Successful endovascular treatment of aortic arch aneurysm in a pediatric patient.

BACKGROUND: Ascending aortic aneurysms are rare pathologies in childhood, especially in the absence of previous diseases such as Marfan syndrome. OBJECTIVE: Present the possibility of successful endovascular management of large vessel aneurysms, using stents and microcatheters with embolization of the aneurysm sac. METHOD: We present the case of a previously healthy ten-year-old patient, in whom a pseudoaneurysm was documented between the origin of the left common carotid artery and left subclavian artery, successfully managed endovascularly, initially with a stent covering the neck of the aneurysm to remodel it and later with embolization of the aneurysm sac using a microcatheter. RESULTS: Aneurysms of large vessels, such common carotid artery and subclavian artery, are at risk of rupture with devastating complications; endovascular management is considered a minimally invasive management option, with favorable results. CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.

ANTECEDENTES: Los aneurismas de la aorta ascendente son patologías poco frecuentes en la infancia, sobre todo en ausencia de enfermedades previas como el síndrome de Marfan. OBJETIVO: Dar a conocer la posibilidad del manejo endovascular exitoso de los aneurismas de grandes vasos, usando stent y micro catéter con embolización del saco aneurismático. MÉTODO: Presentamos el caso de una paciente de 10 años y 2 meses, previamente sana, en quien se documentó un pseudoaneurisma entre el origen de la arteria carótida común izquierda y la arteria subclavia izquierda, que logró manejarse de forma endovascular, inicialmente con un stent cubriendo el cuello del aneurisma con el fin de remodelarlo y posteriormente por medio de microcatéter se realizó embolización del saco del aneurisma con coils, con resultado exitoso. RESULTADOS: Los aneurismas de los grandes vasos, como la arteria carótida común y la arteria subclavia, tienen riesgo de ruptura con complicaciones devastadoras; el manejo endovascular se plantea como una opción poco invasiva de manejo, con resultados favorables. CONCLUSIÓN: El manejo de aneurismas de grandes vasos, por vía endovascular usando stent y microcatéter con embolización del saco aneurismático, es una opción novedosa de manejo que logra resultados exitosos.

Anesthetic management of a child with Loeys-Dietz syndrome undergoing complete aortic arch replacement.

Loeys-Dietz syndrome (LDS) is a connective tissue disease related to ß-transforming growth factor mutations, which causes aneurysms formation, vascular tortuosity and skeletal manifestations. The prognosis is very poor, and mortality occurs at the age of 27 in patients without surgical treatment. Despite being diagnosed in childhood, is not usual surgical aortic replacement in children. We report a case of 12 years old child with LDS and multiple aneurysms in thoracic aorta, undergoing complete aortic arch replacement and our proposal for the anesthetic management, due to surgical complexity and implications in pediatric population.

El síndrome de Loeys-Dietz (SDL) es una enfermedad del tejido conectivo debida a mutaciones del factor de crecimiento transformador beta que provocan formación de aneurismas, malformaciones vasculares y esqueléticas. Tiene mal pronóstico y el fallecimiento sobreviene de media a los 27 años sin tratamiento quirúrgico. A pesar de diagnosticarse en la infancia, es infrecuente la cirugía en niños. Presentamos el caso de una niña de 12 años con SDL y aneurisma múltiple en aorta torácica, programada para recambio completo de arco aórtico, proponiendo estrategias para el manejo anestésico, dada la complejidad y las implicaciones de esta cirugía en la población pediátrica.

Treatment-refractory heart failure as a manifestation of aortic arch atresia.

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.

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.

Tratamiento endovascular exitoso de aneurisma del arco aórtico en un paciente pediátrico / Successful endovascular treatment of aortic arch aneurysm in a pediatric patient

Resumen Antecedentes: Los aneurismas de la aorta ascendente son patologías poco frecuentes en la infancia, sobre todo en ausencia de enfermedades previas como el síndrome de Marfan. Objetivo: Dar a conocer la posibilidad del manejo endovascular exitoso de los aneurismas de grandes vasos, usando stent y micro catéter con embolización del saco aneurismático. Método: Presentamos el caso de una paciente de 10 años y 2 meses, previamente sana, en quien se documentó un pseudoaneurisma entre el origen de la arteria carótida común izquierda y la arteria subclavia izquierda, que logró manejarse de forma endovascular, inicialmente con un stent cubriendo el cuello del aneurisma con el fin de remodelarlo y posteriormente por medio de microcatéter se realizó embolización del saco del aneurisma con coils, con resultado exitoso. Resultados: Los aneurismas de los grandes vasos, como la arteria carótida común y la arteria subclavia, tienen riesgo de ruptura con complicaciones devastadoras; el manejo endovascular se plantea como una opción poco invasiva de manejo, con resultados favorables. Conclusión: El manejo de aneurismas de grandes vasos, por vía endovascular usando stent y microcatéter con embolización del saco aneurismático, es una opción novedosa de manejo que logra resultados exitosos.

