Children's Heart Institute
"Vascular rings" refer to a variety of similar malformations in the chest in which the defect is in the blood vessels. The problem resulting from vascular rings is compression of the windpipe (trachea) and food tube (esophagus). There are many varieties of vascular rings - the double aortic arch is the most common.
The main blood vessel which takes blood from the heart to the body is called the aorta. The aorta, shaped like a candy cane, arises from the heart and travels up toward the head. It then makes a gentle half-circle turn and heads towards the back. The blood vessel then heads straight down to supply the belly and the legs. During the gentle half-circle turn, the aorta normally passes to the left of the trachea and esophagus. Early in fetal life, however, the aorta passes on both sides of the trachea and esophagus. The aorta arises from the heart, splits into two limbs, passes on both sides of the trachea & esophagus, and then reconnects to go to the belly. Thus, the trachea and esophagus are surrounded by a "ring" of aorta.
When neither limb disappears, the trachea and esophagus continue to be surrounded by ring aorta during fetal development and after birth. The problem is not blood flow, but rather compression on the trachea and esophagus. This variety of vascular ring is called a "double aortic arch." Sometimes, the ring of aorta is not tight, and there is no real harm to the patient. Other times, the "ring" can be quite tight, causing significant narrowing of the trachea and/or esophagus.
The only treatment for vascular rings is surgery. Surgery is performed through an incision in the left side of the chest. "Opening" the ring means to divide one of the blood vessels to relieve the compression on the trachea and esophagus. The esophagus is rarely permanently damaged from growing and developing with compression. The trachea, however, can remain narrowed despite having the vascular ring opened. The severity of tracheal narrowing varies from patient to patient, and should be discussed with your doctors.
There are other arrangements of blood vessels which result in a vascular ring, other than a double aortic arch. As common features, they all have:
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