objetivo: Mostrar las variantes anatómicas y el área de los vasos que tiene dicha variación. Materiales y métodos: Se seleccionaron cuatrocientos cadáveres frescos, del Instituto Nacional de Medicina Legal y Ciencias Forenses de Medellín, con edades comprendidas entre los 18 y 40 años, sin lesiones en el tórax o el cuello, a los cuales se les evaluaron sus arcos aórticos. Todas las variantes se fijaron mediante registro fotográfico y las medidas se tomaron con un calibrador Mitutoyo. Conclusión: Este es el segundo trabajo sobre el tema llevado a cabo en Colombia y el primero en usar cadáveres frescos que se incorporaron en la medida que cumplían los criterios de inclusión.

Abstract Introduction: The knowledge of the pattern of the structures that emerge from the aortic arch is important for neck and head interventions mainly. The presence of anatomical variants and the internal area of these vessels is of great importance for the approach of safer endovascular therapies. Objective: To show the anatomical variants present and the area of the vessels that present such variation. Materials and methods: Four hundred fresh cadavers, aged between 18 and 40 years, without chest or neck injuries, were selected to evaluate their aortic arches, in the National Institute of Legal Medicine and Forensic Sciences of the city of Medellin, Colombia. All the variants were fixed photographically and the measurements taken with Mitutoyo calibrator. Conclusion: This is the second work on the subject carried out in our country and the first one using fresh cadavers and in a random way.

Aberrant right subclavian artery in a dog - case report / [Ectopia de artéria subclávia direita em cão - relato de caso]

The brachiocephalic trunk and the left subclavian artery originate from the aortic arch, and both supply blood to the head, neck, and thoracic limbs. Anatomical variations, such as an aberrant right subclavian artery, are congenital conditions rarely observed in dogs, Thus, the objective of the present report was to describe a case of aberrant right subclavian artery in a 9-year-old Dalmatian. However, this anomaly was a finding in which the patient was asymptomatic during its 9 years of life and only at this age did he exhibit signs including sialorrhea, vomiting, hyporexia, and noisy deglutition. Blood count, biochemical profile, and thoracic radiography led to a diagnosis of megaesophagus and aspiration pneumonia. Despite the recommended treatment, the patient did not respond well; as such, the owner elected to euthanize the animal. On necropsy, the right subclavian artery originated directly from the aortic arch, followed a route from left to right dorsally to the esophagus, and then formed an impression of the vascular path over the muscular wall of the esophagus. The esophagus, in turn, exhibited a flaccid wall and dilation in the caudal portion to the vascular path made by the ectopic position of the right subclavian artery.(AU)

O tronco braquiocefálico e a artéria subclávia esquerda emergem do arco aórtico e são responsáveis por fazerem o suprimento sanguíneo para cabeça, pescoço e membros torácicos. Variações anatômicas, como a ectopia da artéria subclávia direita, são alterações congênitas raramente encontradas em cães, cujas alterações do sistema digestivo acontecem em pacientes recém-desmamados e não em adultos. Assim, o objetivo deste relato é descrever um caso de ectopia da artéria subclávia direita em uma cadela, Dálmata, de nove anos de idade. No entanto, essa anomalia foi um achado do qual o paciente foi assintomático durante os nove anos de vida e somente com essa idade apresentou sinais como sialorreia, vômito, hiporexia e deglutição ruidosa. O hemograma e os perfis bioquímicos, associados à radiografia torácica, levaram a um diagnóstico de megaesôfago e pneumonia aspirativa. Mesmo seguindo o tratamento recomendado, houve piora clínica do quadro e o animal foi submetido à eutanásia. À macroscopia, a artéria subclávia direita originava-se direto do arco aórtico, fazia um percurso da esquerda para a direita dorsalmente ao esôfago e, então, formava uma impressão do trajeto vascular sobre a parede muscular do esôfago. O esôfago, por sua vez, apresentava parede flácida e dilatação na porção caudal ao trajeto vascular feito pela posição ectópica da artéria subclávia direita.(AU)

