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[Congenital aortic arch anomalies and vascular rings (author's transl)]
Insights
Aortic arch anomalies, arising in early fetal development, can cause significant tracheo-esophageal compression. Surgical intervention offers a good prognosis for infants, with most patients experiencing symptom resolution post-operation.
Area of Science:
- Embryology and Developmental Biology
- Cardiovascular Surgery
- Pediatric Cardiology
Context:
- Aortic arch anomalies are congenital malformations occurring within the first trimester of intrauterine development.
- While many aortic arch anomalies are asymptomatic, approximately one-third lead to tracheo-esophageal compression, presenting symptoms shortly after birth.
Purpose:
- To review the embryological basis, classification, and clinical management of aortic arch anomalies.
- To evaluate the surgical outcomes for infants and children with symptomatic aortic arch anomalies.
Summary:
- Surgical correction involves procedures such as dividing atretic/hypoplastic arch segments, aberrant subclavian arteries, or the ductus arteriosus to relieve compression.
- The study analyzed 17 patients with various aortic arch anomaly classifications, including double aortic arch and aberrant subclavian arteries.
- Post-operative recovery typically leads to normalization of the trachea and esophagus within months to over a year, with no reported cases of reoperation for secondary strictures.
Impact:
- Surgical treatment for symptomatic aortic arch anomalies demonstrates a favorable prognosis, even in neonates.
- Effective surgical management ensures long-term symptom relief and normal development for the majority of affected pediatric patients.
Abstract:
The anomalies of the aortic arch are consequences of malformations in the first twelve weeks of the intrauterine life. Embryologically it is possible to explain and classify all of them. Most remain asymptomatic and do not need treatment. About one third of all anomalies are causing tracheo-esophageal compression with consequent complaints. In most cases the symptomatology starts in the first days of life. Some of them can be temporarily treated conservatively. The prognosis of patients, who need surgery is good, even in small infants. Surgery consists mainly in section of the atretic or hypoplastic part of the double aortic arch, section of the aberrant subclavian artery, section of the Botal ligament or duct and liberation of the trachea and esophagus. A normal configuration of trachea and esophagus is reached after several months or even more than a year. There are no reports on reoperations for secondary strictures. In the University Hospital of Nijmegen and Leiden we operated on 17 patients. There were 8 patients of group I A --- Edwards classification -- double aortic arch with both aortic arches open and left Botal duct or ligament; 4 patients of group I B, double aortic arch with one atretic aortic arch with a left Botal ligament; 3 patients of group II B, with a left aortic arch and aberrant right subclavian artery and a left Botal ligament; 2 patients of group III B with a right aortic arch and an aberrant left subclavian artery and a left Botal duct or ligament. Of our 17 patients one died during the operation because of a haemorrhage and overtransfusion; an other one died three weeks postoperatively from an endotracheal bleeding. All the other patients are without complaints and remained so.