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1.
Rev. bras. ginecol. obstet ; 43(6): 452-456, June 2021. tab, graf
Article in English | LILACS | ID: biblio-1341140

ABSTRACT

Abstract Objective The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). Methods This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. Results An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed amalformation: one case of hypospadias and 1 case of cleft palate. Conclusion The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers.


Resumo Objetivo O objetivo do presente estudo foi determinar a frequência demalformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). Métodos Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico prénatal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Resultados Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. Conclusão A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.


Subject(s)
Humans , Male , Female , Pregnancy , Adolescent , Adult , Young Adult , Subclavian Artery/abnormalities , Congenital Abnormalities/diagnosis , Ultrasonography, Prenatal , Chromosome Aberrations , Cardiovascular Abnormalities/genetics , Cardiovascular Abnormalities/diagnostic imaging , Subclavian Artery/diagnostic imaging , Echocardiography , Genetic Testing , Retrospective Studies
2.
Arq. bras. med. vet. zootec. (Online) ; 72(6): 2266-2270, Nov.-Dec. 2020. ilus
Article in English | LILACS, VETINDEX | ID: biblio-1142282

ABSTRACT

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)


Subject(s)
Animals , Female , Dogs , Subclavian Artery/anatomy & histology , Subclavian Artery/abnormalities , Congenital Abnormalities/veterinary , Esophageal Achalasia/veterinary
3.
Rev. bras. cir. cardiovasc ; 34(6): 769-771, Nov.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1057502

ABSTRACT

Abstract Aortic arch anomalies are not clinically important unless they cause compression symptoms due to aneurysmatic dilatation. Aortic anomalies need to be treated when they cause complex thoracic aortic diseases, and the treatment approach has evolved over time from open surgical methods, which have high mortality and morbidity rates, to hybrid methods. A case of a 68-year-old male patient with complex aortic arch anomaly treated with hybrid arch repair is reported in this study. Aortic branches were common carotid trunk and aberrant right subclavian artery with a saccular aneurysm.


Subject(s)
Humans , Male , Aged , Subclavian Artery/abnormalities , Vascular Surgical Procedures/methods , Aortic Aneurysm, Thoracic/surgery , Cardiovascular Abnormalities/surgery , Aorta, Thoracic/surgery , Aorta, Thoracic/diagnostic imaging , Subclavian Artery/surgery , Subclavian Artery/diagnostic imaging , Aortic Aneurysm, Thoracic/diagnostic imaging , Cardiovascular Abnormalities/diagnostic imaging , Computed Tomography Angiography
4.
Int. j. morphol ; 37(3): 821-824, Sept. 2019. graf
Article in Spanish | LILACS | ID: biblio-1012359

ABSTRACT

RESUMEN: Durante el desarrollo embrionario suelen ocurrir anomalías a nivel del arco aórtico primordial. Una de estas es la arteria subclavia retroesofágica derecha (ASDR) la cual es un defecto frecuente de los arcos aórticos embriológicos y se debe a la interrupción del cuarto arco aórtico (AO) derecho entre las depresiones para la arteria carótida común y la arteria subclavia. Las variaciones anatómicas y morfológicas del AO y sus ramas son significativas para los procedimientos diagnósticos y quirúrgicos en el tórax y el cuello. Reportar una ASDR encontrada durante disección, correlacionando esta con sus implicancias clínicas. De un cadáver de sexo femenino, de edad y causa de muerte desconocida, fijado con formaldehído al 10 % y aguada destilada. Se realizó disección del corazón y los grandes vasos, en especial el AO, desarrollado según la técnica convencional, para remover el órgano luego de disecar el pericardio y disecar la arteria aorta ascendente y el AO, preservando plenamente su configuración externa y sus ramas colaterales, donde se encontró la ASDR. La ASDR presentó un diámetro en su origen de 12,13 mm. La longitud existente entre la ASDR y la Arteria carótida común Derecha (ACCD) fue de 43,84 mm. El diámetro del esófago a nivel cefálico y caudal de la ASDR alcanzó valores de 17,59 mm y 13,82 mm respectivamente. Por su parte los diámetros de la tráquea a nivel cefálico y caudal a la ASDR, fueron respectivamente 22,12 mm y 13,30 mm. El conocimiento de esta variante anatómica resulta de gran interés a la hora de interpretar y orientar el diagnóstico de las posibles causas de una disfagia asociada a una arteria subclavia retroesofágica y fundamenta aún más la importancia del estudio mediante disección.


