When your baby was born, your baby's height, weight and head circumference were recorded, and these measurements are taken at all regularly scheduled appointments with your child's doctor. He or she will repeat these measurements during a special appointment and determine if there has been any delay in growth.
Your baby's doctor will also listen to your or your baby's lungs to assess his or her breathing and the possibility of fluid in the lungs. The doctor will listen to your or your baby's heart to determine if there are irregular heartbeats (arrhythmia) or an abnormal sound caused by turbulent blood flow (heart murmur).
For babies with truncus arteriosus, much of their medical care is provided by a pediatric cardiologist along with a pediatric cardiac surgeon and often a whole team of specialized staff. For adults with truncus arteriosus, much of their medical care is provided by an adult congenital cardiologist along with a congenital cardiac surgeon and often a whole team of specialized staff.
In order for the pediatric or adult congenital cardiologist to check the condition of your or your baby's heart and make a diagnosis, he or she will order one or more tests:
Echocardiogram. An echocardiogram shows the structure and function of your or your baby's heart. A transducer that emits high-pitched sound waves is moved across the skin over your or your baby's heart. The sound waves echo off internal structures, producing images on a monitor.
In a baby with truncus arteriosus, the echocardiogram reveals the single large vessel leading from the heart, a hole in the wall between the left and right ventricles, and abnormalities in the valve between the large vessel and the ventricles. The test can also show how much blood is flowing to your baby's lungs, and whether there's a risk of high blood pressure in the lungs.
- X-ray. An X-ray exam uses radiation to produce still images of internal organs and structures. A chest X-ray of you or your baby can show the size of the heart, abnormalities in the lungs and excess fluid in the lungs.
Infants with truncus arteriosus must have surgery. Multiple procedures or surgeries may be necessary, especially as your child grows. Medications may be given before surgery to help improve the health of the heart.
Children and adults with surgically repaired truncus arteriosus must have regular follow-up with their cardiology team.
Medications that may be prescribed before surgery include:
- Diuretics. Often called water pills, diuretics increase the frequency and volume of urination, preventing fluid from collecting in the body, which is a common effect of heart failure.
- Ionotropic agents. This type of medication strengthens the heart's contractions.
Most infants with truncus arteriosus have surgery within the first few weeks after being born. The exact procedure will depend on your baby's condition. Most commonly your baby's surgeon will:
- Close the hole between the two ventricles with a patch
- Separate the upper portion of the pulmonary artery from the single large vessel
- Implant a tube (conduit) and valve to connect the right ventricle with the upper portion of the pulmonary artery — creating a new, complete pulmonary artery
- Reconstruct the single large vessel and aorta to create a new, complete aorta
After corrective surgery, your child will need lifelong follow-up care with a cardiologist to monitor his or her heart health. The cardiologist may recommend that your child limit physical activity, particularly intense competitive sports.
Your child will need to take antibiotics before dental procedures and other surgical procedures to prevent infections.
Because the artificial conduit does not grow with your child, follow-up surgeries to replace the conduit valve are necessary as he or she gets older.
Newer minimally invasive procedures use a cardiac catheter to avoid the need for traditional heart surgery as your child grows or previously placed artificial valves deteriorate. The catheter is inserted into a blood vessel in the leg that is then threaded slowly up to the heart to replace the conduit.
In addition, cardiac catheterization with an inflatable balloon tip can be used to open up an obstructed or narrowed artery, which may delay the need for follow-up surgery.
Women who've had surgery to repair truncus arteriosus in infancy need to be evaluated by a cardiologist with expertise in adult congenital heart defects and an obstetrician specializing in high-risk pregnancies before attempting to become pregnant.
Depending on the level of lung damage that occurred before surgery, pregnancy may or may not be recommended. In addition, some drugs taken for heart problems can be harmful to an unborn baby.
Coping and support
Caring for a baby with a serious heart problem, such as truncus arteriosus, can be challenging. Here are some strategies that may help make it easier:
- Seek support. Ask for help from family members and friends. Contact nonprofit organizations, such as the American Heart Association, Adult Congenital Heart Association or Mended Hearts for information about support groups in your area.
Record your baby's health history. Write down your baby's diagnosis, medications, surgery and other procedures, the dates they were performed, your pediatric cardiologist's name and phone number, and any other important information about your baby's care.
It's also helpful to include a copy of the operative report from your child's surgeon in your records. This information will help you recall the care your child has received, and it will be useful to doctors unfamiliar with your child who need to review his or her health history. It will also be helpful when your child moves from pediatric to adult health care.
Talk about your concerns. You may worry about the risks of vigorous activity, even after your child has had corrective surgery. Talk with the cardiologist about which activities are safe for your child.
If some activities are off-limits, encourage your child in other pursuits rather than focusing on what he or she can't do. If other issues about your child's health concern you, discuss them with your child's primary care doctor or pediatric cardiologist.
Preparing for your appointment
When you contact your baby's doctor about your concerns, he or she may ask you to schedule an appointment or may immediately refer you to a heart specialist for children (pediatric cardiologist). This will depend on the severity of the signs and symptoms you have described.
What you can do
If possible, compile a thorough medical history from both sides of your baby's family. Some heart problems are inherited, so it's helpful if you can let your doctor know if anyone in your child's family has ever had early heart problems.
It's also helpful to prepare a list of questions ahead of time so that you can make the most of your time together with your baby's doctor. Here's a sampling of questions you might want to ask:
- What kinds of tests will my child undergo?
- What are the options for treatment? What are the possible complications of treatment?
- What is the long-term outlook for my child?
- What impact would a heart defect or the subsequent treatment have on another condition my baby has?
- If I have another baby in the future, what are the chances of this happening again?
- Are there any brochures or other printed material that I can take home with me? What websites do you recommend visiting?
In addition to the questions that you've prepared to ask your baby's doctor, don't hesitate to ask questions that may come up during your appointment.
What to expect from your doctor
Your child's doctor will likely ask you the following questions:
- Does your baby ever appear blue?
- How long are feeding times, or how much does your baby drink?
- How often and how long is your baby sleeping?
- How does your baby respond to touch?
- Have you noticed changes in any of these behaviors?