Invited Commentary: Vascular Rings Are Common

As the new Editor-in-Chief of the World Journal for Pediatric and Congenital Heart Surgery, I could not resist the opportunity to write a commentary on the manuscript, “Isolated Vascular Rings are Common Cardiovascular Malformations,” which is a review of one of my core clinical areas of interest and expertise.

Dr Evans and colleagues are to be congratulated for their comprehensive review of the diagnosis of vascular rings on fetal echocardiograms performed in Southern Nevada. ¹ Their analysis reveals details about the incidence of vascular rings that have not been previously studied in such a detailed fashion. The fetal diagnosis of vascular rings, of course, yields an opportunity for altering the natural history of a congenital cardiac lesion that was previously diagnosed only after the onset of symptoms. This new paradigm allows us to pause and take advantage of this newfound opportunity to plan for optimal management of the infant at birth.

In their comprehensive review, the authors have found that isolated vascular rings are actually common cardiovascular malformations. With prenatal detection rates approaching 90%, the prevalence figure for isolated vascular rings is very similar to that of the common cyanotic congenital cardiac defect, Tetralogy of Fallot. In particular, the three-vessel trachea view on which they have trained general obstetric sonographers has led to the near universal detection of vascular rings in their fetal population. Once a fetal diagnosis has been obtained, the strategic management of the child can begin.

When I was in Chicago, we also noted the dramatic increase in the number of patients with a fetal diagnosis of vascular rings after the widespread adoption of the three-vessel trachea view. Our substantial institutional history of surgical intervention on children with vascular rings led us to recommend a management algorithm after a fetal diagnosis is made. ²

There are several key management recommendations that have developed over time. If the child has a double aortic arch or pulmonary artery sling, we recommend delivery at a center that could potentially deal with severe neonatal respiratory distress in the first days of life. All of these patients should have a comprehensive echocardiogram soon after birth. If the child demonstrates symptoms of a vascular ring, we recommend advanced cross-sectional imaging with a computed tomography (CT) scan followed by surgical intervention. If the child is asymptomatic, we recommend a CT scan at two to four months of age, when the child can be fed and swaddled and have the scan without general anesthesia. The results of that scan can then be used for management planning.

Another important finding from this analysis is that, in reality, right aortic arch vascular rings are more common than double aortic arch vascular rings. For many years, we thought the double aortic arch was the most common type of vascular ring. This, in retrospect, is secondary to the fact that patients with double aortic arch are much more frequently symptomatic, and therefore, come to clinical attention. The current study notes a preponderance of right aortic arch as the most common type of vascular ring. The fact that the right aortic arch is more commonly diagnosed but not always clinically discovered, leads to the realization that over time some of these patients with a right aortic arch do not develop symptoms and potentially can be observed. I personally have a handful of patients with a right aortic arch who have been followed for decades with no symptoms and no bronchoscopic evidence of more than mild external tracheal compression. Again, this is where advanced cross-sectional imaging will help stratify these patients into those who will require operative intervention and a select few who can simply be observed.

Dr Evans and his group are to be congratulated for their initiative in drawing attention to the prenatal diagnosis of vascular rings. They have discovered that vascular rings are indeed common, and right aortic arch variants are the most common. In the current era, the management of these infants can now be started at the time of fetal diagnosis followed by appropriately timed advanced imaging and treatment protocols initiated prior to the child developing severe symptoms. Using this information, clinicians have an opportunity to alter the natural history of this congenital vascular anomaly.