Doble arco aórtico. Reporte y análisis de caso en un paciente colombiano

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Abstract

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

Translated abstract

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

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Most cited references 21

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Effect of neural crest ablation on development of the heart and arch arteries in the chick.

Jonathan Kirby, Joseph H. Davis, D Bockman … (1987)

Mesenchymal derivatives of the neural crest contribute to the connective tissues and blood vessels of the pharyngeal arches, and participate in the septation of the outflow tract of the heart. The present study was designed to determine the nature and timing of alterations in the development of the heart and arch arteries subsequent to diminished neural crest contributions. The neural crest contributing to the three caudalmost pharyngeal arches was ablated bilaterally in chick embryos and compared with sham or unoperated controls. Heart development was studied by scanning electron microscopy. Arch artery development was studied microscopically after intravascular injection of India ink and clearing of the specimen. Neural crest ablation caused morphological changes in most hearts. Hearts in experimental animals commonly were elongate and were subject to inappropriate development of ventricular and atrial areas. A surgical effect delayed the disappearance of arch arteries one and two, and removal of neural crest produced an additional delay. Neural crest ablation caused failure of arch arteries three, four (right), and six to develop to the proper size in some animals. Survival of those whose sixth arch arteries achieved the proper size caused group measurements to reach normal values again by stage 32. Closure of arch arteries in some animals and maintenance in others produced greater variability in experimental animals than in controls. It is significant that heart morphology was altered before septation of the outflow tract normally occurs. This indicates at the least that another factor, such as altered blood flow, contributes to the abnormal development. Altered flow may result from changes in pharyngeal arch mesenchyme and arch artery endothelium.