Impresión 3D de rara patología congénita de aorta y vasos supraaórticos / 3-Dimensional impression of a rare congenital disease of aortic and supra-aortic vessels

Resumen El divertículo de Kommerell es una anomalía de muy baja incidencia y prevalencia en la edad pediátrica. Se reporta el caso de un paciente de 11 años de edad, con diagnóstico de divertículo de Kommerell con arco aórtico derecho y origen anómalo de arteria subclavia izquierda desde la rama pulmonar izquierda a través de conducto arterioso persistente. Dado que es una anomalía cardiovascular compleja se decidió realizar un modelo impreso en 3 D, el cual proporcionó una mejor comprensión de su distribución espacial, tamaño y forma real, como si fuera una pieza de anatomía patológica. Este modelo ayudó en la toma de decisiones, planificación y seguridad de la ejecución de una posible cirugía cardiaca. Este es el primer reporte de caso de este tipo de anomalía, así como el primer prototipo cardíaco impreso en modo tridimensional realizado en Perú para el tratamiento de la misma.

Abstract Kommerell's diverticulum is an anomaly of very low incidence and prevalence in paediatrics. A case is presented of an 11 year-old patient with a diagnosis of Kommerell's diverticulum with a right aortic arch and a left subclavian artery of anomalous origin from the pulmonary branch through a patent ductus arteriosus. Given that this was a complex cardiovascular anomaly, it was decided to make a print model in 3-D. This provided a better understanding of its spatial distribution, size, and real shape, as it was a histopathology piece. This model helped in taking planning and safety decisions on any possible cardiac surgery. This is the first report of a case of this type of anomaly, as well as the first prototype of a cardiac print in 3-dimensional mode, performed in Peru.

Anillos vasculares: Serie de 28 pacientes atendidos en un Servicio de Endoscopía Respiratoria Pediátrica / Vascular rings: Series of 28 patients of a Pediatrie Respiratory Endoscopy Department

Los anillos vasculares tienen una incidencia del 1 % entre las enfermedades cardiovasculares congénitas. Constituyen una malformación embriológica en la que el arco aórtico, sus ramas o las arterias pulmonares provocan presión sobre la tráquea y/o el esófago. Anatómicamente, se dividen en dos grupos, dependiendo de cómo rodean la tráquea y el esófago: completo o incompleto. La sintomatología suele iniciarse en los primeros meses de vida con estridor bifásico o espiratorio que aumenta con el llanto y la alimentación, tos traqueal de tonalidad metálica, infecciones respiratorias recurrentes, episodios de apnea refleja y cianosis, sibilancias, hiperextensión cervical, retracción esternal e intercostal, y dificultad en la alimentación. Se realizó un análisis retrospectivo de 28 pacientes con diagnóstico de anillo vascular que concurrieron a la consulta en el Servicio de Endoscopía Respiratoria del Hospital Garrahan entre enero de 2015 y septiembre de 2017.

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented.

Anillo vascular completo por doble arco aórtico simétrico, una malformación cardiovascular infrecuente / Complete vascular ring by double symmetrical aortic arch, an infrequent cardiovascular malformation

