What is congenital heart disease?

According to the American Heart Association, approximately 9 of every 1,000 babies born in the U.S. have a congenital heart defect. A congenital heart defect occurs as the baby's heart is developing during pregnancy, before the baby is born. Congenital heart defects are the most common birth defects.

A baby's heart starts to develop at conception, but is completely formed 8 weeks into the pregnancy. Congenital heart defects happen during this important first 8 weeks of the baby's development. Specific steps must take place for the heart to form correctly. Often, congenital heart defects are a result of one of these steps not happening at the right time. For example, a hole is left where a dividing wall should have formed, or a single blood vessel is left where two should have been.

For more information regarding pediatric congenital heart disease services in St. Louis or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

What causes congenital heart disease?

Most 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, no specific cause can be found. Some heart problems do 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 medicines, such as antiseizure medicines or the acne medicine isotretinoin. But, most of the time, there is no clear reason for the heart defect.

Congenital heart problems range from simple to complex. Some heart problems can be watched by the baby's doctor and managed with medicines. Others will require heart surgery, sometimes as soon as in the first few hours after birth. A baby may even "grow out" of some of the simpler heart problems, such as patent ductus arteriosus or atrial septal defect. These types of heart defects may simply close up on their own with growth. Other babies will have a combination of defects and require several operations throughout their lives.

For more information about pediatric congenital heart disease services in St. Louis or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

What are the different types of congenital heart defects?

Experts classify congenital heart defects into several categories to better understand the problems the baby will experience. They include:

Again, in some cases there will be a mix of several heart defects. This creates a more complex problem that can fall into several of these categories.

Some of the problems that cause too much blood to pass through the lungs include the following:

  • Patent ductus arteriosus. This defect occurs when the normal closure of the ductus arteriosus does not occur. Extra blood goes from the aorta into the lungs and may lead to "flooding" of the lungs, rapid breathing, and poor weight gain. Patent ductus arteriosus is often seen in premature infants.

Patent ductus arteriosus anatomy

 

  • Atrial septal defect. In this heart condition, there is a hole between the two upper chambers of the heart - the right and left atria. This heart defect causes abnormal blood flow through the heart. Some children may have no symptoms and appear healthy. However, if the atrial septal defect is large, permitting a large amount of blood to pass to the right side, symptoms will be more noticeable.

Atrial Septal Defect Anatomy

 

  • Ventricular septal defect. In this heart condition, a hole in the ventricular septum (the dividing wall between the two lower chambers of the heart - the right and left ventricles) occurs. Because of this opening, blood from the left ventricle flows back into the right ventricle. This causes an extra volume of blood to be pumped into the lungs by the right ventricle, which can create congestion in the lungs.

Ventricular Septal Defect Heart Anatomy

 

Atrioventricular cCnal Defect Heart Anatomy

For more information about pediatric congenital heart disease or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

 

Some of the problems that cause too little blood to pass through the lungs include the following:

  • Tricuspid atresia. In this congenital heart condition, the tricuspid valve does not form. Therefore, no blood flows from the right atrium to the right ventricle. Tricuspid atresia is characterized by the following:

    • A small right ventricle

    • Poor blood flow to the lungs

    • A bluish color of the skin and mucous membranes caused from a lack of oxygen (cyanosis)

    A series of surgical procedures are often needed to correct this heart condition in order to increase the blood flow to the lungs and establish separate circulations.

Tricuspid Atresia Heart Condition

 

  • Pulmonary atresia. In this congenital heart defect, the pulmonary valve or pulmonary artery are underdeveloped. Normally, the pulmonary valve is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle. With pulmonary atresia, problems with valve development prevent the leaflets from opening, and blood cannot flow forward from the right ventricle to the lungs.
     

  • Transposition of the great arteries. With this congenital heart defect, the positions of the pulmonary artery and the aorta are reversed, thus:

    • The aorta originates from the right ventricle, so most of the oxygen-poor blood returning to the heart from the body is pumped back out without first going to the lungs.