SUMMARY: During embryonic development, anomalies usually occur at the primordial aortic arch (AA) level. One of these is the right retroesophageal subclavian artery (RSA). The anatomical and morphological variations of AA and its branches are significant for diagnostic and surgical procedures in the thorax and neck. The objective of the study was to report an RSA found during the dissection, correlating this with the corresponding clinical implications. A human female cadaver of unknown age and cause of death was used and conserved in 10 % formaldehyde and distilled wash. The dissection of the heart and large vessels was performed. There was particular emphasis one the AA, and development according to conventional techniques, removing the organ after dissecting the pericardium and dissecting the ascending aorta and the AA. External function and its collateral branches were fully preserved, where the RSA was located. The RSA presented a diameter at its origin of 12.13 mm. The length between the RSA and the common right carotid artery (CRCA) was 43.84 mm. The diameter of the esophagus at the cephalic and caudal level of the RSA has values of 17.59 mm and 13.82 mm respectively. Furthermore, the diameters of the trachea at cephalic and caudal level to the RSA, respectively, were 22.12 mm and 13.30 mm. Knowledge of this anatomical variant is of great interest when interpreting and guiding the diagnosis of potential causes of a dysphagia associated with a retroesophageal subclavian artery, and is even more important during dissection studies.


Subject(s)
Humans , Female , Aorta, Thoracic/abnormalities , Subclavian Artery/abnormalities , Anatomic Variation , Cadaver , Esophagus
5.
Rev. chil. radiol ; 25(2): 71-74, jun. 2019. ilus
Article in Spanish | LILACS | ID: biblio-1013852

ABSTRACT

Objetivo: La arteria subclavia aberrante (ASA) o arteria lusoria, es una de las malformaciones más comunes del arco aórtico; en especial su variante derecha. Por otro lado, la arteria subclavia aberrante izquierda es más rara. Presentamos un caso infrecuente de un paciente con síndrome deleción 22q11.2 y ASA izquierda con síntomas en extremidad superior izquierda. La ASA izquierda es una condición muy poco frecuente y hay escasa información sobre su tratamiento quirúrgico. La mayoría de las veces, el ASA es asintomática; especialmente en adultos. Si presenta clínica, sus síntomas son disfagia, tos, disnea, claudicación de la extremidad superior. La presencia de sintomatología es una indicación quirúrgica. El tratamiento quirúrgico de una ASA es discutido. Éste va a depender de la anatomía, comorbilidades y experiencia del cirujano.


Objective: The Aberrant Right Subclavian Artery (ARSA) or Lusoria Artery is one of the most common aortic arch malformations. Aberrant Left Subclavian Artery (ALSA) on the other hand, is a much rarer condition. We present an uncommon case of ALSA in a patient with 22q11.2 deletion syndrome with upper limb symptoms and review the treatment options. ALSA is an exceedingly rare condition and information on its surgical treatment is scarce. In most cases the presence of an ALSA is asymptomatic, especially in adults. Some of the most typical symptoms are dysphagia (dysphagia lusoria), cough, dyspnea, claudication of the upper limb extremity. When symptomatic, the patient has indication of surgery. The treatment of an ALSA is still debatable and it depends on the anatomy, comorbidities and surgeon´s experience.


Subject(s)
Humans , Male , Adult , Subclavian Artery/abnormalities , Subclavian Artery/diagnostic imaging , DiGeorge Syndrome/complications , Subclavian Artery/surgery , Endovascular Procedures/methods
6.
Arch. cardiol. Méx ; 87(4): 345-348, oct.-dic. 2017. graf
Article in Spanish | LILACS | ID: biblio-887545