Resumen Introducción: El doble arco aórtico (DAA) es una malformación cardiovascular infrecuente caracterizada por la persistencia de ambos arcos aórticos posterior al nacimiento. Puede presentarse con rama derecha dominante (70-73%), izquierda o simétrica. Su forma anatómica produce compresión de estructuras mediastínicas como la tráquea y esófago. Caso clínico: Lactante menor hombre de 3 meses, con antecedente de estridor desde nacimiento. Ingresó a nuestro centro por neumonía grave con necesidad de ventilación mecánica prolongada. Evaluado por otorrinolaringología, se realizó revisión de vía aérea, observando estenosis en los últimos 5 anillos traqueales con colapso dinámico de la tráquea. La tomografía computada de tórax demostró DAA completo con emergencias de troncos supraaórticos de ambos arcos de forma simétrica. Se decidió tratamiento quirúrgico a la brevedad. Se abordó por toracotomía anterolateral izquierda, se realizó control vascular y sección del arco aórtico izquierdo distal a emergencia de subclavia, con plastía de aorta y arteria subclavia izquierda, liberado el arco aórtico se realizó pexia y disección de tejido fibrótico que rodeaba tráquea y esófago. Paciente evolucionó favorablemente, con resolución de cuadro respiratorio y ausencia estridor. Fibrobroncoscopía posoperatoria no observó compresión traqueal. El alta hospitalaria fue al 14° día posoperatorio. Actualmente, a seis meses de seguimiento, se encuentra asintomático respiratorio y cardiovascular. Discusión: El DAA puede afectar al 0,03% de la población pediátrica. Usualmente es sintomático con manifestaciones obstructivas como estridor o disfagia por compresión de estructuras mediastínicas, por lo que requiere alta sospecha clínica para su confirmación imagenológica y posterior tratamiento quirúrgico.

Introduction: The double aortic arch (DAA) is an uncommon cardiovascular malformation, characterized by the persistence of both aortic arches after birth. It can be presented with right dominant branch (70-73%), left or symmetrical. It's anatomical shape produces compression of mediastinal structures such as the trachea and esophagus. Clinic case: Infant man 3 months old, with a history of stridor since birth. He was admitted to our center due to severe pneumonia with the need for prolonged mechanical ventilation. Evaluated by otorhinolaryngology, who performed airway revision observing stenosis in the last 5 tracheal rings with dynamic trachea collapse. Chest computed tomography showed complete DAA with emergence of supraaortic trunks of both arches symmetrically. Surgical treatment was decided as soon as possible. It was approached by left anterolateral thoracotomy, vascular control and left aortic arch section distal to subclavian emergency was performed, with aortic and left subclavian artery plasty, aortic arch was released to perform pexia and dissection of fibrotic tissue surrounding the trachea and esophagus. Patient evolved favorably, with resolution of respiratory symptoms and absence of stridor. Postoperative fiberoptic bronchoscopy did not observe tracheal compression. He was discharged on the 14th postoperative day. Currently at six months of follow-up, he is asymptomatic respiratory and cardiovascular. Discussion: AAD can affect 0.03% of the pediatric population. It is usually symptomatic with obstructive manifestations such as stridor or dysphagia due to compression of mediastinal structures, which requires high clinical suspicion for its imaging confirmation and subsequent surgical treatment.

Alterações congênitas do coração e dos grandes vasos em cães / Congenital abnormalities of the heart and large vessels of dogs

As alterações congênitas do coração e dos grandes vasos (ACCGV) são definidas como defeitos morfológicos associados ao nascimento e são as anomalias congênitas mais frequentes dos animais domésticos. O objetivo deste trabalho foi determinar a frequência de diagnósticos de ACCGV em cães no Rio Grande do Sul, com ênfase na Região Metropolitana de Porto Alegre, no período de janeiro de 2000 a dezembro de 2016. Do total de 7.903 necropsias de cães, 27 morreram espontaneamente ou foram submetidos à eutanásia devido às ACCGV, representando 0,3% dos casos. Em 11,1% dos cães apresentaram dois distúrbios congênitos no coração ou nos grandes vasos, totalizando 30 alterações. A idade variou de um dia a 12 anos, com a idade mediana de quatro meses. Em 81,5% acometeu cães com raça, e 18,5% sem raça definida. Em relação ao sexo, 51,8% dos cães eram machos e 48,2%, fêmeas. Estenose subaórtica foi a alteração mais frequente, seguido por defeito do septo atrial, persistência do arco aórtico direito, persistência do ducto arterioso, estenose pulmonar e defeito do septo interventricular, e fibroelastose endocárdica. Dos casos múltiplos, as combinações encontradas foram: persistência do ducto arterioso associado com defeito do septo atrial, estenose subaórtica com defeito do septo interventricular, e defeito do septo atrial e ventricular.(AU)

