KidsMD Health Topics

Vascular Ring

  • A vascular ring is a rare cardiovascular birth defect involving an unusual formation of the aorta and/or its surrounding blood vessels.

    What happens if your child has a vascular ring:

    • The trachea (windpipe) and esophagus are completely encircled and compressed by a "ring" formed by these vessels.
    • The compression frequently causes breathing and digestive problems, often starting in infancy.

    Pioneering surgery

    An operation to divide the vascular ring is the treatment of choice. This kind of surgery was pioneered at Boston Children's Hospital in 1945 with the first successful operation on a child with a double aortic arch.
    Today, this procedure can be performed using robotic surgery equipment, allowing your child the benefits of minimally invasive surgery and improved surgical precision.

  • What causes vascular rings?

    Normally, the aorta develops from one in a series of symmetrical arches. By the end of the second month of fetal development, the other arches are naturally broken down or formed into arteries. When a vascular ring occurs, certain arches that should have disappeared still remain and form a ring structure.
    There are several types of vascular rings, but the two most common of these are:

    • Double aortic arch: With this anomaly, there are two aortic arches, one on the left side of the heart, one on the right. Typically, one of the arches, usually the right arch, is dominant and the other is small and underdeveloped. The trachea and esophagus are encircled by the two aortic arches.
    • Right aortic arch with aberrant subclavian and left ligamentum: Normally, the arch of the aorta curves left from the heart. In this kind of vascular ring, the aortic arch curves right. The left subclavian artery, which branches from the aortic arch, passes behind the esophagus, and a small ligament called the ligamentum arteriosus passes between the left subclavian artery and left pulmonary artery, completing the ring.

    What are the symptoms of a vascular ring?

    Since vascular rings encircle and press on the esophagus and trachea, your child may experience symptoms of obstruction. Symptoms range widely depending on how severe the compression is. Typically, if the condition is severe, symptoms occur during infancy, even as early as the first months of life. Common symptoms your child may experience include:

  • Tests

    How are vascular rings diagnosed?
    If your child experiences symptoms of a vascular ring — particularly noisy breathing or a weak pulse — you will probably be referred to a specialist. Your child may undergo some combination of the following tests:
    Chest x-ray - This initial test may enable your doctor to identify the abnormal formation of the aorta. However, this isn't always possible.

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