Vascular rings

Vascular rings are malformations of the aortic arch in the main blood vessel that leads from the heart (aorta) that partly or completely encircle the windpipe (trachea) or the tube that leads from your mouth to your stomach (esophagus) or both. These defects are present at birth (congenital), but symptoms can occur either in infancy or later in life. Symptoms are caused by some portion of the ring pressing against the trachea or esophagus or both.

Vascular rings that press against the trachea and esophagus can lead to breathing and digestive problems. Respiratory signs and symptoms may include frequent respiratory infections, wheezing or coughing. Digestive signs and symptoms may include trouble swallowing, difficulty feeding or vomiting. Some people don't experience symptoms.

People with vascular rings may also have other congenital heart defects.

Diagnosis

To diagnose vascular rings and rule out other conditions that may be causing the symptoms, your doctor may review your signs and symptoms, conduct a physical exam, and order a chest X-ray. 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 conducted to diagnose vascular rings, such as an echocardiogram or a CT angiogram. Doctors may also use these tests to plan treatment.

In some cases, doctors may perform a barium swallow. In this test, you swallow a substance called barium, and doctors watch you swallow during an X-ray in order to view the inside of the esophagus.

A bronchoscopy may be performed to evaluate the cause of symptoms and to determine the location and severity of compression against the trachea. In this test, a doctor inserts a small, flexible tube through the mouth or nose into the lungs. A light and a small camera attached to the bronchoscope allow the doctor to look inside the trachea and the lungs' airways.

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 circles 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. (1, p8) 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.

March 06, 2018
References
  1. Juraszek AL, et al. Vascular rings and slings. https://www.uptodate.com/contents/search. Accessed Sept. 19, 2017.
  2. Licari A, et al. Congenital vascular rings: A clinical challenge for the pediatrician. Pediatric Pulmonology. 2015;50:511.
  3. Kliegman RM, et al. Other congenital heart and vascular malformations. In: Nelson Textbook of Pediatrics. 20th ed. Philadelphia, Pa.: Elsevier; 2016. https://www.clinicalkey.com. Accessed Sept. 19, 2017.
  4. Backer CL, et al. Vascular rings. Seminars in Pediatric Surgery. 2016;25:165.
  5. Bronchoscopy. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/health-topics/topics/bron#. Accessed Sept. 27, 2017.
  6. Johnson JN (expert opinion). Mayo Clinic, Rochester, Minn. Oct. 30, 2017.