Abstract Background: Ascending aortic aneurysms are rare pathologies in childhood, especially in the absence of previous diseases such as Marfan syndrome. Objective: Present the possibility of successful endovascular management of large vessel aneurysms, using stents and microcatheters with embolization of the aneurysm sac. Method: We present the case of a previously healthy ten-year-old patient, in whom a pseudoaneurysm was documented between the origin of the left common carotid artery and left subclavian artery, successfully managed endovascularly, initially with a stent covering the neck of the aneurysm to remodel it and later with embolization of the aneurysm sac using a microcatheter. Results: Aneurysms of large vessels, such common carotid artery and subclavian artery, are at risk of rupture with devastating complications; endovascular management is considered a minimally invasive management option, with favorable results. Conclusion: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.

Artéria vertebral esquerda anômala ­ relato de caso / Anomalous left vertebral artery ­ case report

Introdução: Os procedimentos cirúrgicos e radiológicos das regiões do tórax e pescoço são frequentes e a identificação precoce de variantes do arco da aorta e especificamente da artéria vertebral ajudam a identificar possíveis obstáculos nesses procedimentos, antes que se tornem um problema. Objetivo: Relatar um caso de variação da artéria vertebral esquerda. Método: Estudo descritivo, retrospectivo de um relato de caso sobre artéria vertebral anômala identificada por meio de uma dissecção de rotina. Resultados: Durante o procedimento se observou que o arco da artéria aorta apresentava um ramo arterial emergindo entre a artéria carótida comum esquerda e a artéria subclávia esquerda. Posteriormente, avançando a dissecção para a região cervical, identificou-se que o ramo variável do arco aórtico se tratava da artéria vertebral esquerda, confirmando seu trajeto pelo forame do processo transverso das vértebras cervicais. Conclusão: Dessa forma, torna-se importante apresentar o presente relato de caso da artéria vertebral anômala, bem como discutir as possíveis implicações cirúrgicas, hemodinâmicas e radiológicas do achado. É fundamental o conhecimento das variações anatômicas da artéria vertebral, as quais, possuem relevância cirúrgica, radiológica e hemodinâmica, já que processos patológicos podem ser observados ou negligenciados por métodos de imagem. Assim, podem-se evitar eventuais lesões iatrogênicas baseadas no conhecimento prévio dessa variação

Introduction: Surgical and radiological procedures of the thorax and neck regions are frequent and early identification of variants of the aortic arch and specifically the vertebral artery help to identify possible obstacles in these procedures, before they become a problem. Objective: To report a case of variation of the left vertebral artery. Methods: Descriptive, retrospective study of a case report on anomalous vertebral artery identified by routine dissection. Results: During the procedure it was observed that the arch of the aortic artery had an arterial branch emerging between the left common carotid artery and the left subclavian artery. Subsequently, advancing the dissection to the cervical region, it was identified that the variable branch of the aortic arch was the left vertebral artery, confirming its path through the foramen of the transverse process of the cervical vertebrae. Conclusion: Thus, it is important to present the present case report of the anomalous vertebral artery, as well as to discuss the possible surgical, hemodynamic and radiological implications of the finding. It is essential to know the anatomical variations of the vertebral artery, which have surgical, radiological and hemodynamic relevance, since pathological processes can be observed or neglected by imaging methods. Thus, one can avoid eventual iatrogenic lesions based on prior knowledge of this variation.