ABSTRACT

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


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


Subject(s)
Humans , Aorta, Thoracic/abnormalities , Subclavian Artery/abnormalities , Abnormalities, Multiple/diagnostic imaging , Diverticulum/complications , Cardiovascular Abnormalities/complications , Aneurysm/complications , Aorta, Thoracic/diagnostic imaging , Subclavian Artery/diagnostic imaging , Cardiovascular Abnormalities/diagnostic imaging , Vascular Ring/etiology , Vascular Ring/diagnostic imaging , Aneurysm/diagnostic imaging
7.
Rev. otorrinolaringol. cir. cabeza cuello ; 77(1): 73-77, mar. 2017. ilus
Article in Spanish | LILACS | ID: biblio-845650

ABSTRACT

Arteria Lusoria (AL) o arteria subclavia aberrante derecha es una malformación vascular rara. Es la más común de las malformaciones del arco aórtico. Fue descrita por primera vez por Hunalud en 1735. La etiología no está clarificada hasta hoy en día. AL puede formar parte de un cuadro sindrómico (síndrome de Down, síndrome de Edwards) o de una malformación cardiaca compleja. Es casi siempre asintomática, pero algunas veces puede revelarse por signos respiratorios o disfagia. Se elige habitualmente el tratamiento conservador. Les presentamos el caso de un niño de 2 años que presentó una AL revelada atipicamente por otitis media a repetición.


Arteria Lusoria (AL) or aberrant right subclavian artery is a rare congenital aortic arch malformation. Hunauld described it for the first time in 1735.The cause is until now no totally clarified. Usually it is associated with chromosomal disorders such as Down's syndrome, Edwards's syndrome or appears in childhood with complexes cardiovascular abnormalities. Patients are frequently asymptomatic, but it may be revealed by respiratories symptoms or by a dysphagia. The treatment is generally conservative. We expose a case report about a child of 2 years old with AL diagnosed after several episodes of purulent otitis.


Subject(s)
Humans , Male , Child, Preschool , Cardiovascular Abnormalities/complications , Cardiovascular Abnormalities/diagnosis , Otitis Media/etiology , Subclavian Artery/abnormalities , Deglutition Disorders/etiology , Otitis Media/therapy
9.
Rev. bras. anestesiol ; 66(3): 318-320, May.-June 2016.
Article in English | LILACS | ID: lil-782885

ABSTRACT

ABSTRACT Aberrant right subclavian artery-esophageal fistula is a rare but potentially fatal complication. It may be associated with procedures, such as tracheostomy and tracheal or esophageal intubation, and yields massive upper gastrointestinal bleeding difficult to identify and to control. A high index of suspicion is essential for early diagnosis and better prognosis. We report a rare case of a patient who survived after emergent surgical procedure for massive upper gastrointestinal bleeding secondary to aberrant right subclavian artery-esophageal fistula after prolonged intubation.


RESUMO A fístula de artéria subclávia direita anômala com o esôfago é uma complicação rara, mas potencialmente fatal. Pode estar associada a procedimentos como traqueostomia e intubação traqueal ou esofágica e originar hemorragia digestiva alta maciça, de difícil identificação e controle. Um elevado índice de suspeição é essencial para o diagnóstico precoce e a melhoria do prognóstico. Relatamos caso raro de doente que sobreviveu após intervenção cirúrgica emergente por hemorragia digestiva alta maciça secundária a fístula de artéria subclávia direita anômala com esôfago, após intubação gástrica prolongada.


Subject(s)
Humans , Male , Adult , Young Adult , Subclavian Artery/abnormalities , Deglutition Disorders/complications , Esophageal Fistula/complications , Cardiovascular Abnormalities/complications , Gastrointestinal Hemorrhage/complications , Intubation, Intratracheal/adverse effects , Aneurysm/complications , Time , Esophageal Fistula/surgery , Gastrointestinal Hemorrhage/surgery
10.
Rev. bras. cir. cardiovasc ; 31(1): 52-59, Jan.-Feb. 2016. tab, graf
Article in English | LILACS | ID: lil-778374