Congenital alterations of the heart and large vessels (CAHLV) are defined as morphological defects associated with birth and are the most frequent congenital anomalies of domestic animals. The aim of this study was to determine the frequency of CAHLV in dogs in Rio Grande do Sul, with emphasis in the Metropolitan Region of Porto Alegre, from January 2000 to December 2016. Of the 7,903 necropsied dogs, 27 died spontaneously or were submitted to euthanasia due to CAHLV, representing 0.3% of the cases. In 11.1% of the dogs, there were two congenital disorders in the heart or in the great vessels, totaling 30 changes. The age of affected dogs ranged from one day to 12 years, with the median age of four months. In 81.5% it affected dogs with breed, and 18.5%, without breed defined. In 51.8% were males, and 48.2%, females. Subaortic stenosis was the most frequent alteration, followed by atrial septal defect, persistent right aortic arch, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect, and endocardial fibroelastosis. Of the multiple cases, the combinations found were: patent ductus arteriosus associated with atrial septal defect, subaortic stenosis with ventricular septal defect, and atrial and ventricular septal defect.(AU)

Reemplazo de arco aórtico y aorta descendente proximal por aneurisma o disección aórtica con prótesis Thoraflex / Aortic arch and proximal descending aorta replacement with the Thoraflex* prosthesis

Resumen: El compromiso simultáneo del arco aórtico y aorta descendente proximal, ya sea por disección o aterosclerosis, constituye uno de los mayores desafíos que puede enfrentar un cirujano cardiovascular. La prótesis híbrida Thoraflex, introducida en los últimos años, ha resultado ser una importante ayuda para el tratamiento quirúrgico de esta compleja y grave patología. Esta consiste en un tubo protésico de Dacron con 4 ramas, para el reemplazo del arco aórtico y sus troncos braquiocefálicos y perfusión corporal distal, y una endoprótesis que queda como "trompa de elefante suspendida" en la aorta descendente proximal. Presentamos en esta oportunidad nuestra experiencia inicial en 4 pacientes, 3 con disección aórtica crónica y una con un aneurisma aterosclerótico, usando la prótesis híbrida Thoraflex.

Abstract: Atherosclerotic aneurysm or dissection of the aortic arch and proximal descending thoracic aorta is one of the major challenges for a cardiovascular surgeon. The new hybrid prosthesis Thoraflex has become an important devise to simplify the surgical treatment of this very complex and technically demanding aortic pathology. This hybrid prosthesis consists of a 4-branched arch graft with a stent-graft at the distal end. The proximal part is a gelatin-coated woven polyester prosthesis. The stented section is a self-expanding endoprosthesis constructed of thin-walled polyester and nitinol ring stents that is left in the proximal descending aorta as a "frozen elephant trunk". We present our initial experience with the Thoraflex prosthesis in four patients, three of them with chronic aortic dissection and one with an atherosclerotic aneurysm.

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Tracheal compression by aberrant brachiocephalic trunk accompanying variant origin of the vertebral artery / Compresión traqueal por un tronco braquiocefálico aberrante acompañado de una variación en el origen de la arteria vertebral

The aorta is the main arterial trunk in the human body, however, its variation was extremely variable. During an educational dissection, aberrant branching pattern of aortic arch was found in a Korean cadaver. The brachiocephalic trunk (innominate artery) originated from the aortic arch at the left side of the trachea. It crossed the trachea and divided into the right common carotid and subclavian arteries. The left vertebral artery originated from the aortic arch between the origins of the left common carotid artery and the left subclavian artery, then the left vertebral artery coursed upward to the transverse foramen of the C7. The author describes this previously novel case report with aberrant brachiocephalic trunk and left vertebral arteries and discusses the clinical implications of such a variant.

