Vascular rings are malformations of the aortic arch in the main blood vessel that leads from the heart. Because of the malformation, the aortic arch and its branches partly or completely encircle the windpipe (trachea), the esophagus or both. The esophagus is the tube that leads from your mouth to your stomach.
These defects are present at birth (congenital), but symptoms can occur in infancy or later in life. Symptoms are caused by some portion of the ring pressing against the trachea or esophagus or both.
Some people with vascular rings don't have symptoms, but vascular rings that press against the trachea and esophagus can lead to breathing and digestive problems. Respiratory signs and symptoms may include:
Digestive signs and symptoms may include:
People with vascular rings may also have other congenital heart defects.
To diagnose vascular rings and rule out other conditions that may be causing the symptoms, your doctor may review your child's signs and symptoms, do a physical exam, and order tests that may include:
Imaging tests. A chest X-ray can show which side of the body the aortic arch is on, and any changes in the trachea that may suggest that a vascular ring is present.
Other imaging tests may be done to diagnose vascular rings, such as an echocardiogram or a CT angiogram or MRI. Doctors may also use these tests to plan treatment.
Surgery is usually needed to treat vascular rings that are pressing against the trachea or esophagus and to avoid complications. Surgeons divide the vascular rings to release the vessels pressing against the trachea and esophagus. The procedure is often conducted during open surgery, but in some cases a minimally invasive approach may be used.
Different surgical approaches may be used, depending on the specific malformation.
Children and adults with vascular rings may need lifelong care and regular follow-up appointments with specialists to monitor their conditions. Multiple specialists, including congenital cardiologists, doctors trained in lung conditions (pulmonologists), doctors trained in digestive conditions (gastroenterologists), and doctors trained in ear, nose and throat conditions may be involved in their care.
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