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1.
Arch. argent. pediatr ; 120(3): e133-e136, junio 2022. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1368481

ABSTRACT

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.


Subject(s)
Humans , Male , Infant , Tracheal Diseases , Tracheal Stenosis/surgery , Tracheal Stenosis/diagnosis , Bronchial Diseases/surgery , Vascular Malformations/complications , Vascular Ring/complications , Heart Defects, Congenital/complications , Pulmonary Artery/abnormalities , Trachea/abnormalities , Bronchi/abnormalities , Bronchi/surgery , Constriction, Pathologic
2.
Arq. bras. med. vet. zootec. (Online) ; 73(2): 406-410, Mar.-Apr. 2021. ilus
Article in English | LILACS, VETINDEX | ID: biblio-1248953

ABSTRACT

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)


Subject(s)
Animals , Dogs , Aorta/abnormalities , Pulmonary Artery/abnormalities , Heart Septal Defects/pathology , Heart Septal Defects/veterinary , Congenital Abnormalities/veterinary
3.
Neumol. pediátr. (En línea) ; 16(1): 48-52, 2021. ilus
Article in Spanish | LILACS | ID: biblio-1284224

ABSTRACT

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.


Subject(s)
Humans , Male , Adolescent , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Radiography, Thoracic , Tomography, X-Ray Computed , Hemoptysis/etiology
4.
Arch. argent. pediatr ; 118(2): e170-e173, abr. 2020. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1100428

ABSTRACT

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.


Subject(s)
Humans , Female , Child , Pulmonary Artery/abnormalities , Arteriovenous Fistula/diagnostic imaging , Embolization, Therapeutic , Pulmonary Artery/diagnostic imaging , Arteriovenous Fistula/therapy
5.
Article in Portuguese | LILACS | ID: biblio-1358880

ABSTRACT

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)


Subject(s)
Humans , Female , Child , Pulmonary Artery/abnormalities , Pulmonary Artery/pathology , Congenital Abnormalities , Radiography, Thoracic , Tomography, X-Ray Computed , Hypertension, Pulmonary
8.
Rev. bras. cir. cardiovasc ; 34(6): 687-693, Nov.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1057504

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Adult , Young Adult , Pulmonary Artery/abnormalities , Bland White Garland Syndrome/surgery , Mitral Valve Insufficiency/surgery , Vascular Surgical Procedures/methods , Retrospective Studies , Treatment Outcome , Hospital Mortality , Cardiac Surgical Procedures/methods , Mitral Valve/surgery
9.
Rev. colomb. cardiol ; 26(5): 300-303, sep.-oct. 2019. graf
Article in English | LILACS, COLNAL | ID: biblio-1092942

ABSTRACT

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.


Subject(s)
Humans , Infant , Aorta , Pulmonary Artery/abnormalities , Ductus Arteriosus, Patent , Hypertension, Pulmonary , Bronchopulmonary Dysplasia , Infant, Premature , Echocardiography
11.
J. bras. pneumol ; 45(4): e20180137, 2019. tab, graf
Article in English | LILACS | ID: biblio-1012569

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Arteriovenous Malformations/etiology , Arteriovenous Malformations/therapy , Arteriovenous Malformations/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Telangiectasia, Hereditary Hemorrhagic/complications , Catheterization/methods , Angiography/methods , Echocardiography , Radiography, Thoracic/methods , Retrospective Studies , Treatment Outcome , Embolization, Therapeutic/methods , Septal Occluder Device , Computed Tomography Angiography
12.
Rev. chil. radiol ; 24(4): 142-150, dic. 2018. tab, ilus
Article in Spanish | LILACS | ID: biblio-978170

ABSTRACT

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.


Subject(s)
Humans , Male , Middle Aged , Coronary Vessel Anomalies/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Artery/embryology , Tomography, X-Ray Computed , Coronary Vessel Anomalies/classification , Coronary Vessel Anomalies/embryology , Coronary Vessel Anomalies/therapy , Fistula/congenital , Hemodynamics
13.
Arch. pediatr. Urug ; 89(2): 108-112, abr. 2018. ilus
Article in Spanish | LILACS | ID: biblio-950129

ABSTRACT

Las anomalías de las arterias coronarias son una condición clínica de muy baja incidencia y de estas el origen anómalo de la arteria coronaria derecha desde el tronco de la arteria pulmonar (ARCAPA) representa cerca de 0,002% en la población general. Se puede asociar a la presencia de otras anomalías cardíacas congénitas. Presentamos el caso de una recién nacida, pretérmino de 33 semanas, gemelar, a la cual se le realiza el diagnóstico de un probable origen anómalo de coronaria derecha en un estudio ecocardiográfico de rutina realizado en la unidad de terapia intensiva. Se confirma el diagnóstico mediante cateterismo cardíaco y se realiza la corrección quirúrgica definitiva a los 6 meses de edad, estando la niña actualmente asintomática y con una calidad de vida normal. Destacamos la baja frecuencia de la ocurrencia de dicha patología, realizamos una revisión sobre los tópicos principales en el desarrollo del árbol vascular coronario y las principales anomalías del mismo. Jerarquizamos la importancia de realizar un estudio ecocardiográfico adecuado como valoración de pacientes internados en unidades de terapia intensiva neonatal.


