What is acyanotic heart disease?

Congenital heart disease (CHD) is any abnormal heart structure (defect) present at birth. There are two types of CHD:

• Cyanotic congenital heart disease: Cyanotic heart disease involves heart defects that reduce the amount of oxygen delivered to the rest of the body.
• Acyanotic congenital heart disease: With this type of heart defect, blood contains enough oxygen, but it’s pumped throughout the body abnormally. These defects don’t interfere with the amount of oxygen or blood delivered to the rest of the body.

How common is acyanotic heart disease?

Congenital heart disease affects 8 to 9 per 1,000 live births. Approximately 75% are acyanotic.

What are the types of acyanotic heart disease?

There are several kinds of acyanotic congenital heart defects:

• Aortic stenosis: The aorta is the main artery that carries blood away from your heart to the rest of the body. The aortic valve sits between the heart and aorta. It opens and closes with each heart cycle to let blood out of the heart and keep blood from leaking back into the heart. When the aortic valve becomes narrowed and doesn’t open well, it’s called aortic stenosis.
• Atrial septal defect: An atrial septal defect (ASD) is a hole in the wall that separates the heart’s two upper chambers, called the atria. Most ASDs don't close on their own. Tiny ASDs can often be left alone, but larger ASDs may require a procedure or heart surgery to close them.
• Atrioventricular septal defect: An atrioventricular septal defect is a hole in the center of the heart, in the wall that separates the upper chambers from the lower chambers. Most people with atrioventricular septal defect have abnormalities of some of their heart valves (the mitral and tricuspid valves) as well. The defect was previously also called an atrioventricular canal defect or an endocardial cushion defect.
• Bicuspid aortic valve: The aortic valve is a one-way passageway between the heart and the aorta. The valve normally has three flaps that open and close to regulate the flow of blood. A bicuspid aortic valve has only two flaps, and sometimes this causes the valve to function abnormally.
• Coarctation of the aorta: With aortic coarctation, the aorta is pinched or narrowed, limiting blood flow.
• Patent ductus arteriosus: A baby's blood does not need to go through its lungs for oxygen. Your baby gets oxygen from you instead. The ductus arteriosus is a small blood vessel that allows a fetus’ blood to skip the lungs. With patent ductus arteriosus (PDA), the hole doesn’t close when it’s supposed to. Extra blood gets pumped from the aorta to the lung (pulmonary) arteries. PDA happens more often in babies who are born early.
• Pulmonary stenosis: The pulmonary artery carries oxygen-poor blood from the right side of the heart to the lungs to be oxygenated. The pulmonary valve sits between the right side of the heart and the pulmonary artery. When the pulmonary valve becomes narrowed and doesn’t open well, it's called pulmonary stenosis.
• Ventricular septal defect: A ventricular septal defect (VSD) is like an atrial septal defect, but it’s a hole in the wall that separates the heart’s two lower chambers (the ventricles). VSD is the most common congenital heart defect. Many of these holes can close on their own, but some may require a procedure or a surgery to close them.

What are the symptoms of acyanotic heart disease?

Babies born with acyanotic heart disease may have a heart murmur, but others may not have any signs at first. Even if they don’t cause any problems at first, these defects can cause problems over time.

People with one of these defects often develop high blood pressure (hypertension), as the heart has to pump harder to do its job. They also may develop pulmonary hypertension, high blood pressure in the lungs. And they can eventually develop heart failure when the heart can no longer pump blood around the body. Those conditions may cause:

• Breathlessness.
• Dizziness.
• Fatigue (extreme tiredness).
• Syncope (fainting).

What causes acyanotic heart disease?

Scientists don’t fully understand what causes congenital heart defects. Some evidence has shown that they may be related to:

• Abnormal chromosomes.
• Genetics.
• Illnesses in the mother during pregnancy, such as diabetes, phenylketonuria (a blood disorder), drug use or viral infection.

How is acyanotic heart disease diagnosed?

• Chest X-ray: A chest X-ray takes pictures inside the chest to reveal any structural abnormalities.
• Electrocardiogram: Electrocardiogram (EKG or ECG) measures the electrical activity of the heart.
• Echocardiogram: An echocardiogram (echo) uses ultrasound to create images of the heart’s valves and chambers.
• Heart catheterization: Right heart catheterization can tell how well your heart is pumping. A thin tube is inserted into a vein and then advanced to the heart. The test can measure how much blood the heart pumps per minute and how much oxygen is in the blood in each heart chamber. The test is also called pulmonary artery catheterization.
• Left heart catheterization: This test is similar to right heart catheterization, but it’s done on the left side of the heart. Dye is injected into the bloodstream. The dye highlights how blood flows through the arteries and whether there are blockages or abnormal passages. It’s also called coronary angiography.

How is acyanotic heart disease treated?

Acyanotic heart disease sometimes corrects itself during childhood. Other times, the heart defect remains into adulthood but doesn’t require treatment. But if the defect eventually causes symptoms, you may require treatment including:

• Catheter procedure to place a plug into the defect.
• Medications to help the heart work more efficiently or to control blood pressure.
• Surgery to close the defect.

How can I prevent congenital heart disease?

Scientists aren’t sure what causes heart defects, and there are no proven strategies to prevent them.

What is the outlook for people with acyanotic heart disease?

Many people with acyanotic heart disease live long, fulfilling lives. But as you get older, a congenital heart defect may cause problems that can affect your lifestyle. Some complications, such as heart failure, can shorten your life span.

How do I take care of myself with an acyanotic heart defect?

A person with an acyanotic heart defect should have regular checkups with an adult congenital cardiologist. That’s a physician who specializes in adults who were born with heart conditions. An adult congenital cardiologist can help you:

• Determine what level of physical activity is safe.
• Eat a heart-healthy diet.
• Monitor your heart’s condition over time as you age.
• Take medications that will lessen the strain on the heart, such as drugs to lower blood pressure.
• Weigh the decision to become pregnant, which can put stress on the heart.
• Help decide when a procedure or surgery might be necessary to treat your heart condition.

A note from Cleveland Clinic

Acyanotic heart disease is a congenital heart defect that affects the normal flow of blood. Sometimes the problem corrects itself during childhood. But some heart defects remain and may eventually require treatment. If you have acyanotic heart disease, you should have regular visits with an adult congenital cardiologist to monitor the condition.