ABSTRACT

Abstract Aberrant origin of vertebral artery is rare. The anatomical features and clinical significance of this lesion remain to be clarified. A comprehensive collection of the pertinent literature resulted in a cohort of 1286 cases involving 955 patients and 331 cadavers. There were more left than right and more unilateral than bilateral aberrant vertebral arteries. Patients with aberrant origin of vertebral artery were often asymptomatic and in only 5.5% of the patients their symptoms were probably related to the aberrant origin of vertebral artery. The acquired cardiovascular lesions were present in 9.5% of the patients, 20.9% of which were vertebral artery-associated lesions. Eight (0.8%) patients had a vertebral artery dissection. Logistic regression analysis showed significant regressions between bovine trunk and left vertebral artery (P=0.000), between the dual origins of vertebral artery and cerebral infarct/thrombus (P=0.041), between associated alternative congenital vascular variants and cervical/aortic dissection/atherosclerosis (P=0.008). Multiple logistic regression demonstrated that side of the aberrant origin of vertebral artery (left vertebral artery) (P=0.014), arch branch pattern (direct arch origin) (P=0.019), presence of the common trunk (P=0.019), associated acquired vascular disorder (P=0.034) and the patients who warranted management (P=0.000) were significant risk predictors for neurological sequelea. The patients with neurological symptoms and those for neck and chest operations/ interventions should be carefully screened for the possibility of an aberrant origin of vertebral artery. The results from the cadaver metrology study are very helpful in the design of the aortic stent. The arch branch pattern has to be taken into consideration before any maneuver in the local region so as to avoid unexpected events in relation to aberrant vertebral artery.


Subject(s)
Female , Humans , Male , Aorta, Thoracic/abnormalities , Subclavian Artery/abnormalities , Vertebral Artery/abnormalities , Aorta, Thoracic/pathology , Cardiovascular Diseases/etiology , Cardiovascular Diseases/pathology , Nervous System Diseases/etiology , Nervous System Diseases/pathology , Risk Factors , Subclavian Artery/pathology , Vascular Malformations/complications , Vertebral Artery Dissection/etiology , Vertebral Artery Dissection/pathology , Vertebral Artery/pathology
11.
Rev. chil. neurocir ; 41(2): 127-130, nov. 2015. ilus
Article in Spanish | LILACS | ID: biblio-869734

ABSTRACT

El síndrome del desfiladero torácico es una entidad clínica compleja que abarca varias situaciones donde hay compresión del paquete vásculo-nervioso que suministra las extremidades superiores en el canal cervicotorácico. El síndrome se puede dividir en neurogénico y vascular de acuerdo con la estructura comprometida, siendo la primera la más frecuente. Entre las causas descritas están las costillas cervicales, anomalías de la primera costilla, músculos anómalos o hipertrofiados, traumatismos y tumores de la región. Describimos el caso de una paciente de 47 años que presenta un cuadro de cervicobraquialgia asociado con parestesias y paresia en la mano izquierda compatible con síndrome del desfiladero torácico en que la causa de la compresión era la arteria escapular descendente. Fue realizada una revisión de la literatura siendo encontrado sólo un caso similar.


The thoracic outlet syndrome is a complex clinical entity that encompasses several situations where there is compression of the neurovascular bundle that supplies the upper limbs in the cervicothoracic channel. It can be divided in neurogenic and vascular according to the structure committed, the first being the most frequently found. Among the causes described there are cervical ribs, anomalies of the first rib, anomalous or hypertrophied muscles, trauma and tumors of the region. We report the case of a 47 year old patient who presents with a clinical picture of cervicobrachialgia associated with paresthesia and paresis on the left hand compatible with thoracic outlet syndrome in which the cause of compression was the descending scapular artery.We conducted a literature review and found only one similar case.


Subject(s)
Humans , Female , Middle Aged , Aorta, Thoracic , Subclavian Artery/abnormalities , Brachial Plexus Block , Brachial Plexus Neuritis , Scapula/blood supply , Thoracic Outlet Syndrome/complications , Thoracic Outlet Syndrome/diagnosis , Thoracic Outlet Syndrome/etiology , Aneurysm, Ruptured/complications , Anterior Cerebral Artery/abnormalities
12.
Rev. méd. Chile ; 143(8): 1081-1084, ago. 2015. ilus
Article in English | LILACS | ID: lil-762675

ABSTRACT

We report a 61 years old male presenting with a right cerebral infarction, along with a type I persistent left proatlantal artery (PA), which is a form of primitive carotid-basilar anastomosis. The patient had an absence of the ipsilateral vertebral artery (VA) and hypoplasia of the contralateral VA, while the basilar artery was supplied by the PA. Other vascular anomalies present were a fusiform aneurysm of the right subclavian artery, and an A1 segment aplasia of the hypoplastic anterior right cerebral artery, which originated from the anterior communicating artery. To our knowledge these anomalies were not described previously.


