Complications

Congenital heart disease complications that might develop years after the initial treatment include:

• Abnormal heart rhythms (arrhythmias). Arrhythmias occur when the electrical impulses that coordinate heartbeats don't function properly, causing your heart to beat too fast, too slowly or irregularly. In some people, severe arrhythmias can cause sudden cardiac death if not treated.

• Heart infection (endocarditis). Your heart comprises four chambers and four valves, which are lined by a thin membrane called the endocardium. Endocarditis is an infection of this inner lining, which generally occurs when bacteria or other germs enter your bloodstream and lodge in your heart. Untreated, endocarditis can damage or destroy your heart valves or trigger a stroke.

  If you have an artificial (prosthetic) heart valve or your heart was repaired with prosthetic material, or if your heart defect wasn't completely repaired, your doctor might prescribe ongoing antibiotics to lower your risk of developing endocarditis.

• Stroke. Stroke occurs when the blood supply to a part of your brain is interrupted or severely reduced, depriving brain tissue of oxygen. A congenital heart defect can allow a blood clot to pass through your heart and travel to your brain.

  Certain heart arrhythmias also can increase your chance of blood clot formation leading to a stroke.

• Heart failure. Heart failure, also known as congestive heart failure, means your heart can't pump enough blood to meet your body's needs. Some types of congenital heart disease can lead to heart failure.

  Over time, certain conditions such as coronary artery disease or high blood pressure gradually sap your heart of its strength, leaving it too weak or too stiff to fill and pump efficiently.

• Pulmonary hypertension. This is a type of high blood pressure that affects the arteries in your lungs. Some congenital heart defects cause more blood to flow to the lungs, causing pressure to build and making your heart work harder. This eventually causes your heart muscle to weaken and sometimes to fail.
• Heart valve problems. In some types of congenital heart disease, the heart valves are abnormal.

Coping and support

One important thing to do if you're an adult with congenital heart disease is to become educated about your condition. Topics you should become familiar with include:

• The name and details of your heart condition and its past treatment
• How often you should be seen for follow-up care
• Information about your medications and their side effects
• How to prevent heart infections (endocarditis), if necessary
• Exercise guidelines and work restrictions
• Birth control and family planning information
• Health insurance information and coverage options
• Dental care information, including whether you need antibiotics before major dental procedures
• Symptoms of your congenital heart disease and when you should contact your doctor

Many adults with congenital heart disease lead full, long and productive lives. But it's important not to ignore your condition. Become informed about your disease; the more you know, the better you'll do.

Preparing for an appointment

If you have a congenital heart defect, make an appointment with your doctor for follow-up care, even if you haven't developed complications. You'll likely be referred to a doctor trained in diagnosing and treating heart conditions (cardiologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as restrict your diet or fast. Make a list of:

• Your symptoms, if any, including any that may seem unrelated to congenital heart disease, and when they began
• Key personal information, including a family history of heart defects and treatment you received as a child
• All medications, vitamins or other supplements you take and their doses
• Questions to ask your doctor

Take a family member or friend with you, if possible, to help you remember the information you get. For congenital heart disease, questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• What tests do I need?
• What treatments are available? Which do you recommend for me?
• Are there diet or activity restrictions I need to follow?
• How often should I be screened for complications from my heart defect?
• I have other health conditions. How can I best manage these conditions together?
• Are there brochures or other printed material that I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

• Do your symptoms come and go, or do you have them all the time?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• What, if anything, worsens your symptoms?
• What's your lifestyle like, including your diet, tobacco use, physical activity and alcohol use?

Overview

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

Other names for this condition are:

• PAPVR (partial anomalous pulmonary venous return).
• PAPVC (partial anomalous pulmonary venous connection).

In this condition, some of the blood vessels of the lungs attach to the wrong place in the heart. These blood vessels are called the pulmonary veins.

In a typical heart, oxygen-rich blood returns from the lungs to the upper left heart chamber, called the left atrium. Then the blood goes to the body.

In PAPVR, blood flows from the lungs into the upper right heart chamber, called the right atrium, and then goes to the lungs, even though that blood already has oxygen. This means the right side of the heart has to handle extra blood flow. This may cause swelling of the right heart chambers.

Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur.

Symptoms

Symptoms of partial anomalous pulmonary venous return (PAPVR) can include trouble breathing or fatigue. Sometimes, there are no noticeable symptoms.

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 a heart condition, call your child's healthcare professional.

Call your baby's healthcare professional if the baby has trouble breathing or other symptoms of PAPVR.

Causes

The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby's heart is forming before birth. An unborn baby also is called a fetus.

Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby.

Risk factors

What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include:

• Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how the unborn baby's heart forms. Diabetes that develops during pregnancy is called gestational diabetes. It generally doesn't increase a baby's risk of congenital heart defects.
• Genetics. Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.
• Rubella, also called German measles. Having rubella during pregnancy can cause changes in an unborn baby's heart. 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.
• Some medicines. Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with a healthcare professional about the medicines you take.
• Smoking. If you smoke, quit. Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions.
• Alcohol use. Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby.

Diagnosis

To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard if there is a hole in the heart.

PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life.

Tests

Tests are needed to diagnose partial anomalous pulmonary venous return (PAPVR).

An ultrasound of the heart called an echocardiogram can sometimes confirm PAPVR. This test uses sound waves to make pictures of the beating heart. An echocardiogram often shows the pulmonary veins, the size of the heart chambers and any holes in the heart. It also measures the speed of blood flow.

Sometimes, an echocardiogram may not detect PAPVR. Other tests such as a CT scan or MRI may be used.

Treatment

Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if:

• A lot of oxygen-rich and oxygen-poor blood mixes in the heart.
• The right side of the heart is a lot larger than usual.

If you don't have symptoms, surgery may not be needed. If surgery for another heart condition is needed, surgeons may repair PAPVR at the same time.

There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options. During repair surgery, the heart surgeon will:

• Reconnect the pulmonary veins to the left upper heart chamber.
• Close any holes in the heart.

A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It's best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.