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It’s natural for you to be concerned right now about your child’s health—a diagnosis of complete atrioventricular canal defect can be overwhelming. But you can rest assured that at Boston Children's Hospital, your child is in expert hands.
Consistently ranked among the top pediatric hospitals in the United States, Boston Children’s is home to the world's most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.
Atrioventricular canal defect (AV canal or AVC) is a combination of several heart problems that result in a large defect in the center of the heart. The condition is congenital, which means it is present at birth.
The combination of defects includes:
If it’s not treated, AV canal can cause many problems involving the heart and lungs. When there are holes in the heart, blood flow follows the path of least resistance, which usually means that excess blood flows to the right side of the heart and out the pulmonary artery to the lungs. In those with atrioventricular canal defect, the extra volume of blood handled by the right side of the heart and the lungs can be quite significant, sometimes as much as three times the normal amount.
This extra blood flow through the lung blood vessels often results in higher blood pressure in the lungs and the right side of the heart. Over time, the increased flow of blood and the higher pressure can lead to pathologic changes in the lung blood vessels. Over the long term, these changes in the lung blood vessels can become difficult to treat and irreversible.
As the heart is pumping more blood than normal to the lungs, this adds to the amount of work the right ventricle and the heart as a whole have to do. The heart can become larger or dilated over time, and more muscular than normal. Sometimes as the heart becomes larger, the atrioventricular valve becomes more leaky, which makes the heart less efficient.
Because blood is pumped at high pressure through the septal openings, the lining of the right and left ventricles can become irritated and inflamed. Bacteria in the bloodstream will sometimes infect this injured area, causing a serious illness known as bacterial endocarditis.
Genetics may play an important role in the development of atrioventricular canal defect.
Open heart surgery at Children’s has among the highest success rates in the United States among large pediatric cardiac centers. In particular, the methods used to repair AV canal have improved greatly in the past two decades, and the operation has a high likelihood of success.
Complications after surgery are not common, but may include the following:
Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who have had congenital heart disease repair will have an ongoing relationship with their cardiologist. We will treat complications, and will advise on daily-life issues such as exercise and activity levels, nutrition and precautions related to pregnancy.
Surgical techniques for AV canal and its associated defects are continually being refined, and long-term outcomes are continually improving. Nevertheless, patients will need lifelong monitoring and medication, since they’ll always be at some risk for arrhythmias, infections, leaky valves, heart failure or stroke.
A small minority of patients (~10%) will need repeat surgery at some point in later childhood or as an adult, most commonly to address residual mitral valve problems.
Atrioventricular canal defect occurs in two out of every 10,000 live births, equally in boys and girls.
It’s important for parents to know that nothing you’ve ingested—and no virus you’ve had—is responsible for your child’s AV canal.
Genetics may play an important role in the development of atrioventricular canal defect. And some studies have investigated a possible link between the use of retinoic acid (for the treatment of acne and facial wrinkles) while pregnant and the development of atrioventricular canal in the fetus.
The structural cause of AV canal is as follows: When the heart is forming during the first eight weeks of fetal development, it begins as a hollow tube. Over time, partitions that form within the tube eventually become the walls dividing the right side of the heart from the left.
Atrial and ventricular septal defects occur when the partitioning process doesn’t occur completely, leaving openings in the atrial and ventricular walls (septa). The valves that separate the upper and lower heart chambers are formed toward the end of this eight-week period, and often they don’t develop properly. Frequently, instead of two separate AV valves (tricuspid and mitral valve), there is a single large common valve that sits between the upper and lower chambers of the heart.
If your child has any of these symptoms, your pediatrician will probably refer you to a pediatric cardiologist for testing, diagnosis and a determination of treatment.
Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily or is uninterested in eating.
Some studies have investigated a possible link between the use of retinoic acid (a medication used for the treatment of dermatologic problems such as acne and facial wrinkles) while pregnant and the development of atrioventricular canal in the fetus.
Surgical techniques for AV canal and its associated defects are continually being refined, and long-term outcomes are continually improving. Nevertheless, patients will need lifelong monitoring and medication, since they will always be at some risk for arrhythmias, blood clots, infections, leaky valves, heart failure or stroke.
If you’re a teen who’s had surgical repair of a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with medical appointments and procedures … some delay of your natural wish for independence … feeling different … and assuming a lot of personal responsibility for maintaining your own good health.
If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor or counselor to get help.
If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood.
You may need lifelong monitoring and medication, since you’ll continue to be at some risk for arrhythmias, blood clots, infections, leaky valves, heart failure or stroke. Going forward, your cardiologist will also advise you on activity levels, pregnancy issues and certain lifestyle choices.
Fortunately, Boston Children’s can help adults with congenital heart defects. Many adults who were patients as babies or children continue to be monitored by the clinicians who have followed them since childhood.
In addition, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.
BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.
Your child’s cardiologist will offer recommendations for post-operative follow-up care, including:
As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”