Subject(s)
Humans , Male , Middle Aged , Intracranial Aneurysm/diagnosis , Subclavian Artery/abnormalities , Vertebral Artery/abnormalities , Brain Ischemia/complications , Cerebral Infarction/diagnosis , Endarterectomy, Carotid , Fatal Outcome , Stroke/complications
13.
Rev. bras. ginecol. obstet ; 37(6): 252-257, 06/2015. graf
Article in Portuguese | LILACS | ID: lil-752525

ABSTRACT

OBJETIVO: Avaliar a artéria subclávia direita durante a ecografia do primeiro trimestre, descrever a técnica para a sua avaliação e, em caso de identificação de artéria subclávia direita aberrante (ARSA), determinar sua associação com alterações cromossómicas e/ou malformações cardíacas e sua orientação. MÉTODOS: Estudo prospectivo que consistiu na avaliação da artéria subclávia direita no primeiro trimestre (comprimento crânio caudal entre 45 e 84 milímetros), em todas as gestações únicas, consecutivas, por um único examinador, ecógrafo Voluson E8 (GE Healthcare, Zipf, Áustria) com sonda transabdominal RAB 4-8-D, 2 a 8 MHz, por um período não superior a 2 minutos, numa população geral de baixo risco. Com a ajuda do power/color Doppler, a artéria subclávia direita foi classificada como normal ou aberrante. Foi utilizada análise de regressão estatística (IBM SPSS Statistics for Windows, versão 20.0) para estudar o grau de associação entre a falha na avaliação/classificação da artéria subclávia direita e o comprimento crânio-caudal fetal e o índice de massa corporal materno. RESULTADOS: A mediana da idade materna foi de 30 anos (variando entre 17 e 43 anos) e a mediana do tempo de gestação no momento da avaliação da artéria subclávia direita foi de 12 semanas de gestação (variando entre 11 e 13 semanas de gestação). A avaliação da artéria subclávia direita foi possível em 138/176 (78,4%) dos casos. ARSA foi diagnosticada em um único caso (0,7%). Esse feto com ARSA também apresentou um foco hiperecogênico no ventrículo cardíaco esquerdo. Foi realizada ecocardiografia fetal às 16 semanas de gestação, que confirmou o diagnóstico de ARSA e foco hiperecogênico. A amniocentese revelou cariótipo normal, 46, XX. CONCLUSÃO: É possível fazer o diagnóstico de ARSA na ecografia do primeiro trimestre. O nosso único caso de ARSA apresentou um cariótipo normal sem malformações cardíacas associadas. .


PURPOSE: To determine the feasibility of evaluation of the right subclavian artery during the first trimester ultrasound scan, as well as to describe the technique for its evaluation and, in case of aberrant right subclavian artery (ARSA) identification, to determine its association with chromosomal abnormalities and/or cardiac malformations and its management. METHODS: A prospective study for evaluation of the right subclavian artery during the first trimester ultrasound scan (crown-to-rump length between 45 and 84 mm), in all consecutive single pregnancies, by a single examiner, using a Voluson E8 system (GE Healthcare, Zipf, Austria) with a 2 to 8 MHz RAB 4-8-D transabdominal probe, within a short period of time (less than 2 minutes), in a general low risk population. Color and/or power Doppler flow mapping was used to classify the right subclavian artery as normal or aberrant. Regression analysis with the IBM SPSS Statistics software for Windows, version 20.0 was used to determine the significance of the association between failure to examine/classify the right subclavian artery and both fetal crown-rump length and maternal body mass index. RESULTS : Median maternal age was 30 years (range: 17-43 years) and median gestational age at the time of evaluation of the right subclavian artery was 12 weeks (range: 11-13 weeks). The evaluation of the right subclavian artery was successful in 138/176 (78.4%) of the cases. ARSA was diagnosed in a single case (0.7%). This fetus with ARSA also presented a hyperechogenic focus on the left cardiac ventricle. Fetal echocardiography at 16 weeks of gestation was performed and confirmed ARSA and the hyperechogenic focus. Amniocentesis revealed a normal 46, XX karyotype. CONCLUSION: ARSA can be identified during a routine first trimester ultrasound scan. Our single ARSA case had a normal karyotype and no associated cardiac malformations. .


