Complications

Some potential complications that can occur with a congenital heart defect include:

• Congestive heart failure. This serious complication may develop in babies who have a significant heart defect. Signs of congestive heart failure include rapid breathing, often with gasping breaths, and poor weight gain.
• Slower growth and development. Children with more-serious congenital heart defects often develop and grow more slowly than do children who don't have heart defects. They may be smaller than other children of the same age and, if the nervous system has been affected, may learn to walk and talk later than other children.
• Heart rhythm problems. Heart rhythm problems (arrhythmias) can be caused by a congenital heart defect or from scarring that forms after surgery to correct a congenital heart defect.
• Cyanosis. If your child's heart defect causes oxygen-poor blood to mix with oxygen-rich blood in his or her heart, your child may develop a grayish-blue skin color, a condition called cyanosis.
• Stroke. Although uncommon, some children with congenital heart defects are at increased risk of stroke due to blood clots traveling through a hole in the heart and on to the brain.
• Emotional issues. Some children with congenital heart defects may feel insecure or develop emotional problems because of their size, activity restrictions or learning difficulties. Talk to your child's doctor if you're concerned about your child's moods.
• A need for lifelong follow-up. Children who have heart defects should be mindful of their heart problems their entire lives, as their defect could lead to an increased risk of heart tissue infection (endocarditis), heart failure or heart valve problems. Most children with congenital heart defects will need to be seen regularly by a cardiologist throughout life.

Prevention

Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child's overall risk of birth defects and possibly heart defects, too, such as:

• Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby's heart development. Be sure to get vaccinated before you try to conceive.
• Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor.
• Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don't take any drugs, herbs or dietary supplements without consulting your doctor first. Don't smoke or drink alcohol during pregnancy.
• Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.

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 an 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?

Overview

Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that's present at birth. That means it's a congenital heart defect.

Other names for this condition are:

• Total anomalous pulmonary venous connection.
• TAPVC.

In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart.

In a typical heart, oxygen-rich blood flows from the lungs to the upper left heart chamber, called the left atrium. Blood then goes to the rest of the body.

In TAPVR, the connection of veins is changed. Blood flows through the upper right heart chamber, called the right atrium. This change in blood flow causes oxygen-poor blood to mix with oxygen-rich blood. As a result, blood flowing to the body doesn't have enough oxygen.

The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease.

Symptoms

Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby's skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see.

Symptoms of TAPVR may appear soon after birth. But some people don't have symptoms until later in life.

When to see a doctor

Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child's healthcare professional.

Causes

Most congenital heart defects are caused by changes that happen early as the unborn baby's heart is developing before birth. An unborn baby is also called a fetus. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known.

Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

Risk factors

Possible risk factors for congenital heart defects, including total anomalous pulmonary venous return (TAPVR), may include:

• Rubella, also called German measles. Having rubella during pregnancy can change how the baby's heart develops. A blood test can be done before pregnancy to see if you're immune to rubella. If you're not, you can get a vaccine.
• Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how the baby's heart grows during pregnancy. Diabetes that develops during pregnancy is called gestational diabetes. Gestational diabetes generally doesn't increase the risk of congenital heart disease.
• Genetics. Although TAPVR doesn't usually run in families, changes in genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions.
• Smoking. If you smoke, quit. Smoking during pregnancy or exposure to secondhand smoke increases the risk of congenital heart defects in the baby.
• Alcohol use. Drinking alcohol during pregnancy has been linked to heart conditions in the baby.
• Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart defects. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk to your healthcare team about the medicines you take.

Diagnosis

To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard.

An echocardiogram is the test used to diagnose total anomalous pulmonary venous return. It uses sound waves to create images of the heart as it beats. An echocardiogram can show the pulmonary veins, any holes in the heart and the size of the heart chambers. It also shows blood flow through the heart and heart valves.

Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

Treatment

Total anomalous pulmonary venous return (TAPVR) is treated with surgery. The surgery usually is done when a child is a baby. The timing of surgery depends on whether there's a blockage. To repair the heart, surgeons connect the pulmonary veins to the left upper heart chamber. They also close the hole between the upper heart chambers.

A person with TAPVR needs regular health checkups for life to check for infection, blockages or irregular heartbeats. A doctor trained in congenital heart diseases should provide care. This type of healthcare professional is called a congenital cardiologist.