Outcome and Prognosis
About 95% of patients who undergo surgical correction of a vascular ring survive long term, and most of these are soon relieved of their symptoms. In those infants with no intracardiac or extracardiac defects, surgery for vascular rings carries essentially no mortality.
Among those with less optimal long-term results are patients with an anomalous left pulmonary artery with and without complete tracheal rings and those with severe associated congenital cardiac defects. In those patients with a severely deformed trachea or tracheomalacia, additional reconstruction procedures may be required in the future.
A number of patients continue to show evidence of some pulmonary function abnormalities years after surgery. Several studies report that measured pulmonary function studies show some degree of airway obstruction in as many as 50% of patients 7-8 years postoperatively. Also, a large number of patients appear to have a pronounced bronchial responsiveness to histamine.
Future and Controversies
Video-assisted thoracic surgical (VATS) techniques have been used for some pediatric thoracic surgical procedures. Several centers have successfully employed this technology for patent ductus arteriosus ligation.
While some reported use of VATS for vascular ring division exists, this remains a controversial area. Most of the cases reported are those in which an atretic arch or a ligamentum arteriosum was divided.
Use of this technology for division of a patent arch remains in question. The main objection to its use in such cases is the increased intraoperative risk of bleeding. The concern is that if vascular clips are applied to a patent arch and the arch is then divided, the amount of recoil of the divided ends that usually occurs may cause one of the clips to dislodge, resulting in severe hemorrhage. Because of the constraints of video-assisted techniques, the surgeon may have difficulty ascertaining when complete occlusion of the arch by the vascular clips has occurred. Because present open techniques provide excellent operative results with extremely low mortality rates and low morbidity, minimally invasive methods will have to provide the same assurance of safety and efficacy to be applicable.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Jane M Eggerstedt, MD, to the development and writing of this article.
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Further Reading
Keywords
aortic arch anomalies; dysphagia lusoria; pulmonary sling; compression of the trachea and/or esophagus; double aortic arch; right aortic arch with left ligamentum arteriosum; right aortic arch with mirror-image branching; left ligamentum arteriosum and left aortic arch with retroesophageal right subclavian artery, right-sided descending aorta, and right ligamentum arteriosum; anomalous innominate artery; retroesophageal right subclavian artery with left-sided aorta and left ligamentum arteriosum, ring-sling complex, stridor, cyanosis, respiratory distress, apnea, brassy cough, asthma, recurrent pneumonia, dysphagia, difficulty with feedings, vascular rings
