Hemoptisis asociada a maniobra de valsalva secundaria a malformación arteriovenosa pulmonar rota en paciente con cirugía de Fontan. Revisión a propósito de un caso / Hemoptysis associated with valsalva maneuver secondary to ruptured pulmonary arteriovenous malformation in a patient with Fontan surgery. Review regarding a case

Se presenta un caso poco frecuente de hemoptisis en un paciente adolescente con cirugía de Fontan, causada por el sangrado de una malformación arteriovenosa pulmonar (MAVP), tras la práctica de un instrumento de viento (saxofón). El paciente se estudió con angiografía y se realizó cierre percutáneo, consiguiendo posteriormente mejoría clínica y evitando la aparición de nuevos episodios de hemoptisis. Actualmente existe escasa literatura que reporte casos de hemoptisis en pacientes con cirugía de Fontan y ningún caso publicado en relación a gatillantes relacionados a las maniobras de valsalva; como la generada al practicar instrumentos de viento. Por esta razón, se decide publicar este caso clínico en pro de generar mayores conocimientos en este grupo de pacientes con cardiopatías congénitas operadas con procedimientos paliativos como es la cirugía de Fontan.

A rare case of hemoptysis is presented in an adolescent patient with Fontan surgery, caused by bleeding from a pulmonary arteriovenous malformation (PAVM), after playing a wind instrument (saxophone). The patient was studied with angiography and percutaneous closure was performed, subsequently achieving clinical improvement and preventing the appearance of new episodes of hemoptysis. Currently, there is little literature on hemoptysis in patients with Fontan surgery and no case published in relation to triggers related to valsalva maneuvers; like that generated when practicing wind instruments. For this reason, it was decided to publish this clinical case in order to generate greater knowledge in this group of patients with congenital heart disease operated on with palliative procedures such as Fontan surgery.

Un caso pediátrico de telangiectasia hemorrágica hereditaria (Síndrome de Rendu-Osler-Weber) con compromiso pulmonar / A pediatric case of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome) with pulmonary involvement

La telangiectasia hemorrágica hereditaria (HHT), o síndrome de Rendu-Osler-Weber, se considera dentro del grupo de las enfermedades raras, pues afecta a 1 de cada 3.000 a 8.000 individuos. Tiene un patrón de herencia autosómica dominante y la mayor parte de los casos se debe a una alteración en la codificación de los genes endoglina (ENG) y activina receptor-like kinase 1 (ALK1), dando origen a los tipos 1 y 2, respectivamente. Esta alteración genética se traduce en una displasia del endotelio de la pared vascular debido a haploinsuficiencia para endoglina y se manifiesta clínicamente con epistaxis a repetición, telangiectasias mucocutáneas y malformaciones arteriovenosas (MAV) viscerales. A continuación, se presenta el caso clínico de una paciente pediátrica de 11 años de edad que se manifestó por disnea e hipoxemia severa debido a múltiples fístulas arteriovenosas pulmonares.

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is considered among rare diseases, as it affects 1 in every 3,000 to 8,000 individuals. It follows an autosomal dominant inheritance pattern, and most cases are due to alterations in the coding of the endoglin (ENG) and activin receptor-like kinase 1 (ALK1) genes, leading to types 1 and 2, respectively. This genetic alteration results in vascular endothelial dysplasia due to haploinsufficiency for endoglin and clinically manifests with recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations (AVMs). Herein, we present the clinical case of an 11-year-old pediatric patient who exhibited severe dyspnea and hypoxemia due to multiple pulmonary arteriovenous fistulas.

Malformaciones arteriovenosas pulmonares: presentación de un caso clínico pediátrico y revisión de la literatura / Pulmonary arteriovenous malformations: presentation of a pediatric case and review of the literature

Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

Entrecruzamiento ("criscross") de ramas pulmonares: una disposición inhabitual / Crossed pulmonary arteries

Se presentan dos casos clínicos de "criscross" de ramas pulmonares, una forma infrecuente de malformación del origen de estas arterias desde lados opuestos del tronco pulmonar y cuyos trayectos se cruzan en su camino hacia sus respectivos pulmones. De manera aislada es una condición benigna, sin consecuencias hemodinámicas y de buen pronóstico.