Subject(s)
Humans , Female , Pregnancy , Adolescent , Adult , Young Adult , Subclavian Artery/abnormalities , Subclavian Artery/diagnostic imaging , Ultrasonography, Prenatal , Chromosome Aberrations , Feasibility Studies , Pregnancy Trimester, First , Prospective Studies
14.
Arch. cardiol. Méx ; 85(2): 158-160, abr.-jun. 2015. ilus
Article in English | LILACS | ID: lil-754926

ABSTRACT

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.


Los anillos vasculares pueden causar síntomas relacionados a compresión de tráquea y esófago y ocurren en menos del 1% de todas las malformaciones cardiovasculares. El doble arco aórtico incompleto con arco aórtico a la derecha y arteria subclavia izquierda aberrante es la forma más rara y se presenta en el 0.04 a 0.1% de las series de autopsia. Se presenta un caso de esta malformación con un divertículo de Kommerell. El divertículo tiene riesgo de complicaciones severas como disección y/o ruptura.


Subject(s)
Humans , Infant , Male , Abnormalities, Multiple , Aorta, Thoracic/abnormalities , Cough/etiology , Diverticulum/complications , Subclavian Artery/abnormalities , Chronic Disease
15.
Rev. chil. cir ; 67(1): 70-75, feb. 2015. ilus
Article in Spanish | LILACS | ID: lil-734742

ABSTRACT

Background: Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system and forming arterial, venous and capillary nests. Case report: We report a female consulting for the first time at the age of 15 for an AVM located in the left posterior region of the neck. The patient was operated at that time, performing an extensive excision of the lesion and covering the defect with a skin flap. The lesion relapsed three years later. Vascular imaging revealed the malformation with afferent arteries from the subclavian, vertebral and left occipital arteries and a great posterior cervical nest that drained through a great anomalous vein to the subclavian vein. A surgical ligation of all the anomalous branches of the subclavian vein was performed, to perform a local excision in a second intervention. The patient got pregnant, delaying the intervention and had a normal delivery at the age of 19. At 21 years of age, she consulted again due to a great growth of her AVM, with repeated bleeding episodes, requiring transfusions. The patient was treated with embolization of the nest and the afferent arteries. Finally at 23 years of age, the lesion was excised again and the defect was covered with a skin flap. The patient had a good postoperative evolution.


Introducción: Las Malformaciones Arterio-Venosas (MAV) son alteraciones estructurales congénitas del desarrollo del sistema vascular en que se observan comunicaciones anómalas arterio-venosas conformando un "nido" arterio-venoso-capilar. Caso Clínico: Mujer que consulta a los 15 años de edad por una MAV en la región cervical posterior izquierda desde su nacimiento. Se efectúa una resección amplia de la lesión hasta el plano aponeurótico cubriendo el defecto con un colgajo de rotación cutáneo-adiposo. Evoluciona bien, pero recidiva luego de tres años. Un estudio vascular a los 18 años de edad evidencia la MAV con arterias aferentes desde la arteria subclavia, vertebral y occipital izquierdas, un gran "nido" cervical posterior que drena por una gran vena anómala hacia la vena subclavia izquierda. Se efectúa una ligadura quirúrgica de todas las gruesas ramas anómalas de la arteria subclavia, para efectuar una nueva resección local en un segundo tiempo. Sin embargo, esto se pospone pues la paciente se embaraza y tiene un parto normal a los 19 años. Vuelve a consultar a los 21 años por un gran crecimiento de su MAV con hemorragias a repetición, algunas muy profusas que obligan a transfundirla. Una AngioRNM confirma la MAV con un gran nido cervical posterior con nuevas aferencias. Se efectúa embolizaciones directas locales con espuma de polidocanol y micropartículas, y finalmente con alcohol. Una vez reducido el nido se emboliza además las principales aferencias por vía endovascular y se efectúa a los 23 años una amplia resección de la MAV cubriendo el defecto con un colgajo miocutáneo de dorsal ancho izquierdo. Ha evolucionado bien hasta ahora. Conclusión: El diagnóstico y tratamiento de una MAV compleja representa un gran desafío al equipo médico tratante que acompaña y atiende al paciente en el transcurso del tiempo, y a veces por el resto de la vida.