La aorta es el tronco arterial principal del cuerpo humano, sin embargo, su variación es extremadamente variable. Durante una disección de rutina de un cadáver coreano, se encontró un patrón de ramificación aberrante del arco aórtico. El tronco braquiocefálico se originaba del arco aórtico en el lado izquierdo de la tráquea. Cruzaba la tráquea y se dividía en las arterias carótida común y subclavia derecha. La arteria vertebral izquierda se originó a partir del arco aórtico entre los orígenes de la arteria carótida común izquierda y la arteria subclavia izquierda, luego la arteria vertebral izquierda ascendió hacia el foramen transversal de la séptima vértebra cervical. Se describe un caso de tronco braquiocefálico aberrante y la correspondiente arteria vertebral izquierda y se discuten las implicaciones clínicas de tal variante.

Síndrome de Turner en una adolescente / Turner syndrome in an adolescent

Se presenta el caso clínico de una adolescente ecuatoriana de 17 años de edad, blanca, quien nació producto de un embarazo normal y parto eutócico. Posterior al nacimiento se le realizó estudio genético que mostró la presencia de un cariotipo X0, por lo cual se le diagnosticó síndrome de Turner. A los 3 años fue operada de hipoplasia del arco aórtico con una evolución favorable. En la actualidad la paciente tiene baja talla con orejas de baja implantación, así como implantación alta del cabello en la nuca, tórax plano en forma de escudo, escaso bello pubiano y ausencia de menstruación

The case report of a 17 years Ecuadorian white adolescent is presented who was born from a normal pregnancy and eutocic delivery. After birth, a genetic study that showed the presence of a X0 Karyotype was carried out, reason why she was diagnosed Turner syndrome. She was operated of hypoplastic aortic arch when she was 3 years with a favorable clinical course. At the present time the patient has short height with ears of low implantation, as well as high implantation of the hair in the back of the neck, flat thorax in shield form, scarce pubic hair and amenorrhea

Right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum / Arco aórtico à direita associado a artéria inominada esquerda aberrante originada de divertículo de Kommerell

Abstract We report a case of an uncommon thoracic aorta anomaly-right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum-that went undiagnosed until adulthood.

Resumo É apresentado o caso de uma rara anomalia da aorta torácica - arco aórtico à direita associado a artéria inominada esquerda aberrante com origem em divertículo de Kommerell -, com diagnóstico apenas na vida adulta.

Ramos colaterais do arco aórtico e suas principais ramificações no cachorro-do-mato (Cerdocyon thous) / Collateral branches of the aortic arch and its main rami in crab-eating fox (Cerdocyon thous)

O cachorro-do-mato (Cerdocyon thous) pertence à família Canidae, encontra-se amplamente distribuído pela América do Sul. Devido à escassez literária a respeito da morfologia desta espécie objetivamos colaborar com o conhecimento da anatomia do arco aórtico e seus ramos colaterais. Foram estudados quatro animais, sendo 2 machos e 2 fêmeas oriundos da Mina de Bauxita, Paragominas, Pará, Brasil/Terra Ltda, sob autorização da SEMA-PA nº 455/2009, que morreram por causas naturais. O sistema arterial foi preenchido com látex corado em vermelho e fixado em solução aquosa de formoldeído 10%. O arco aórtico de Cerdocyon thous apresentou em sua origem apenas dois vasos, o tronco braquiocefálico e a artéria subclávia esquerda. Do tronco braquiocefálico originou-se o tronco bicarotídeo, que se bifurcou nas artérias carótidas comum direita e esquerda, e artéria subclávia direita. Das artérias subclávias ocorreu a formação do tronco costocervical, artéria vertebral, artéria torácica interna, artéria cervical superficial e ao atingir o espaço axilar passa a ser chamada de artéria axilar em ambos os antímeros. Concluímos que o arco aórtico de Cerdocyon thous é similar ao de outros animais domésticos com relação ao número de ramificações em sua origem.(AU)

