Vascular rings: How and Why Placed?

 

Vascular rings

Overview

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:

  • Frequent respiratory infections
  • Wheezing
  • Coughing

Digestive signs and symptoms may include:

  • Trouble swallowing
  • Difficulty feeding
  • Vomiting

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 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.

  • Barium swallow. In this test, your child swallows a substance called barium. X-rays are used to see how the barium moves inside of the esophagus. The barium allows the doctor to see whether or not there's an indentation that may be caused by vascular rings.
  • Upper endoscopy. A long, flexible tube with a camera allows your child's doctor to examine the esophagus. The endoscope is inserted through the mouth into the throat as a tiny camera at the tip of the tube sends images to a video monitor.
  • Bronchoscopy. A bronchoscopy may be done 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 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.


Diagnosis

Your child's doctor may initially suspect a problem because he or she hears a heart murmur during a routine exam. A heart murmur is a sound that occurs when blood flows through the heart or blood vessels fast enough to make a sound that a doctor can hear with a stethoscope.

Most heart murmurs are innocent, meaning that there is no heart defect and the murmur isn't dangerous to your child's health. Some murmurs, however, may mean blood is flowing through your child's heart abnormally because he or she has a heart defect.

Tests to diagnose a congenital heart defect

If it's possible your child has a heart defect, your doctor or your child's doctor may order several tests to see if your child has a heart problem. In addition to a regular physical exam, these could include:

  • Fetal echocardiogram. This test allows your doctor to see if your child has a heart defect before he or she is born, allowing your doctor to better plan treatment. In this test, your doctor performs an ultrasound. The sound waves from the ultrasound are used to create a picture of your baby's heart.
  • Echocardiogram. Your child's doctor may use a regular echocardiogram to diagnose a congenital heart defect after your child has been born.

    In this noninvasive test, your child's doctor performs an ultrasound to produce images of the heart. An echocardiogram allows the doctor to see your child's heart in motion and to identify abnormalities in the heart muscle and valves.

  • Electrocardiogram. This noninvasive test records the electrical activity of your child's heart and can help diagnose heart defects or rhythm problems. Electrodes connected to a computer and printer are placed on your baby's chest and show waves that indicate how your child's heart is beating.
  • Chest X-ray. Your child may have a chest X-ray to see if the heart is enlarged, or if the lungs have extra blood or other fluid in them. These could be signs of heart failure.
  • Pulse oximetry. This test measures how much oxygen is in your child's blood. A sensor is placed over the end of your child's finger to record the amount of oxygen in your child's blood. Too little oxygen could suggest your child has a heart problem.
  • Cardiac catheterization. In this test, a thin, flexible tube (catheter) is inserted into a blood vessel at your baby's groin and guided through it into the heart.

    Catheterization is sometimes necessary because it may give your child's doctor a much more detailed view of your child's heart defect than an echocardiogram. In addition, some treatment procedures can be done during cardiac catheterization.

  • Cardiovascular magnetic resonance imaging (MRI). This type of imaging is becoming increasingly used to diagnose and evaluate congenital heart defects in adolescents and adults. Newer MRI technology provides faster imaging and higher resolution than other methods, such as echocardiography.

Treatment

A congenital heart defect may have no long-term effect on your child's health — in some instances, such defects can safely go untreated. Certain defects, such as small holes, may even correct themselves as your child ages.

Some heart defects, however, are serious and require treatment soon after they're found. Depending on the type of heart defect your child has, doctors treat congenital heart defects with:

  • Procedures using catheterization. Some children and adults now have their congenital heart defects repaired using catheterization techniques, which allow the repair to be done without surgically opening the chest and heart. Catheter procedures can often be used to fix holes or areas of narrowing.

    In procedures that can be done using catheterization, the doctor inserts a thin tube (catheter) into a leg vein and guides it to the heart with the help of X-ray images. Once the catheter is positioned at the site of the defect, tiny tools are threaded through the catheter to the heart to repair the defect.

  • Open-heart surgery. Depending on your child's condition, he or she may need surgery to repair the defect. Many congenital heart defects are corrected using open-heart surgery. In open-heart surgery, the chest has to be opened.

    In some cases, minimally invasive heart surgery may be an option. This type of surgery involves making small incisions between the ribs and inserting instruments through them to repair the defect.

  • Heart transplant. If a serious heart defect can't be repaired, a heart transplant may be an option.
  • Medications. Some mild congenital heart defects, especially those found later in childhood or adulthood, can be treated with medications that help the heart work more efficiently.

    Drugs known as angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs) and beta blockers and medications that cause fluid loss (diuretics) can help ease stress on the heart by lowering blood pressure, heart rate and the amount of fluid in the chest. Certain medications can also be prescribed to help irregular heartbeats (arrhythmias).

