Home Am I at Risk? Congenital Heart Defects & Heart Failure

Congenital Heart Defects & Heart Failure

What are congenital heart defects?

Congenital (inborn) heart defects are structural abnormalities of the heart that are present at birth. They occur in about 1 in 100 newborns. These defects happen while the baby is developing the heart’s valves, chambers, and blood vessels.

There are many types of congenital heart defects, ranging from simple to complex, small to large, and producing mild to severe symptoms. Simple defects include holes in the inner walls that separate the chambers of the heart (called septal defects), narrowed heart valves (see heart valve disease), and abnormal blood vessels. Complex defects include combinations of one or more simple defects, abnormally placed blood vessels, and other structural abnormalities.

Although congenital heart defects are present at birth, they may not produce symptoms immediately. Symptoms depend on the type and severity of the defect. Milder forms may not produce symptoms at all or not until adulthood. More severe defects can be life-threatening, causing symptoms such as rapid breathing or a bluish discoloration of the skin.

How are congenital heart defects related to heart failure?

Congenital heart defects can disrupt the heart's ability to pump blood over time, leading to heart failure. The most common types of congenital heart defects are abnormal holes in the heart and narrowing of heart blood vessels or valves. Click here for more detailed information on heart valve disease.

A ventricular septal defect (VSD) is a hole in the heart muscle wall that separates the left lower chamber (the left ventricle) from the right lower chamber (right ventricle), allowing oxygen-rich blood to flow from the left ventricle into the right ventricle instead of out to the rest of the body. A VSD is the most common form of congenital heart defect in newborns.2, 27 About 85% to 95% of small VSDs and 10% to 15% of large VSDs may close on their own.27 A large VSD causes the heart to work too hard to pump blood, which can lead to heart failure.

An atrial septal defect (ASD), is a hole in the part of the muscle wall that separates the upper left chamber (left atrium) of the heart from the upper right chamber (right atrium). This allows oxygen-rich blood from the left (larger) atrium to flow into the right (smaller) atrium rather than into the left ventricle (the heart’s main pumping chamber) and the rest of the body. Large ASDs may, over time, stretch and eventually weaken the right side of the heart, setting the stage for heart failure. ASDs occur 2 to 3 times more often in women than in men and account for one third of congenital heart defects in adults.28, 29

Coarctation of the aorta is severe narrowing of the aorta, the main artery that carries oxygen-rich blood from the heart to the rest of the body. This defect causes the heart to pump with more force to get blood through the narrowed aorta. It’s 2 to 5 times more common in men and boys than in women and girls.28

How do congenital heart defects affect my risk of developing heart failure?

About 85% of babies born with heart defects will survive to reach adulthood. However, more than half of adults with congenital heart defects are at medium to high risk for complications, repeat surgeries, and premature death.30

The most common complications for adults with congenital heart defects are heart failure and atrial fibrillation.30, 31 When the chambers, valves, and major blood vessels of the heart don’t form properly, the heart has to work harder to pump out enough blood. Over time, the extra strain can weaken the heart and lead to heart failure. Two thirds of people aged 40 and older with untreated coarctation of the aorta have symptoms of heart failure; no women-specific data are provided.28 Atrial fibrillation, a risk factor for heart failure, occurs more frequently in adults aged 40 or older who have an ASD. Women are more than twice as likely as men to have ASD.28

Other complications include high blood pressure, premature coronary artery disease, and heart attacks, all of which are major risk factors for heart failure. A Dutch study of about 7500 adults (nearly 50% were women) with congenital heart defects found that women had a 33% higher risk than men of having increased blood pressure in the arteries of the lung, which causes the heart to work harder to pump blood.29 Over time, the increased workload weakens the heart until it becomes an inefficient pump.

What can I do to prevent heart failure if I have a congenital heart defect?

The best way to keep from developing heart failure is to treat the inborn defect. You need to make sure you are maintaining a healthy lifestyle, including eating a healthy diet and not smoking, and following any treatment prescribed by your doctor. Some congenital heart defects are mild and don’t need treatment; others need to be treated with medications, surgery, or catheter-based procedures. About 50% to 60% of congenital heart defects are treated surgically in infancy or early childhood.

Surgery can alleviate symptoms and reduce the risk of heart failure by improving the flow of blood, but it does not mean the heart is "normal." About 50% of patients with congenital heart defects in one large US medical center needed two or more surgeries.30

Most adults with congenital heart defects should have lifelong monitoring by a cardiologist (heart specialist). If you have a congenital heart defect and plan to get pregnant, check with your cardiologist to assess your risk. Pregnancy causes the heart to work harder to pump 30% to 50% more blood than normal. The most common heart disease complications in pregnant women with congenital heart defects are heart failure and heart rhythm problems.32


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