Crab-eating fox (Cerdocyon thous) belongs to the Canidae family distributed in South America. Due to literary scarcity regarding the morphology of this species, we objectifiked to collaborate with description of the anatomy of the aortic arch and its collateral branches. We studied four foxes, 2 males and 2 females, from Mina Bauxita, Paragominas, Pará, Brazil/Terra Ltda, under authorization of SEMA-PA nº 455/2009, that died of natural causes. The arterial system was filled with red latex and fixation with 10% formaldehyde solution. The aortic arch of Cerdocyon thous showed that in its origin are two vessels, brachiocephalic trunk and left subclavian artery. From brachiocephalic trunk originates the bicarotid trunk, bifurcated into left and right carotid common arteries, and right subclavian artery. The subclavian arteries originates the costocervical trunk, vertebral artery, internal thoracic artery, superficial cervical artery, to reach the axillary space, and passes to be called axillary artery on both sides. We conclude that the aortic arch of Cerdocyon thous is similar to the one in other domestic animals regarding the number of ramificastions at its origin.(AU)

The arteries root from the aortic arch in the sparrowhawk (Accipiter nisus, Falconiformes: Accipitridae) / La raíz de las arterias desde el arco aórtico en el gavilán (Accipiter nisus, Falconiformes: Accipitridae)

This study was aimed at determining the morphology of the aortic arch in the sparrowhawk. For this purpose, arteries near the heart of six sparrowhawks were assessed. Latex injection method was applied to the three materials and barium sulphate solution was injected into the aorta for angiography in three other materials. It was observed that two major arteries arose from aortic arch in the sparrowhawk: the left brachiocephalic trunk and the right brachiocephalic trunk. These trunks were contiguous arteries but separately originated from the aorta. The brachiocephalic trunks were divided into the common carotid and subclavian arteries after their origins. First, the common carotid arteries are given off by the brachiocephalic trunks. The common carotid artery was giving off esophagotracheobronchial artery and vertebral trunk. Vertebral trunk was locating under the brachial plexus. The subclavian artery was continuations of the brachiocephalic trunk and it was bifurcating to the axillar artery and the pectoral trunk just from its own beginning. The axillary artery passed the brachial plexus crosswise from above, and reached to the wing. The sternoclavicular artery stemmed from ventral aspect of the begining of the axillary artery. The thickest branch of the subclavian artery was the pectoral trunk, which was branched the cranial external thoracic artery, the caudal external thoracic artery, the dorsal thoracic artery, and the internal thoracic artery. It is hoped that the results of this morphological study will contribute to the species specific anatomical data in the birds.

El objetivo fue determinar la morfología del arco aórtico en el gavilán. Para este fin, se evaluaron las arterias próximas al corazón de seis gavilanes. En tres aves se inyectó una solución de látex, mientras que en las tres restantes se inyectó a través de la aorta sulfato de bario para someterlas luego a un estudio por angiografía. Se observó el origen de dos arterias principales del arco aórtico del gavilán: el tronco braquiocefálico izquierdo y el tronco braquiocefálico derecho. Estos troncos se presentaron como arterias contiguas, pero que se originaron por separado de la aorta. Los troncos supraaórticos se dividieron después de su origen en las arterias carótida común y subclavia. Las arterias carótidas comunes se emiten a partir de los troncos supraaórticos. La arteria carótida común emitió una arteria esófago-traqueobronquial y el tronco vertebral. El tronco vertebral se localizó por debajo del plexo braquial. La arteria subclavia se continuó a partir del tronco braquiocefálico y se bifurcó en arteria axilar y tronco de las arterias pectorales justo desde su propio inicio. La arteria axilar cruzó el plexo braquial transversalmente desde arriba, y llegó al ala. La arteria esternoclavicular derivó de la cara ventral del inicio de la arteria axilar. La rama más gruesa de la arteria subclavia fue el tronco de las arterias pectorales, que se ramificó en arteria torácica externa craneal, arteria torácica externa caudal, arteria torácica dorsal, y la arteria torácica interna. Se espera que los resultados de este estudio morfológico sean una contribución para aportar datos anatómicos específicos en esta especie de aves.

