Congenital Heart Defects

image of pregnant person, newborn baby sleeping, and young child having heart checked

This webpage provides important information about congenital heart defects in children and adults. It provides recommendations for people who want to get pregnant and resources for children and adults living with congenital heart defects.

About Congenital Heart Defects

Congenital heart defects are the most common birth defects, affecting about 1 in every 110 births, or 40,000 babies born in the United States each year. About 1 in 4 babies born with a heart defect has a critical congenital heart defect. Critical congenital heart defects are more serious and require surgical or medical intervention within the first year of life.

More than half of people living with congenital heart defects are adults, and that number continues to rise each year due to improved diagnostics and treatment options.

What Causes Congenital Heart Defects?

Congenital heart defects occur when the fetus’ heart develops abnormally during pregnancy, affecting its ability to circulate blood. Common examples include holes in the heart and narrow or leaky valves.

Most of the time, the cause of congenital heart defects is unknown. Different factors can work together to increase risk, including genetic factors, maternal conditions such as diabetes or obesity, diet, cigarette smoking, certain infections, and contact with some environmental chemicals. Researchers are working to better understand what causes congenital heart defects.

There are different types of congenital heart defects, and the severity can vary. To search for more information visit the birth defects glossary.

  • Atrial Septal Defect. Causes opening(s) in the wall between the right and left atria, allowing a mixing of oxygen-rich and oxygen-poor blood.
  • Atrioventricular Septal Defect. Affects the valves and walls between the heart's upper and lower chambers so that blood does not flow the way it should.
  • Coarctation of the Aorta*. Characterized by localized narrowing of the aorta, which causes abnormal cardiac circulation and pressure when the heart pumps.
  • Double-outlet Right Ventricle*. Affects the connection of the pulmonary artery and the aorta where they are connected to the right ventricle. This causes oxygen-poor blood to be circulated to the body. It is usually accompanied by a ventricular septal defect (VSD).
  • d-Transposition of the Great Arteries*. The pulmonary artery and the aorta are switched in position so that blood flows incorrectly between the lungs, heart, and the rest of the body.
  • Ebstein Anomaly*. The tricuspid valve forms lower than it should between the right heart chambers and does not close properly causing abnormal blood flow.
  • Hypoplastic Left Heart Syndrome*. The left side of the heart, including the aorta, aortic valve, left ventricle, and mitral valve, is underdeveloped so the heart cannot properly pump oxygen-rich blood to the body.
  • Interrupted Aortic Arch*. The aorta forms incorrectly so there is a missing piece between the ascending and descending aorta. This means the aorta cannot pump oxygen-rich blood to the body the way it should. It usually occurs with a ventricular septal defect (VSD).
  • Pulmonary Atresia*. The pulmonary valve does not form so blood cannot flow from the right ventricle to the pulmonary artery.
  • Single Ventricle*. When only one ventricular chamber forms in the heart instead of two, allowing oxygen-rich and oxygen-poor blood to mix.
  • Tetralogy of Fallot*. A combination of four heart defects that affects how blood flows to the heart and lungs. The four defects are ventricular septal defect, pulmonary valve stenosis or atresia, displacement of the aorta to the right, and hypertrophy of right ventricle.
  • Total Anomalous Pulmonary Venous Return*. The vessels that bring oxygen-rich blood back to the heart from the lungs are improperly connected.
  • Tricuspid Atresia*. The absence of the tricuspid valve. The opening between the right atrium and right ventricle is closed, and normal circulation is not possible.
  • Truncus Arteriosus*. When a single common blood vessel comes out of the heart, instead of the usual two vessels.
  • Ventricular Septal Defect. Opening(s) in the wall between the right and left lower chambers of the heart, allowing oxygen-rich and oxygen-poor blood to mix.

    • * Defects with an asterisk are considered critical congenital heart defects.

Detecting Heart Defects

Most congenital heart defects can be diagnosed during routine prenatal care. Every baby born in New York State is also universally screened using a pulse oximetry test within 24 hours of birth. This is a painless, noninvasive test that measures the amount of oxygen in the blood and can detect potential problems with the heart. Early diagnosis has been shown to reduce infant mortality by facilitating earlier intervention.

Survival

Most babies born with a congenital heart defect will survive to at least one year of age. For babies who survive beyond a year, about 93% will survive to 35 years of age and older.

Living with Congenital Heart Defects

Congenital heart defects can increase your risk of:

  • Developmental delays or learning difficulties
  • Problems with physical activity, growth, and appetite
  • Heart rhythm problems
  • Congestive heart failure
  • Infective endocarditis
  • Kidney and liver disease
  • Sudden cardiac arrest or stroke

Treatment for Congenital Heart Defects

Individuals with congenital heart defects should work with their cardiology care providers to develop treatment and management plans specific to their needs. Treatment and management options vary, and may include the following:

  • Cardiology visits
  • Medications
  • Corrective surgical procedures
  • Cardiac catheterizations
  • Heart transplants

Children and adults with congenital heart defects are more likely to be hospitalized compared to others. To reduce the risk of health issues throughout life, it is important to have consistent medical care with a cardiology specialist.

If your child is 18 year old, work with them to successfully transition from pediatric to adult cardiology care. It is important for adults with congenital heart defects to continue to receive specialized cardiology care throughout their lives.

Preventing Heart Defects in Babies

People with congenital heart defects should develop a plan with their health care provider before getting pregnant to reduce pregnancy risks as well as risks of having a baby with a congenital heart defect. A baby’s risk of a congenital heart defect is higher if their mother or father has one. The risk is even higher if the baby’s sibling has one.

If you are planning on becoming pregnant, or are pregnant, it is important to identify your prenatal care provider as soon as possible. Talk to them about family medical history, diseases, and medications that may affect the risk of congenital heart defects. Some specific topics to discuss with your provider include:

  • Safe medications in pregnancy
  • Recommended vaccinations before and during pregnancy
  • Healthy foods and exercise before and during pregnancy
  • Avoiding alcohol, recreational drugs, and tobacco
  • Taking a folic acid vitamin
  • Reviewing genetic counseling and testing options

Additional Resources