Coronary artery anomalies are a clinical condition of very low incidence. Out of these, the anomalous origin of the right coronary artery from the trunk of the pulmonary artery (ARCAPA) is estimated to represent 0.002% of the general population. It may be associated with the presence of other congenital cardiac anomalies. The study presents the case of a 33 week preterm newborn twin who is diagnosed with an anomalous origin of the right coronary in a routine echocardiographic study performed in the Intensive Care Unit. The diagnosis is confirmed by cardiac catheterization and definitive surgical correction is performed at 6 months of age, the child being currently asymptomatic and enjoying a normal quality of life. We stand out the low frequency of the occurrence of this pathology. We performed a review of the main topics in the development of the coronary vascular tree and their main anomalies. We emphasize on the importance of performing an adequate Echocardiographic study as an assessment of patients admitted to Neonatal Intensive Care Units.


As anomalias das artérias coronárias representam uma condição clínica de incidência muito baixa; e a origem anômala da artéria coronária direita do tronco da artéria pulmonar (ARCAPA) representa somente uma estimativa de 0,002% na população geral. Pode estar associada à presença de outras anomalias cardíacas congênitas. Apresentamos o caso de um recém-nascido, prematuro de 33 semanas, gêmeo, diagnosticado com provável origem anômala de coronária direita em estudo ecocardiográfico de rotina realizado na Unidade de Terapia Intensiva. Confirmou-se o diagnóstico através de cateterismo cardíaco e realizou-se a correção cirúrgica definitiva aos 6 meses de idade; a doença atualmente é assintomática e a menina tem uma qualidade de vida normal. Ressaltamos a baixa frequência da ocorrência da referida patologia; realizamos uma revisão dos principais tópicos no desenvolvimento da árvore vascular coronariana e suas principais anomalias. Destacamos a importância de realizar um estudo ecocardiográfico adequado como estratégia de avaliação de pacientes internados em Unidades de Terapia Intensiva Neonatal.


Subject(s)
Humans , Pulmonary Artery/abnormalities , Truncus Arteriosus/physiopathology , Echocardiography , Coronary Vessel Anomalies
14.
Arch. argent. pediatr ; 116(1): 102-105, feb. 2018. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-887438

ABSTRACT

El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.


Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.


Subject(s)
Humans , Male , Infant , Pulmonary Artery , Pulmonary Artery/abnormalities , Abnormalities, Multiple/diagnostic imaging , Coronary Vessel Anomalies/diagnostic imaging , Echocardiography , Incidental Findings
17.
Rev. bras. cir. cardiovasc ; 32(1): 29-37, Jan.-Feb. 2017. tab, graf
Article in English | LILACS | ID: biblio-843458

ABSTRACT

Abstract Objective: This study evaluated clinical and diagnostic findings, treatment methods, and follow-up of cases of anomalous coronary arteries from the pulmonary artery. Methods: The study included all cases diagnosed with anomalous coronary arteries from the pulmonary artery between January 2012 and January 2016. Data from patients’ demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, intensive care unit stay, and follow-up were evaluated. Results: The study included 12 patients (8 male, 4 female), 10 with anomalous left coronary artery from the pulmonary artery (ALCAPA) and 2 with anomalous right coronary artery from the pulmonary artery (ARCAPA). Median age at diagnosis was 4 months (range, 1 month - 10 years old) and median weight was 5.5 kg (range, 3-30 kg). The most common complaints were murmur (n=7) and respiratory distress (n=5). In 4 cases, the initial diagnosis was dilated cardiomyopathy. Electrocardiographs were pathologic in all cases. Echocardiographic examination revealed medium to severe mitral valve regurgitation in 4 cases and reduced (< 40%) ejection fraction in 6 patients. Of the 12 patients, 8 underwent direct implantation of the left coronary artery into the aorta, 2 underwent implantation of the right coronary artery into the aorta, and the remaining 2 underwent a Takeuchi procedure. There were no early mortalities. Median hospital stay was 20 days (range, 5-35 days). Median follow-up duration was 18 months (range, 5-36 months), and no cases required further surgery during follow-up. Conclusions: Anomalous coronary arteries from the pulmonary artery can be successfully repaired providing there is early diagnosis and effective, appropriate intensive care unit follow-up. Therefore, coronary artery origins should be evaluated carefully, especially in cases with dilated cardiomyopathies.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Pulmonary Artery/abnormalities , Bland White Garland Syndrome/surgery , Bland White Garland Syndrome/diagnostic imaging , Echocardiography , Extracorporeal Membrane Oxygenation , Retrospective Studies , Coronary Vessels , Electrocardiography , Cardiac Surgical Procedures , Intensive Care Units
18.
Rev. bras. anestesiol ; 67(1): 85-88, Jan.-Feb. 2017. graf
Article in English | LILACS | ID: biblio-843359

ABSTRACT

Abstract Background and objectives: Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. Case report: We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Conclusion: Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery.


