Congenital heart defect repair

What is congenital heart disease?

Congenital heart disease (CHD) is an issue with your heart’s structure that’s present at birth. These issues — which keep blood from flowing normally — may include:

• A hole in your heart wall.
• Issues with your blood vessels (too many or too few, blood flowing too slowly, to the wrong place or in the wrong direction).
• Problems with your heart valves that control blood flow.

Some cases of CHD are simple and may not cause any symptoms. But others can be life-threatening and require treatment in infancy.

Healthcare providers can detect heart defects early (before or shortly after birth). But sometimes, people don’t get a CHD diagnosis until childhood, adolescence or adulthood.

What are the types of congenital heart disease?

There are two main groups of CHD:

• Cyanotic (low oxygen in the blood) congenital heart disease.
• Acyanotic (blood oxygen level is acceptable) congenital heart disease.

Cyanotic congenital heart disease

These heart anomalies reduce the amount of oxygen your heart can deliver to the rest of your body. Babies born with cyanotic congenital heart disease usually have low levels of oxygen and need surgery. Examples include:

• Left heart obstructive lesions: These reduce blood flow between the heart and the rest of your body (systemic blood flow). Examples include hypoplastic left heart syndrome (when your heart is too small on the left side) and interrupted aortic arch (aorta is incomplete).
• Right heart obstructive lesions: These reduce blood flow between your heart and lungs (pulmonary blood flow). Examples include tetralogy of Fallot (a group of four anomalies), Ebstein’s anomaly, pulmonary atresia and tricuspid atresia (valves don’t develop correctly).
• Mixing lesions: The body mixes systemic and pulmonary blood flow. One example is transposition of the great arteries, which means the two main arteries leaving your heart are in the wrong place. Another is truncus arteriosus, when your heart has only one main artery, instead of two, to carry blood to your body.

Acyanotic congenital heart disease

This involves an issue that makes blood pump through your body in an abnormal way. For example:

• Hole in the heart: One of your heart’s walls can have an abnormal opening. Depending on the location of the hole, this may be called atrial septal defect, atrioventricular canal, patent ductus arteriosus or ventricular septal defect.
• Problem with the aorta: The aorta is the main artery that carries blood away from your heart to the rest of your body. It can be too narrow (aortic coarctation). Or the aortic valve (which opens and closes to regulate blood flow) may be restricted in opening or have only two flaps instead of three (called bicuspid aortic valve).
• Problem with the pulmonary artery: The pulmonary artery carries blood from the right side of your heart to your lungs to get oxygen. If this artery is too narrow, it’s called pulmonary artery stenosis.

How common is congenital heart disease?

Congenital heart disease is the most common type of congenital condition. CHDs affect 1% of U.S. births.

Symptoms and Causes

What are the symptoms?

Congenital heart disease symptoms may start as soon as a baby is born or may not appear until later in life. They can include:

• Cyanosis (bluish skin, lips or nails).
• Excessive sleepiness.
• Fast breathing or trouble breathing.
• Fatigue (extreme tiredness).
• Getting unusually tired or out of breath during exercise.
• Heart murmur (a swishing sound your heart makes that may indicate abnormal blood flow).
• Poor blood circulation.
• Weak pulse or pounding heartbeat.

The signs and symptoms of congenital heart defects vary widely, depending on:

• Age.
• The number of heart issues (a person can be born with more than one).
• The severity of the condition.
• The type of congenital condition.

What causes congenital heart disease?

CHD happens when the fetal heart doesn’t develop correctly in the uterus. Scientists don’t fully understand why that happens, but it may be related to:

• Having abnormal chromosomes or genetics that come from parents or happen randomly.
• Drinking alcohol, smoking or breathing in secondhand smoke during pregnancy.
• Taking medications during pregnancy like certain drugs for high blood pressure, high cholesterol or acne.
• Having an illness during pregnancy (diabetes, phenylketonuria or a viral infection like rubella).

Researchers consider these to be the risk factors for congenital heart disease.

What are the complications of congenital heart disease?

Congenital heart defects can make you more likely to have:

• Abnormal heart rhythms (arrhythmias).
• Heart failure.
• Kidney disease.
• Blood clots.
• Pulmonary hypertension.
• Endocarditis.
• Liver disease.
• Speech issues.
• Attention-deficit/hyperactivity disorder (ADHD).

Diagnosis and Tests

How is congenital heart disease diagnosed?

Sometimes a healthcare provider finds a congenital condition before a baby is born. If your provider finds anything unusual during a routine prenatal ultrasound, you and the fetus may need further testing. For example, a fetal echocardiogram uses harmless sound waves to create pictures of the fetal heart.

Providers detect other heart issues soon after a baby is born. For example, they can diagnose cyanotic CHD with pulse oximetry. The simple, painless test uses sensors on your baby’s fingers or toes to find out if oxygen levels are too low. Sometimes, people don’t get a congenital heart defect diagnosis until later in life.

What tests will be done to diagnose congenital heart disease?

Tests that can help diagnose CHD in newborns, children or adults include:

• Physical exam: During an exam, a healthcare provider will listen to your heart for any abnormal sounds.
• Chest X-ray: A chest X-ray takes pictures of the inside of your chest to reveal any structural abnormalities.
• Electrocardiogram: Electrocardiogram (EKG or ECG) measures the electrical activity of your heart.
• Echocardiogram: An echocardiogram (echo) uses ultrasound to create images of your heart’s valves and chambers.
• Heart catheterization: A healthcare provider can tell how well your heart is pumping and circulating blood by performing a heart catheterization. Providers also call it cardiac catheterization or coronary angiography.
• Magnetic resonance imaging (MRI): MRI for heart disease can create detailed pictures of your heart.