Sometimes, a combination of treatments is necessary. In addition, some catheter or surgical procedures have to be done in steps, over a period of years. Others may need to be repeated as a child grows.

Long-term treatment

Some children with congenital heart defects require multiple procedures and surgeries throughout life. Although the outcomes for children with heart defects have improved dramatically, most people, except those with very simple defects, will require ongoing care, even after corrective surgery.

  • Lifelong monitoring and treatment. Even if your child has surgery to treat a heart defect, your child's condition will need to be monitored for the rest of his or her life.

    Initially, your child with a congenital heart defect will be monitored and have regular follow-up appointments with a pediatric cardiologist. As your child grows older, his or her care will transition to an adult congenital cardiologist, who can monitor his or her condition over time. A congenital heart defect can affect your child's adult life, as it can contribute to other health problems. Adults who have congenital heart defects may need other treatments for their condition.

    As your child ages, it's important to remind him or her of the heart condition that was corrected and the need for ongoing, lifelong care by doctors experienced in evaluating and treating congenital heart disease. Encourage your child to keep his or her doctor informed about the heart defect and the procedures performed to treat the problem.

  • Exercise restrictions. Parents of children with congenital heart defects may worry about the risks of rough play and activity even after treatment. Although some children may need to limit the amount or type of exercise, many can participate in normal or near-normal activity.

    Your child's doctor can tell you which activities are safe for your child. If some activities do pose distinct dangers, encourage your child to participate in other activities instead of focusing on what he or she can't do. Although every child is different, most children with congenital heart defects grow up to lead healthy, productive lives.

  • Infection prevention. Depending on the type of congenital heart defect your child had, and the surgery used to correct it, your child may need to take extra steps to prevent infection.

    Sometimes, a congenital heart defect can increase the risk of infections — either in the lining of the heart or heart valves (infective endocarditis). Because of this risk, your child may need to take antibiotics to prevent infection before additional surgeries or dental procedures.

    Children who are most likely to have a higher risk of infection include those whose defect was repaired with a prosthetic material or device, such as an artificial heart valve.

    Ask your child's cardiologist if preventive antibiotics are necessary for your child.

Coping and support

It's natural for many parents to feel worried about their child's health, even after treatment of a congenital heart defect. Although many children who have congenital heart defects can do the same things children without heart defects can, here are a few things to keep in mind if your child has had a congenital heart defect:

  • Developmental difficulties. Because some children who have congenital heart defects may have had a long recovery time from surgeries or procedures, their development may lag behind that of other children their age. Some children's difficulties may last into their school years, and they may have difficulties learning to read or write, as well.
  • Emotional difficulties. Many children who have developmental difficulties may feel insecure about their abilities and may have emotional difficulties as they reach school age.
  • Support groups. Having a child with a serious medical problem isn't easy and, depending on the severity of the defect, may be very difficult and frightening. You may find that talking with other parents who've been through the same situation brings you comfort and encouragement.

Talk with your child's doctor about ways to help you or your child with difficulties related to your child's heart condition. He or she can suggest resources, such as support groups or therapists that may be helpful to you or your child.

Preparing for your appointment

If your child has a life-threatening heart defect, it will likely be detected soon after birth, or possibly before birth as a part of routine exams during pregnancy.

If you suspect your child has a heart defect later in infancy or childhood, talk to your child's doctor. Be prepared to describe your child's symptoms and provide a family medical history, since some heart defects tend to be hereditary.

Your child's doctor may also want to know if the mother of the child had any medical conditions or used any medications or alcohol while pregnant that may have been a risk factor for developing a congenital heart defect.

What you can do

  • Write down any signs and symptoms your child is experiencing, including any that may seem unrelated to heart problems. Write down when each symptom began.
  • Make a list of all medications, vitamins or supplements that the mother of the child has been taking.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions may help you make the most of your time together. You might want to ask the following questions:

  • Are these signs and symptoms related to my family history?
  • What kinds of tests does my child need? Do these tests require any special preparation?
  • Does my child need treatment? If so, when?
  • What is the best treatment?
  • Do you think my child will experience any long-term complications?
  • How will we monitor for possible complications?
  • If I have more children, what are the odds of this condition occurring in them?
  • Are there any brochures or other printed material that I can take home with me? What websites do you recommend visiting?

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

  • When did you first notice your child's symptoms?
  • Can you describe your child's symptoms?
  • When do these symptoms occur?
  • Have the symptoms been continuous or occasional?
  • Do the symptoms seem to be getting worse?
  • Do you have any family history of congenital heart defects?
  • Does anything seem to improve your child's symptoms?
  • Has your child been growing and meeting developmental milestones as expected?

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