Resumo Justificativa e objetivos: A ausência congênita unilateral de uma artéria pulmonar (ACAP) é uma anomalia rara. Embora existam vários relatos sobre pacientes grávidas com ACAP, não há relatos de casos que descrevam anestesia para cesariana em pacientes com ACAP. Relato de caso: Apresentamos uma paciente com ACAP que foi submetida a duas cesarianas, aos 24 e 26 anos, sob raquianestesia para a cirurgia e analgesia epidural para a dor no pós-operatório. Nas duas cesarianas, a raquianestesia e a analgesia epidural possibilitaram o manejo bem-sucedido da anestesia, sem a ocorrência de qualquer hipertensão pulmonar ou insuficiência cardíaca direita. Conclusão: Raquianestesia combinada com analgesia epidural é um método anestésico útil para cesarianas em pacientes com ACAP.


Subject(s)
Humans , Female , Pregnancy , Adult , Young Adult , Pulmonary Artery/abnormalities , Cesarean Section/methods , Pain, Postoperative/prevention & control , Pregnancy Complications, Cardiovascular , Infusions, Intravenous , Midazolam/administration & dosage , Bupivacaine/administration & dosage , Analgesia, Epidural/methods , Cesarean Section, Repeat/methods , Ropivacaine , Amides , Hypnotics and Sedatives , Anesthesia, Epidural , Anesthesia, Obstetrical/methods , Anesthesia, Spinal/methods , Anesthetics, Local/administration & dosage
19.
Rev. méd. Chile ; 145(1): 121-125, ene. 2017. ilus
Article in Spanish | LILACS | ID: biblio-845511

ABSTRACT

Anomalies of the origin of coronary arteries are detected in 0.5-1.5% of all angiographies. Anomalous origin of the left main trunk is the most uncommon and its origin from pulmonary artery in adults is exceptional, usually because it is associated with a short survival. We report a 49-year-old female, presenting with a two months history of angina. The exercise electrocardiogram suggested ischemia. A coronary angiography was performed, showing the absence of the left main trunk in the left coronary sinus, a dilated right coronary artery, with no lesions and extensive collateral circulation to the anterior descending and circumflex arteries, with inverted flow and the left main trunk draining to the pulmonary artery. The left ventricle was mildly dilated with middle and apical anterior hypokinesia. Global systolic function was conserved. A surgical correction was decided, occluding the left main anomalous origin and performing a coronary artery bypass grafting from the left internal thoracic artery. The patient was discharged with no complications. At two years of follow-up she is symptom free and has a normal physical capacity.


Subject(s)
Humans , Female , Middle Aged , Pulmonary Artery/abnormalities , Coronary Artery Disease/complications , Coronary Vessel Anomalies/complications , Angina Pectoris/etiology , Pulmonary Artery/diagnostic imaging , Coronary Artery Disease/diagnostic imaging , Coronary Angiography , Coronary Vessel Anomalies/diagnostic imaging , Angina Pectoris/diagnostic imaging
20.
Rev. méd. Chile ; 144(10): 1277-1286, oct. 2016. ilus, tab
Article in Spanish | LILACS | ID: biblio-845442

ABSTRACT

Background: Coronary anomalies are rare heart diseases that can cause sudden cardiac death, especially in young individuals. Coronary computed tomography angiography delivers a three-dimensional view of excellent quality with information of the origin, course and anatomic relationships of the anomalous vessel, allowing differentiation between benign and malignant variants, entities that require different management and treatment. Aim: To show that coronary computed tomography angiography is the non-invasive technique of choice for the evaluation of a coronary anomaly due to its high diagnostic accuracy. Material and Methods: Between 2008 and 2014, 368 coronary computed tomography angiographies were carried out. Six (1.6%) patients had a coronary anomaly. Results: The age of these six patients ranged from 38 to 82 years (five were women). The coronary computed tomography angiography was requested due to cardiac symptoms (angina and dyspnea) in two patients, after a selective coronary arteriography to obtain additional information of the coronary anomaly in three patients, and after a cardiorespiratory arrest in one patient. Three patients had an anomaly of the right coronary artery, two patients had an anomaly of the left coronary artery and one patient had absent left main coronary artery. In five patients the coronary anomaly was malignant. Conclusions: Coronary computed tomography angiography is a robust diagnostic method to evaluate patients with suspected coronary anomalies.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Coronary Angiography/methods , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessels/diagnostic imaging , Computed Tomography Angiography/methods , Aorta/abnormalities , Aorta/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Reproducibility of Results , Coronary Vessel Anomalies/pathology
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