Two clinical cases of "crisscrossed" pulmonary arteries are presented. This is an unusual malformation in which arteries from opposite sides of the pulmonary trunk cross along their course towards their respective lungs. Usually, it is a benign condition when found as an isolated malformation, with no hemodynamic consequences and good prognosis.

Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico / Ring-sling complex association with pig bronchus: a pediatric case report

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

Origen anómalo de una de las ramas de la arteria pulmonar (OAAP): tratamiento quirúrgico y resultados / Anomalous origin of one of the branches of the pulmonary artery (AOPA): Surgical treatment and outcome

Resumen Introducción: El origen anómalo de una de las ramas de la arteria pulmonar procedente de la aorta ascendente es poco frecuente. Objetivo: Identificar las características clínicas y quirúrgicas de los pacientes sometidos a reimplante de la rama afectada. Método: Se realizó un estudio observacional, transversal, descriptivo, retrospectivo y retrolectivo, en el que se incluyeron los pacientes diagnosticados de origen anómalo de alguna de las ramas de la arteria pulmonar y que fueron tratados mediante cirugía correctiva en el Instituto Nacional de Cardiología Ignacio Chávez, en el periodo del 1 de enero de 2003 al 31 de enero de 2019. De los expedientes se extrajeron las características demográficas, los antecedentes quirúrgicos, los diagnósticos, los reportes ecocardiográficos y tomográficos, la técnica quirúrgica y el estado posquirúrgico. Resultados: Se encontraron nueve pacientes sometidos a cirugía de corrección, con un promedio de edad de 2 ± 2 años, un peso de 11.4 ± 1.5 kg y una talla de 82 ± 15 cm. El 67% fueron de sexo masculino. La rama afectada con más frecuencia fue la derecha; el 68% se originaban directamente de la aorta y el 42% de un conducto arterioso persistente. La corrección con circulación extracorpórea tuvo un tiempo promedio de pinzamiento aórtico de 35 minutos y de soporte circulatorio de 45 minutos. Se utilizó el implante directo o con material sintético. Las complicaciones fueron falla ventricular, sangrado, arritmias y neumonía nosocomial. La mortalidad fue del 11%. Conclusiones: El tratamiento quirúrgico para el reimplante de la rama anómala de la arteria pulmonar es el procedimiento de elección, y con cuidados quirúrgicos y posteriores tiene un buen pronóstico a mediano y largo plazo.

Abstract Introduction: The anomalous origin of one of the branches of the pulmonary artery from the ascending aorta is rare. Objective: To identify the clinical and surgical characteristics of the patients undergoing reimplantation of the affected branch. Method: An observational, cross-sectional, descriptive, retrospective and retrolective study was performed; patients diagnosed with anomalous origin of one of the branches of the pulmonary artery and treated by corrective surgery at the Instituto Nacional de Cardiología Ignacio Chavez, in the period from January 1, 2003 to January 31, 2019, were included. It was extracted from the files: demographic characteristics, surgical antecedents, diagnoses, echocardiographic and tomographic reports, surgical technique and post-surgical status. Results: Nine patients underwent correction surgery, with an average age of 2 ± 2 years, 11.4 ± 1.5 kg and height 82 ± 15 cm; 67% were male. The most frequent affected branch was the right, 68% originated directly from the aorta and 42% from a patent ductus arteriosus. The correction with extracorporeal circulation had an average aortic cross-clamp of 35 minutes and circulatory support of 45 minutes, the direct or synthetic implant was used. The complications were ventricular failure, bleeding, arrhythmias and nosocomial pneumonia. Mortality was 11%. Conclusions: Surgical treatment for reimplantation of the anomalous branch of the pulmonary artery is the procedure of choice, which with surgical and subsequent care has a good prognosis in the medium and long term.

