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Home Congenital Heart Defects
FlowerCongenital Heart Defects
Programs that treat this condition
Fetal Cardiology Program   Adult Congenital Heart Service  
Interventional Catheterization Program   Heart Transplant Program  
Cardiovascular Genetics Program   Cardiac Psychiatry Program  
Advanced Fetal Care Center   Cardiology Outpatient Services  
Genetics Program  
What is a congenital heart defect (congenital heart disease)?
Image One out of every 100 infants born in the United States has a congenital (present at birth) heart defect -- a problem that occurred as the baby's heart was developing during pregnancy, before the baby is born. Congenital heart defects are the most commonly occurring birth defect.

A baby's heart begins to develop at conception, and is completely formed by eight weeks into the pregnancy. Congenital heart defects happen during these crucial first eight weeks of the baby's development. Specific steps must take place in order for the heart to form correctly. Often, congenital heart defects are a result of one of these crucial steps not happening at the right time, leaving a hole where a dividing wall should have formed, or a single blood vessel where two ought to be.

What causes congenital heart defects?
The vast majority of congenital heart defects have no known cause. Mothers will often wonder if something they did during the pregnancy caused the heart problem. In most cases, nothing can be linked to the heart defect. Some heart problems occur more often in families, so there may be a genetic link to some heart defects. Some heart problems are likely to occur if the mother had a disease while pregnant and was taking medications, such as anti-seizure medicines. In 85 to 90 percent of the cases, however, there is no identifiable reason as to why the heart defect occurred. For more information, see congenital heart defects -- causes.
What are the different types of congenital heart defects?
Image Congenital heart problems range from simple to complex. Some heart problems can be watched by the child's physician and managed with medications, while others will require surgery, sometimes as soon as in the first few hours after birth. A child may even "grow out" of some of the simpler heart problems, as these defects may resolve on their own as the child grows. Other infants will have a combination of malformations and require several operations during their lifetime.

To better understand congenital heart defects, it may help to review About the Heart and Blood Vessels for more information on how the heart normally works.

Congenital heart defects can be classified into several categories according to the problems your child may experience. They include the following:
  • Problems that cause too much blood to pass through the lungs -- These defects allow oxygen-rich (red) blood that should be traveling to the body to re-circulate through the lungs, causing increased pressure and stress in the lungs. Examples include the following:
    • patent ductus arteriosus (PDA)
    • atrial septal defect (ASD)
    • ventricular septal defect (VSD)
    • atrioventricular canal (AV canal or AVC)
  • Problems that cause too little blood to pass through the lungs — These defects allow blood that has not been to the lungs to pick up oxygen (and, therefore, is oxygen-poor) to travel to the body. The body does not receive enough oxygen with these heart problems, and the baby will be cyanotic, or "blue." Examples include the following:
    • tricuspid atresia (TA)
    • pulmonary atresia (PA)
    • transposition of the great arteries (TGA)
  • Problems that cause too little blood to travel to the body -- These defects are a result of underdeveloped chambers of the heart or blockages in blood vessels that prevent the proper amount of blood from traveling to the body to meet its needs. Examples include:
    • coarctation of the aorta
    • aortic stenosis
    • pulmonary stenosis
  • A combination of several heart defects -- These combinations create a more complex problem that can fall into several of these categories. Complex combination of heart defects include:
    • hypoplastic left heart syndrome (HLHS)
    • truncus arteriosis
    • total anomalous pulmonary venous return (TAPVR)
Who treats congenital heart defects?
Babies with congenital heart problems are treated by specialists called pediatric cardiologists. These physicians diagnose heart defects and help manage the health of children before and after surgical repair of the heart problem. Specialists who surgically correct heart problems in the operating room are known as pediatric cardiovascular surgeons or pediatric cardiothoracic surgeons.

A new subspecialty within cardiology is emerging as the number of adults with congenital heart disease (CHD) is now greater than the number of babies born with CHD, as a result of the advances in diagnostic procedures and treatment interventions that have been made since 1945.

In order to achieve and maintain the highest possible level of wellness, it is imperative that those individuals born with CHD who have reached adulthood transition to the appropriate type of cardiac care. The type of care required is based on the type of CHD a person has. Those persons with simple CHD can generally be cared for by a community adult cardiologist. Those with more complex types of CHD will need to be cared for at a center that specializes in adult CHD.

For adults with CHD, guidance is necessary for planning key life issues such as college, career, employment, insurance, activity, lifestyle, inheritance, family planning, pregnancy, chronic care, disability, and end of life. Knowledge about specific congenital heart conditions and expectations for long-term outcomes and potential complications, and risks must be reviewed as part of the successful transition from pediatric care to adult care. Parents should help pass on the responsibility for this knowledge and accountability for ongoing care to ensure the transition to adult specialty care and optimize the health status of the young adult with CHD.

Always consult with your child's doctor regarding your child's diagnosis and treatment.
Contact Children's Hospital Boston Cardiovascular Program physicians for a second opinion.
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