Introducción: Los procedimientos quirúrgicos y radiológicos en las regiones de tórax y cuello son frecuentes y la identificación temprana de variantes del arco aórtico y específicamente de la arteria vertebral ayuda a identificar posibles obstáculos en estos procedimientos, antes de que se conviertan en un problema. Objetivo: Reportar un caso de variación de la arteria vertebral izquierda. Métodos: Estudio descriptivo retrospectivo de un reporte de caso sobre una arteria vertebral anómala identificada a través de una disección de rutina. Resultados: Durante el procedimiento se observó que el arco aórtico presentaba una rama arterial que emergía entre la arteria carótida común izquierda y la arteria subclavia izquierda. Posteriormente, avanzando la disección a la región cervical, se identificó que la rama variable del arco aórtico era la arteria vertebral izquierda, confirmándose su trayecto por el foramen de la apófisis transversa de las vértebras cervicales. Conclusión: Por lo tanto, es importante presentar el presente caso clínico de arteria vertebral anómala, así como discutir las posibles implicaciones quirúrgicas, hemodinámicas y radiológicas del hallazgo. Es fundamental conocer las variaciones anatómicas de la arteria vertebral, las cuales tienen relevancia quirúrgica, radiológica y hemodinámica, ya que los procesos patológicos pueden ser observados o despreciados por métodos de imagen. Así, se pueden evitar posibles lesiones iatrogénicas en base al conocimiento previo de esta variación

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.

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.

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.

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

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.

Tratamiento endovascular del arco aórtico asegurando la salida de los troncos supraaórticos mediante fenestración in situ / Endovascular treatment of the aortic arch securing the supraaortic trunks through in-situ fenestration

El tratamiento endovascular para las lesiones del arco aórtico es cada vez más frecuente gracias al empleo de endoprótesis fenestradas. En situaciones de urgencia no siempre es posible la disponibilidad de este tipo de prótesis. Presentamos el caso de un paciente con infección reciente por COVID-19 y pseudoaneurisma del arco aórtico sintomático tratado mediante endoprótesis torácica con fenestración manual y guías precargadas.(AU)

The use of endovascular repair for aortic arch lesions is increasing thanks to fenestrated endoprothesis. In emergency situations the availability of these endografts is not always possible. We present the case of a patient with recent COVID-19 infection and a symptomatic aortic arch pseudoaneurysm treated with a physician-made fenestrated endograft and externalized guidewires.(AU)

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.

Insuficiencia respiratoria aguda como forma de debut de una anomalía congénita (doble arco aórtico) / Acute respiratory failure as a form of debut of a congenital anomaly (double aortic arch)

El doble arco aórtico es una anomalía vascular poco frecuente que provoca compresión traqueal y esofágica, habitualmente en losprimeros meses de vida. La estenosis traqueal asociada con el doble arco aórtico conlleva un alto riesgo de morbilidad, mortalidady reestenosis. Presentamos el caso de un paciente en que su anomalía no fue diagnosticada, y permaneció en silencio hasta la edad adulta cuando comenzó a padecer clínica compresiva. (AU)

The double aortic arch is a rare vascular anomaly that usually causes tracheal and esophageal compression in the first months oflife. Tracheal stenosis associated with double aortic arch carries a high risk of morbidity, mortality, and restenosis. We present thecase of a patient in whom his anomaly was not diagnosed, and he remained silent until adulthood when he began to suffer from compression symptoms. (AU)

Right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum.

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.

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.

Endocardial left ventricular pacing after accidental aortic wall perforation.

Inadvertent endocardial placement of a pacing lead in the left ventricle through the aortic valve is a rare complication with an unknown incidence because of inadequate reporting. Reported cases are usually the result of lead insertion via the subclavian artery. A possible but very unusual situation is endocardial lead insertion in the left ventricle after aortic arch perforation. We report the case of a 72-year-old woman in whom a screw-in pacing lead accidentally perforated the aortic arch and continued its way through the ascending aorta, aortic valve and left ventricle, after insertion through the left subclavian vein. We describe how this complication was diagnosed, the predisposing factors, the risks it carries and the ways in which devastating consequences have so far been avoided, as the patient refused any surgical intervention including lead removal.

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.

[Persistent fifth aortic arch with patent ductus arteriosus]. / Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding occasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

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.

Double incomplete aortic arch and Kommerell's Diverticulum as a cause of chronic cough.

Vascular rings which can cause symptoms related the trachea and esophagus compression occur in less than 1% of all cardiovascular malformations. Double incomplete aortic arch with right-sided aorta and aberrant left subclavian artery is the rarest one, and its present in 0.04-0.1% of autopsy series. A case of this malformation with a Kommerell's Diverticulum is presented. This diverticulum has risk of severe complications such as dissection and/or rupture.