Clinical and anatomopathological descriptions of dog with arterious Hemitruncus: case report / [Descrições clínicas e anatomopatológicas de cão com hemitruncus arterioso: relato de caso

The anomalous origin of the pulmonary trunk in the ascending aorta, defined as arterious hemitruncus, is a rare congenital malformation in dogs, caused by a defect in the spiral septum. Thus, given the unusual occurrence in the canine species, the systemic severity and the high lethality, the aim of this study was to describe this heart disease in a three-month-old male German Spitz puppy, emphasizing clinical changes of the necropsy and microscopics. The animal had cyanosis, dyspnea and weakness and was forwarded for necropsy after sudden death. Numerous changes were detected in the post-mortem examination, including in the heart, as cardiomegaly and absence of the arterial ligament, which was replaced by the complete fusion between the ascending aorta and the pulmonary trunk, after leaving both the left and right ventricles, respectively and, among the microscopic findings, cardiomyocyte hypertrophy stood out. The association of these findings with the history indicated the diagnosis of arterious hemitruncus followed by cardiorespiratory failure, emphasizing the importance of out complementary cardiological exams in young symptomatic patients for the survival of those affected. Arterious hemitruncus, although rare, must be added in the differential diagnosis of other heart diseases that cause similar clinical signs.(AU)

A origem anômala do tronco pulmonar em aorta ascendente, definida como hemitruncus arterioso, é uma malformação congênita rara em cães, causada por defeito no septo espiral. Assim, diante da ocorrência incomum na espécie canina, da gravidade sistêmica e da alta letalidade, o objetivo deste trabalho foi descrever essa doença cardíaca em um filhote de três meses de idade, macho, Spitz Alemão, enfatizando as alterações clínicas, de necropsia e microscópicas. O animal apresentava cianose, dispneia e fraqueza e foi encaminhado para necropsia após morte súbita. Inúmeras alterações foram detectadas no exame post-mortem, inclusive no coração, como cardiomegalia e ausência do ligamento arterioso, o qual foi substituído pela fusão completa entre aorta ascendente e tronco pulmonar, após a saída de ambas dos ventrículos esquerdo e direito, respectivamente, e, dentre os achados microscópicos, destacou-se a hipertrofia de cardiomiócitos. A associação desses achados com o histórico indicou o diagnóstico de hemitruncus arterioso seguido de insuficiência cardiorrespiratória, ressaltando-se a importância de exames complementares cardiológicos em pacientes jovens sintomáticos na sobrevida dos acometidos. O hemitruncus arterioso, apesar de raro, deve ser acrescido no diagnóstico diferencial de outras cardiopatias que causam sinais clínicos similares.(AU)

Agenesia unilateral de la arteria pulmonar: presentación de dos casos y revisión de la literatura / Unilateral agenesis pulmonary artery: presentation of two cases and review of the literature

Unilateral agenesis of the pulmonary artery is a rare malformation, sometimes asymptomatic and underdiagnosed. Right agenesis is usually isolated, while the left agenesis is more frequently associated with cardiovascular malformations. Some patients have recurrent respiratory infections, exercise limitation, hemoptysis, and/or pulmonary hypertension. The diagnosis is suspected by chest radiograph showing a hypoplastic lung ipsilateral to the agenesis. It is confirmed with contrast-enhanced chest computed tomography. In asymptomatic patients, management should be conservative, however, the search for collateral vessels should be started from adolescence, because they can bleed. In symptomatic patients or those with serious complications, the treatment must be interventional. The objective of this article is to present two patients with right and left pulmonary artery agenesis respectively and review the literature.

La agenesia unilateral de la arteria pulmonar es una malformación poco frecuente, en ocasiones asintomática y subdiagnosticada. La agenesia derecha suele ser aislada, en cambio la izquierda se asocia más frecuentemente a malformaciones cardiovasculares. Algunos pacientes presentan infecciones respiratorias recurrentes, limitación al ejercicio, hemoptisis y/o hipertensión pulmonar. El diagnóstico se sospecha al solicitar una radiografía de tórax que muestra un pulmón hipoplásico ipsilateral a la agenesia. Se confirma con tomografía computada de tórax con contraste. En pacientes asintomáticos el manejo debe ser conservador, sin embargo, se debe iniciar la búsqueda de vasos colaterales especialmente durante la adolescencia, los que pueden sangrar. En cambio, en los pacientes sintomáticos o que presentan complicaciones graves, el tratamiento debe ser intervencional. El objetivo de este trabajo es presentar a dos pacientes con agenesia de la arteria pulmonar derecha e izquierda respectivamente y hacer una revisión de la literatura.

