Understanding Adult Congenital Heart Disease

A young female patient sits on an exam table talking to a female doctor.
More than 1 million adults in the United States have congenital heart disease. Your heart condition may have been treated when you were a child. Regular checkups with your doctor can help make sure you stay healthy as an adult.

If you were born with a heart problem, it can affect you throughout your life.

As an adult, you may wonder if symptoms you now feel are connected to your heart condition. You might not know if your doctors will understand the long-term impact of your childhood heart treatment.

That’s why it’s important for adults with congenital heart disease to see experts like the doctors at the OHSU Knight Cardiovascular Institute.

Who has adult congenital heart disease?

There are more than 1.6 million adults in the United States with congenital heart disease.

Congenital heart disease is the most common birth defect in the U.S. Thanks to major advances in medical and surgical care, more than 90% of children born with heart defects live well into adulthood.

Learn more about congenital heart disease in children.

Types of adult congenital heart disease 

There are more than 40 types of congenital heart disease. Some cause mild symptoms, while others are more severe.

If you have a congenital heart condition, you were probably diagnosed soon after you were born or as a child. Your condition was probably treated and managed during your childhood. But your condition can continue to affect your health when you’re an adult. 

Aorta and blood vessel conditions

Anomalous coronary artery

Your coronary artery is in the wrong place. This defect is often detected in babies. Sometimes the defect does not cause problems until adulthood. Anomalous coronary artery can be treated with medication or surgery.

Coarctation of the aorta

Your aorta is the largest blood vessel in your body. It sends blood from your heart to the rest of your body. If part of the aorta is too narrow, it makes it hard for blood to pass through. This can strain your heart muscle and cause high blood pressure. Doctors treat coarctation of the aorta by widening the narrowed part through surgery or catheterization.

Patent ductus arteriosis

Your aorta carries blood from your heart to the rest of your body. But before birth, there is also a connection to your lungs through a blood vessel called the ductus arteriosis. Normally, it closes up in a few days after birth. If not, doctors call it patent ductus arteriosus, or PDA. If you had PDA as a baby, you may have needed a procedure to close it.

Peripheral pulmonic stenosis

The pulmonary valve lets blood move from the heart to the lungs. In some people, the opening of the valve is too narrow. This forces the heart to work harder to pump blood. Mild forms usually need no treatment. In more serious cases, catheterization can open the valve.

Total or partial anomalous pulmonary venous return

When people are born with total anomalous pulmonary venous connection (TAPVR), all four of the blood vessels that bring blood back from the lungs connect to the heart in the wrong place. Most babies with this defect have surgery soon after birth. In people with partial anomalous pulmonary venous connection (PAPVR), only some of these veins connect to the wrong side of the heart. If you have a mild case, you may need no treatment. In more serious forms of PAPVR, you probably had surgery as a baby.

Vascular ring

The aorta carries blood from your heart to the rest of the body. Some people are born with their aorta wrapped around their windpipe and/or esophagus. This can affect breathing and swallowing. 

Other aorta or blood vessel conditions

Other aorta or blood vessel conditions

Heart valve conditions

Aortic stenosis and regurgitation

Blood exits your heart through your aortic valve. If your aortic valve is too narrow, it can cause aortic stenosis. This means the lower left chamber (ventricle) of the heart has to work harder. An abnormal aortic valve can also cause aortic regurgitation. When the valve doesn’t shut tightly, blood can leak back into the heart from the aorta, causing it to work harder. Both conditions can weaken the heart over time.

Ebstein’s anomaly

Your heart has four different chambers. The “door” between the upper right and lower right chambers is called the tricuspid valve. If you were born with Ebstein's anomaly, your tricuspid valve is not shaped correctly. It is also a bit lower than normal. These differences make it harder for blood to flow normally in the heart. If you have a severe Ebstein’s anomaly, you may need surgery.

Pulmonary valve stenosis and regurgitation

The chamber on the lower right side of your heart (the right ventricle) sends blood into your lungs. There, the blood picks up oxygen to take through your body. If the pulmonary valve (doorway) between the right ventricle and the lungs is too narrow, it can stop blood from flowing through properly. This condition is called pulmonary valve stenosis. If the valve lets blood leak back into the heart, this is called pulmonary valve regurgitation. 

Other structural heart conditions

Atrial septal defect

If you were born with an atrial septal defect, there was a hole in the wall between the top two chambers (atria) of your heart. Over time, this hole may cause the heart to work harder, increasing the risk of heart failure and high blood pressure. Atrial septal defects often have no symptoms and can go undetected for years. Your doctor may recommend closing the hole either through surgery or a cardiac catheterization. 

Atrioventricular canal defects

Atrioventricular canal defect, or AV canal defect, is a combination of problems with your heart structure. First, there are holes in the walls between the chambers (sections) of your heart. Also, there are problems with the valves that let blood flow back and forth between chambers. Many people born with AV canal defects have surgery as a child. If not, you may be able to have surgery as an adult. 

Hypoplastic left heart syndrome

In this condition, the left side of the heart is underdeveloped at birth. The aorta, left ventricle and valves are not formed or are too small. Babies born with hypoplastic left heart syndrome usually have three operations over several years to allow the right ventricle to take over for both sides.

Tetralogy of Fallot

Tetralogy of Fallot is a combination of four heart problems:

  • There’s a hole in the wall between the lower ventricles (sections) of your heart.
  • Blood flow from your heart to your lungs is partly blocked.
  • The aorta, the largest blood vessel in your body, is slightly misplaced.
  • The lower right ventricle (section) of your heart muscle is abnormally thick.

Most people born with this condition have surgery when they are very young.  

Transposition of the great arteries

In people born with this defect, the two main arteries that carry blood out of the heart—the pulmonary artery and the aorta—are switched in position. This causes blood to circulate through the heart and lungs the wrong way. Transposition of the great arteries is usually repaired within the first weeks of life. As an adult, you may need follow-up procedures and a pacemaker or medicines to control your heart rhythm and blood pressure.

Ventricular septal defect

A ventricular septal defect (VSD) is a hole in the wall separating your lower heart chambers (ventricles). If you were born with a severe VSD, you probably had surgery as a young child. If the hole is small, or if it was fixed when you were a child, you may have few symptoms. If your VSD was repaired in adulthood, you may need to see a congenital heart disease specialist for regular check-ups.

Less common conditions

  • Cor triatriatum
  • Double outlet right ventricle
  • Single ventricle heart defects, such as tricuspid atresia
  • Truncus arteriosus  

Genetic conditions affecting the heart and blood vessels

  • Loeys-Dietz syndrome
  • Marfan syndrome
  • Noonan syndrome
  • Scimitar syndrome
  • Shone syndrome
  • Turner syndrome
  • Williams syndrome

Learn more about heart-related genetic syndromes in children

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