Subject(s)
Humans , Adolescent , Female , Arteriovenous Malformations/surgery , Arteriovenous Malformations/complications , Subclavian Artery/abnormalities , Embolization, Therapeutic , Arteriovenous Malformations/diagnosis , Neck , Recurrence , Surgical Flaps
16.
Ann Card Anaesth ; 2014 Oct; 17(4): 302-305
Article in English | IMSEAR | ID: sea-153704

ABSTRACT

We present a case of severe tracheobronchial compression from a complex aorto‑subclavian aneurysm in a patient with Turner’s syndrome undergoing open surgical repair. Significant airway compression is a challenging situation and requires careful preoperative preparation, maintenance of spontaneous breathing when possible, and consideration of having an alternative source of oxygenation and circulation established prior to induction of general anesthesia. Cardiopulmonary monitoring is essential for safe general anesthesia and diagnosis of unexpected intraoperative events.


Subject(s)
Adult , Airway Obstruction/complications , Anesthesiology/methods , Anesthetics, Inhalation , Aortic Aneurysm, Thoracic/complications , Aortic Aneurysm, Thoracic/surgery , Bronchial Diseases/complications , Cardiopulmonary Bypass/methods , Catheterization, Swan-Ganz/methods , Circulatory Arrest, Deep Hypothermia Induced/methods , Constriction, Pathologic/complications , Echocardiography, Transesophageal/methods , Female , Humans , Methyl Ethers , Positive-Pressure Respiration/methods , Subclavian Artery/abnormalities , Subclavian Artery/surgery , Tracheal Stenosis/complications , Turner Syndrome/surgery
17.
Article in English | IMSEAR | ID: sea-162152

ABSTRACT

The purpose of this paper is to report a congenital subclavian artery to subclavian vein fistula in a three weeks old male infant who presented with congestive heart failure and pulmonary hypertension. He had been scanned by various imaging modalities including echocardiography, cardiac computed tomography, diagnostic cardiac catheterization, cardiac magnetic resonance angiocardiography and cranial computed tomoangiography. He had successful surgical ligation after failure of device occlusion trial at age of 9 months.


Subject(s)
Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessel Anomalies/diagnostic imaging , Fistula/diagnostic imaging , Fistula/diagnostic imaging , Humans , Infant , Male , Subclavian Artery/abnormalities , Subclavian Artery/diagnostic imaging , Subclavian Artery/diagnostic imaging , Subclavian Vein/abnormalities , Subclavian Vein/diagnostic imaging , Subclavian Vein/diagnostic imaging
18.
Arch. argent. pediatr ; 112(5): e217-e221, oct. 2014. ilus
Article in Spanish | LILACS | ID: lil-734279

ABSTRACT

La deficiencia de biotinidasa es una alteración metabólica autosómica recesiva, que afecta la escisión de biotina disminuyendo su reciclado. Estudios en familiares del caso índice encontraron que generalmente ambos padres son portadores y los hermanos presentan el gen alterado; solo los homocigotos tienen síntomas que varían según el grado de deficiencia. Las madres pueden tener deficiencia moderada y mantenerse asintomáticas. En un estudio que utiliza células humanas expuestas a deficiencia de biotina, disminuyó el crecimiento celular y contribuyó al desarrollo de paladar hendido. La deficiencia de biotina en embarazadas ocasiona malformaciones en los productos. En recién nacidos, la deficiencia de biotinidasa se ha relacionado con síndrome VACTERL y páncreas anular. Se presenta el caso de una lactante con deficiencia de biotinidasa y defecto congénito de anillo vascular que rodea y comprime tráquea y esófago, alterando la deglución y la respiración. La niña fue suplementada con biotina e intervenida, con excelentes resultados.