Fístulas arteriovenosas pulmonares en un paciente pediátrico: un reporte de caso / Pulmonary arteriovenous fistulas in a pediatric patient: a case report

Las fístulas arteriovenosas pulmonares son malformaciones congénitas dadas por la comunicación directa anómala entre arterias y venas, con una incidencia mundial de 2-3 : 100 000 habitantes. La presentación es, en general, única, asintomática, y aparecen en forma incidental como hallazgo imagenológico en la adultez, y su tratamiento de elección es la embolización endovascular.Se describe la inusual presentación clínica en una paciente de 10 años, que ingresó por disnea, tos, cianosis central y cefalea. Se encontró hipoxemia persistente, hipocratismo digital, nódulos parahiliares pulmonares, gases arteriales con gradiente alvéolo-arterial aumentado. La tomografía axial computarizada de tórax de alta resolución confirmó la presencia de una malformación arteriovenosa pulmonar en la región parahiliar derecha, la cual no se asociaba con la enfermedad de Rendu-Osler-Weber. La paciente fue tratada con embolización endovascular transcutánea. Tras 1,5 años de seguimiento, no hubo recaídas. Son pocos los casos reportados de estas fístulas en la edad pediátrica

Pulmonary arteriovenous fistulas are congenital malformations due to anomalous direct communication between arteries and veins; the incidence is 2-3 : 100,000 inhabitants. This condition is usually asymptomatic and incidentally appearing in adult imaging findings. Transcutaneous endovascular embolization is the technique of choice for treatment. The unusual presentation in a 10-year-old patient is described; she was presented to the Emergency Department with dyspnea, cough, central cyanosis and headache. The examination revealed persistent hypoxemia and digital clubbing; chest X-ray with images suggestive of parahilar nodules, arterial blood gases with increased alveolar arterial gradient. The high resolution computed tomography of the thorax revealed pulmonary arteriovenous malformation in the right parahilar region not associated with Rendu-Osler-Weber disease. The patient was treated with transcutaneous endovascular embolization, and after a year and a half of follow-up there were no relapses. There are few reported cases of pulmonary arteriovenous fistulas in the pediatric age.

Agenesia da artéria pulmonar direita: descrição de caso / Agenesis of the right pulmonary artery: a case report

RESUMO: Agenesia isolada da artéria pulmonar direita ou esquerda é uma rara anomalia congênita dada pela falta do desenvolvimento embriológico de um dos arcos aórticos. A apresentação clínica é variável, sendo a mais comum na infância, na forma de hipertensão pulmonar contralateral. No adulto, a hemoptise pode ser uma das manifestações clínicas, e nos indivíduos assintomáticos esta anomalia pode ser reconhecida em exames de diagnóstico por imagem. Relatamos o caso de paciente do sexo feminino, 63 anos, encaminhada para investigação de imagem nodular no hilo pulmonar esquerdo observada em radiografia torácica. Tomografia computadorizada evidenciou agenesia da artéria pulmonar direita com suprimento arterial pulmonar homolateral ocorrendo por vasos colaterais, pulmão esquerdo com proeminência das artérias e veias hilares e peri-hilares, provavelmente simulando uma nodulação na radiografia realizada previamente, volume discretamente reduzido do pulmão direito com espessamento liso dos septos interlobulares secundário, provavelmente devido ao aumento da microcirculação colateral intraparenquimatosa. (AU)

ABSTRACT: Isolated agenesis of the right or left pulmonary artery is a rare congenital anomaly due to the lack of embryological development of one of the aortic arches. The clinical presentation is variable, being the most common in childhood, in the form of contralateral pulmonary hypertension. In adults, hemoptysis may be one of the clinical manifestations, and in asymptomatic individuals, this anomaly can be recognized in diagnostic imaging tests. We report the case of a female patient, 63 years old, referred for investigation of the nodular image in the left pulmonary hilum observed on chest radiography. Computed tomography showed right pulmonary artery agenesis with homolateral pulmonary arterial supply occurring by collateral vessels, left lung with a prominence of the hilar and peri-hilar arteries and veins probably simulating nodulation on the radiograph previously performed, a discreetly reduced volume of the right lung with smooth thickening of the septa secondary to probably increased intraparenchymal collateral microcirculation. (AU)

Long-term assessment of left ventricular ejection fraction and mitral regurgitation following takeuchi repair

Abstract Objective: This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). Methods: Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records. Results: Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients. Conclusion: Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.