Doble arco aórtico. Reporte y análisis de caso en un paciente colombiano / Double aortic arch. Report and analysis of a case in a Colombian patient

El doble arco aórtico es una malformación cardiovascular congénita poco frecuente, que consiste en la presencia de dos arcos aórticos formando un anillo vascular completo que puede comprimir la tráquea y/o el esófago. En este estudio se presenta el caso de un paciente colombiano, quien ingresó al servicio de urgencias con broncoespasmo y estridor laríngeo, tanto inspiratorio como espiratorio. La angiotomografía computarizada de tórax mostró un doble arco aórtico completo. En el análisis del caso se presenta la revisión epidemiológica, embrionaria, genética, fisiopatológica y clínica de esta malformación. Finalmente se muestra cómo el análisis integral desde las ciencias básicas permite guiar el proceso diagnóstico y terapéutico.

The double aortic arch is a rare congenital cardiovascular malformation, in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. The case of a Colombian patient is presented, who arrived to the emergency room with bronchospasm and laryngeal stridor, inspiratory as well as expiratory. The computed tomography angiography showed a complete double aortic arch. In the analysis of this case the epidemiologic, embryonic, genetic, pathophysiologic and clinical review of this malformation is presented. Finally, how the integral analysis from the basics sciences can guide the diagnostic and therapeutic process is shown.

Ramos colaterais do Arco aórtico do preá (Galea spixii Wagler, 1831) / Collateral branches of the aortic arch of galea (Galea spixii Wagler, 1831)

O preá é um roedor típico da caatinga pertencente à família Caviidae. Considerando a inexistência de dados sobre o arco aórtico do preá, foi realizado este estudo tendo como objetivo descrever os ramos colaterais do arco aórtico neste cavídeo, e dessa forma, contribuir com dados para biologia da espécie. Foram utilizados vinte preás machos provenientes de estudos anteriores e encontravam-se armazenados em freezer no Centro de Multiplicação de Animais Silvestres (CEMAS/UFERSA). Os animais foram descongelados, a cavidade torácica foi aberta, a aorta canulada e o sistema vascular lavado com solução salina e em seguida, injetado látex Neoprene corado com pigmento vermelho, amarelo ou branco. Posteriormente, os animais foram fixados em formol e depois de 72 horas, dissecados e analisados, sendo obtidos desenhos esquemáticos e os exemplares mais representativos fotografados. O arco aórtico do preá emitiu como ramos colaterais, o tronco braquiocefálico e a artéria subclávia esquerda. O tronco braquiocefálico originou na maioria das peças estudadas, a artéria carótida comum esquerda e o tronco braquiocarotídeo, do qual surgem as artérias subclávia direita e carótida comum direita. As artérias subclávias direita e esquerda em todos os animais estudados emitiram a artéria vertebral, a artéria torácica interna, a artéria cervical superficial, o tronco costocervical e a artéria axilar. O padrão da formação do arco aórtico do preá assemelhou-se ao observado em outros roedores, tais como no mocó, no porquinho-da-índia e na chinchila...

Galea spixii is a typical rodent of the caatinga belonging to the Caviidae family. Considering the lack of data on the aortic arch of the galea, this study aimed to describe the collateral branches of the aortic arch in this specie and to contributes to knowledge of its biology. Twenty males from previous studies were used which had been stored in a freezer at Multiplication Center of Wild Animals (CEMAS/UFERSA). The animals were thawed, the thoracic cavity was opened and the aorta cannulated vasculature was washed with saline and then injected with Neoprene latex colored with red pigment, yellow or white. Subsequently, the animals were fixed in formalin and after 72 hours dissected and analyzed, to obtain schematic drawings and the most representative samples were photographed. The cavy aortic arch issued as collateral branches, the brachiocephalic trunk and the left subclavian artery. The brachiocephalic trunk afforded in most of the specimens the left common carotid artery and the brachiocarotid trunk, where the right subclavian and right common carotid arteries arise. The right and left subclavian arteries in all animals studied issued the vertebral artery, internal thoracic artery, superficial cervical artery costocervical trunk and axillary artery. The pattern of formation of the aortic arch of galea was similar to that observed in other rodents, such as the kerodon, the guinea pig and chinchilla....