Biotinidase deficiency is an autosomal recessive metabolic disorder that affects the cleavage of biotin. Family studies of the index case found that both parents are usually carriers and siblings have the altered gene, but only homozygotes have manifestations that vary depending on the deficiency grade. Mothers may have moderate deficiency and be asymptomatic; biotin deficiency in pregnant women causes defects in children. In a study, using human cells exposed to biotin deficiency, cell growth decreased contributing to the development of cleft palate. In newborns, biotinidase deficiency has been associated with VACTERL syndrome and annular pancreas. The case of an infant with biotinidase deficiency and congenital defect of the vascular ring is presented. This defect surrounds and compresses the trachea and esophagus, disturbing swallowing and breathing. Infant was supplemented with biotin and surgically intervened with excellent results.


Subject(s)
Female , Humans , Infant , Aorta, Thoracic/abnormalities , Biotinidase Deficiency/complications , Subclavian Artery/abnormalities , Esophagus
19.
Arch. cardiol. Méx ; 84(3): 155-161, jul.-sep. 2014. ilus
Article in Spanish | LILACS | ID: lil-732022

ABSTRACT

Objetivos: Dentro de las malformaciones vasculares de las arterias en el tórax encontramos: 1) doble arco aórtico; 2) arco aórtico derecho y conducto arterioso izquierdo persistente; 3) arteria subclavia derecha aberrante; 4) arteria pulmonar izquierda aberrante, y 5) arteria innominada anómala. Se revisan los pacientes con arteria subclavia derecha aberrante y su manejo. Métodos: Se estudiaron en forma retrospectiva los expedientes de 29 pacientes en edad pediátrica, de enero de 1992 a diciembre 2012, con las siguientes variables: edad de inicio de los síntomas, manifestaciones clínicas, defectos cardiovasculares, método diagnóstico y abordaje quirúrgico. Resultados: El mayor número de pacientes cursó de forma asintomática, únicamente el 31% lo hizo con síntomas durante el primer año de vida, y se llevó a cabo el diagnóstico en un 35% mediante cateterismo. Fue la persistencia del conducto arterioso la cardiopatía más frecuente, con un 13%; el síndrome de Down se encontró en un 21% de los pacientes. El tratamiento más utilizado fue la sección de la arteria subclavia aberrante para la liberación del esófago. Conclusiones: Es importante la sospecha diagnóstica en pacientes con sintomatología durante la alimentación, con trastornos de la deglución con sólidos y, en algunos casos, con disfagia o hasta con dificultad respiratoria. Un número significativo de estos pacientes no son diagnosticados oportunamente, algunos alcanzan la edad adulta sin diagnóstico. El hallazgo de esta malformación ocurre durante los estudios de imagen, cuando se evalúa la aorta o en el estudio del reflujo gastroesofágico, ya que con el trago de bario se aprecia la compresión extrínseca del esófago.


Objectives: Congenital vascular malformations of the major arteries in the chest have been classified into 5 groups: 1) double aortic arch; 2) right aortic arch with left ligament or persistent ductus arteriosus; 3) aberrant subclavian artery; 4) aberrant left pulmonary artery, and 5) anomalous innominate artery. We reviewed the patients with aberrant right subclavian artery and their treatment. Methods: We studied retrospectively the records of 29 patients with aberrant right subclavian artery in childhood, from January 1992 to December 2012, analyzing the following variables: age at onset, clinical manifestations, associated cardiovascular defects, diagnosis and surgical approach method. Results: We found that most patients have an asymptomatic course, only 31% of them course with symptoms during the first year of life, with an incidental diagnosis of 35% during catheterization or other imaging studies. Patent ductus arteriosus was the most frequently associated congenital malformation, with 13%. Down's syndrome was found in 21%. The most common treatment was surgical section of the aberrant subclavian artery to release the esophagus. Conclusions: This vascular abnormality must be suspected in those patients with dysphagia, dyspnea, chest pain during feeding or breathing difficulties. A significant number of patients are not diagnosed in time, some reach adulthood without a diagnosis. This malformation is often found in imaging studies when evaluating the aorta or in a gastroesophageal reflux study, in which the barium bolus reveals the extrinsic compression of the esophagus.


Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Aneurysm/diagnosis , Aneurysm/surgery , Cardiovascular Abnormalities/diagnosis , Cardiovascular Abnormalities/surgery , Deglutition Disorders/diagnosis , Deglutition Disorders/surgery , Subclavian Artery/abnormalities , Academies and Institutes , Cardiology , Retrospective Studies , Subclavian Artery/surgery
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