Sling de la arteria pulmonar en paciente preescolar con compromiso de la vía aérea / Pulmonary artery sling in preschool patient with airway compromise

Resumen Introducción: El sling de la arteria pulmonar izquierda es una rara malformación congénita del arco aórtico en la que la rama pulmonar izquierda se origina a partir de la arteria pulmonar derecha. Generalmente se sitúa entre la tráquea y el esófago, que frecuentemente la comprimen. Dependiendo del grado de compromiso de la vía aérea, el esófago y las anomalías cardíacas asociadas, las manifestaciones clínicas varían. El tratamiento de esta malformación es quirúrgico; con el paso de los años, este abordaje ha mejorado, y se ha logrado una disminución de la morbilidad y mortalidad en esta condición. Caso clínico: Se presenta el caso de una paciente preescolar con hallazgo de sling de la arteria pulmonar izquierda con compromiso de la vía aérea. Conclusiones: Después de la corrección quirúrgica, se logró una respuesta clínica favorable y una mejoría significativa en la vía aérea, evidenciada en los hallazgos en la fibrobroncoscopia, sin necesidad de intervenciones adicionales.

Abstract Background: Left pulmonary artery sling is a rare congenital malformation of the aortic arch in which this artery originates from the right pulmonary artery and courses over the trachea and the esophagus. Clinical manifestations vary depending on the degree of airway compromise, the esophagus and associated cardiac anomalies. The treatment for this malformation is surgical; over the years, this approach has improved, achieving a decrease in morbidity and mortality in this condition. Case report: A preschool patient with incidental finding of sling of the left pulmonary artery with compromise of the airway is presented. Conclusions: After the surgical procedure, a favorable clinical outcome was observed and a significant improvement in the airway was shown by a fibrobronchoscopy, without any additional intervention.

Unusual distal right pulmonary artery origin from right ductus arteriosus with uncommon left-sided aortic arch / Inusual origen de la parte distal de la arteria pulmonar derecha en un ductus arterioso derecho en arco aórtico izquierdo no común

Abstract A bilateral persistent ductus arteriosus with right proximal pulmonary artery agenesis is a very unusual anatomic variant. These malformations are assumed to occur during the transformation of the sixth aortic arch with the pulmonary trunk in the development of the aorta and its branches. A 2-months preterm baby was referred by severe bronchopulmonary dysplasia. Transthoracic echocardiography showed a large pulmonary trunk and left pulmonary artery. No right proximal pulmonary artery was found. Cardiac computed tomography study showed bilateral ductus arteriosus. The right proximal pulmonary artery was originated from an arterial structure coming off the right subclavian artery, a right ductus arteriosus. The aortic arch was left-sided, with a peculiar variant at the exit of the supraaortic vessels. Right subclavian artery emerged as the first supraaortic vessel, followed by a common trunk, giving rise to both carotids and the left subclavian artery as the last vessel. Right lung was hypoplastic with interstitial disease. The calibre of the right proximal pulmonary artery was inadequate to perform a corrective surgery. LDA closure was performed, keeping a prostaglandin infusion to maintain right ductus arteriosus patency until right proximal pulmonary artery size will be big enough to perform a successful surgical reconstruction. Unfortunately, right ductus arteriosus became narrow at its origin and she developed severe pulmonary hypertension and the patient died.

Resumen El ductus arterioso bilateral con agenesia proximal de la arteria pulmonar derecha es una variante anatómica rara producida por alteraciones de la transformación del sexto arco aórtico en las arterias pulmonares, la aorta y sus vasos, durante el desarrollo embrionario. Se presenta el caso de un bebé prematuro de dos meses, que fue derivado a nuestro hospital por displasia broncopulmonar severa. El ecocardiograma transtorácico no identificó el origen de la arteria pulmonar derecha. El estudio por tomografía computarizada cardiaca mostró ductus arterioso bilateral e identificó el origen distal de la arteria pulmonar derecha en una rama de la arteria subclavia derecha, identificada como ductus arterioso derecho. El pulmón derecho era hipoplástico y presentaba alteraciones intersticiales. A su vez, el patrón de los vasos supraaórticos era anómalo. El primer vaso correspondió a la arteria subclavia derecha, seguido por tronco común de ambas carótidas y por último la arteria subclavia izquierda. El calibre de la arteria pulmonar derecha fue inadecuado para realizar una cirugía correctiva, por lo cual se decidió cerrar el ductus arterioso izquierdo y mantener infusión de prostaglandina hasta que la arteria pulmonar derecha tuviera un calibre adecuado para la cirugía. Infortunadamente, el ductus arterioso derecho se cerró en su origen, desarrollándose hipertensión pulmonar severa y la paciente falleció.

Pulmonary arteriovenous malformations: diagnostic and treatment characteristics / Malformações arteriovenosas pulmonares: características diagnósticas e de tratamento

ABSTRACT Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

RESUMO Objetivo: Apresentar uma série de casos de malformações arteriovenosas pulmonares (MAVP) e descrever os principais achados clínicos, a quantidade e localização das MAVP, as complicações clínicas e os tratamentos realizados. Métodos: Estudo retrospectivo observacional que avaliou pacientes com MAVP divididos em dois grupos: telangiectasia hemorrágica hereditária (THH) e MAVP idiopática (MAVPi). Resultados: Foram avaliados 41 pacientes, sendo 33 pacientes portadores de MAVP. Após a avaliação clínica, 27 e 6 foram diagnosticados com THH e MAVPi, respectivamente. No grupo THH a média de idade foi de 49,6 anos e 88,9% eram do sexo feminino. Desses pacientes, 4 tinham SpO2 < 90% e o achado clínico mais frequente era epistaxe. No grupo MAVPi a média de idade foi de 48,1 anos, sendo que 83,3% eram do sexo feminino. Desses, 3 tinham SpO2 < 90%. Após a realização de angiotomografia de tórax observou-se que a maior parte das MAVP se situava nos lobos inferiores, totalizando 56,4% e 85,7% nos grupos THH e MAVPi, respectivamente. O tratamento por embolização foi realizado em 23 pacientes nos dois grupos, enquanto 10 aguardavam o procedimento até o momento da escrita deste estudo. Um paciente submetido à embolização foi encaminhado para ressecção pulmonar. Conclusões: Em ambos os grupos de pacientes com MAVP observou-se uma predominância de mulheres e de fístulas localizadas nos lobos inferiores. A maioria era assintomático respiratório com SpO2 > 90%. O tratamento de escolha para todos foi a embolização percutânea por cateter.

Anomalías congénitas de arterias coronarias, estudio de aquellas con Importancia hemodinámica / Evaluation of the hemodynamically significant congenital anomalies of coronary arteries

Resumen: Las anomalías de las arterias coronarias son poco frecuentes, con una prevalencia de 0,21- 5,79%. Su presentación clínica es amplia, pudiendo ser asintomáticas o presentarse como isquemia miocárdica y muerte súbita, la que puede estar ligada o no al ejercicio. Existen varias clasificaciones, siendo las más usadas las que las agrupan desde un punto de vista anatómico en relación con el segmento afectado (origen, curso o terminación) y desde el punto de vista funcional (hemodinámicamente significativa y no significativa). Actualmente la Tomografía Computada Cardiaca se considera el estándar de referencia, siendo de suma importancia su adecuada caracterización ya que, de requerir tratamiento, este generalmente es quirúrgico. El objetivo de este trabajo es identificar, caracterizar y clasificar las anomalías congénitas de las arterias coronarias por su importancia hemodinámica mediante la presentación de casos clínicos y revisión de la literatura.

Abstract: Coronary artery anomalies are rare, with a prevalence ranging from 0.21 to 5.79%. Their clinical presentation is variable; being either asymptomatic or presenting as myocardial ischemia and sudden death, which may or may not be linked to exercise. There are several classifications, the most commonly used being those that sort them from an anatomical point of view in relation to the affected segment (origin, course or termination) and from a functional point of view (hemodynamically significant and not significant). Currently, Cardiac Computed Tomography is considered the reference standard allowing an adequate characterization of the anomaly, which is highly relevant since, if treatment is needed, it usually involves surgery. The purpose of this review is to identify, characterize and classify congenital anomalies of the coronary arteries according to their hemodynamic significance through the presentation of clinical cases and review of the literature.