    • The pulmonary artery originates from the left ventricle, so that most of the oxygen-rich blood returning from the lungs goes back to the lungs again

Transposition of the Great Arteries Heart Anatomy

 

  • Tetralogy of Fallot. This congenital heart defect is characterized by the following four defects:

    1. An abnormal opening, or ventricular septal defect, that allows blood to pass from the right ventricle to the left ventricle without going through the lungs

    2. A narrowing (stenosis) at, or just beneath, the pulmonary valve that partially blocks the flow of blood from the right side of the heart to the lungs

    3. Thickening or enlargement of the right ventricle

    4. An "overriding" aorta (the aorta lies directly over the ventricular septal defect)

    Tetralogy of Fallot can cause cyanosis - a bluish color of the skin and mucous membranes due to lack of oxygen.

Tetralogy of Fallot Heart Anatomy

 

  • Double outlet right ventricle. A complex form of congenital heart defect, in which both the aorta and the pulmonary artery are connected to the right ventricle.
     

  • Truncus arteriosus. During normal fetal development, the aorta and pulmonary artery start as a single blood vessel, and then the vessel divides into two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaveing a large connection between the aorta and the pulmonary artery.

Truncus Arteriosus Heart Anatomy

 

For more information about pediatric congenital heart disease or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

 

Some of the problems that cause too little blood to travel to the body include the following:

  • Coarctation of the aorta. In this heart condition, the aorta is narrowed or constricted. This obstructs blood flow to the lower part of the body and increases blood pressure above the constriction. Usually there are no symptoms at birth, but they can develop as early as the first week of life. If severe symptoms of high blood pressure and congestive heart failure develop, surgery may be considered.

Coarctation of the Aorta Heart Anatomy

 

  • Aortic stenosis. In aortic stenosis, the aortic valve between the left ventricle and the aorta did not form properly and is narrowed. This makes it difficult for the heart to pump blood to the body. A normal valve has three leaflets or cusps, but a stenotic valve may have only one cusp (unicuspid) or two cusps (bicuspid). Although aortic stenosis may not cause symptoms, it may worsen over time. A cardiac catheterization procedure may be needed to correct the blockage, or surgery may be needed to replace the valve with an artificial one.

Aortic Stenosis Heart Anatomy

 

  • Hypoplastic left heart syndrome. This heart condition consists of a combination of several abnormalities of the heart and the great blood vessels. In hypoplastic left heart syndrome, most of the structures on the left side of the heart, including the left ventricle, mitral valve, aorta, and aortic valve, are underdeveloped. The degree of underdevelopment differs from child to child. The left ventricle may not be able to pump enough blood to the body. Hypoplastic left heart syndrome is fatal without treatment.

Hypoplastic Left Heart Syndrome Heart Anatomy

 

For more information regarding any of the type of congenital heart disease or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

Who treats congenital heart defects?

Babies with congenital heart problems are treated by specialists called pediatric cardiologists. These doctors diagnose heart defects and help manage the health of children before and after surgical repair of the heart problem. Specialists who correct heart problems in the operating room are known as pediatric cardiovascular surgeons or pediatric cardiothoracic surgeons.

At the St. Louis Children’s and Washington University Heart Center, our goal is to treat your children and return them as quickly as possible to you - where they belong.

Our pediatric cardiologists care for more children with heart disease than any other heart center in the region. In many cases, we are creating the new standards of care for children with complex heart conditions.

The cardiology team at St. Louis Children's Hospital includes some of the world’s foremost experts in child heart care. Our collective experience includes more than 150 specialists and experts focused on managing children with heart problems. That’s the reason why we are a destination center for referring physicians.

Our team includes 20 cardiologists, 3 cardiothoracic surgeons, 7 cardiac anesthesiologists, 23 nurse practitioners/physician assistants and 102 nurses. We also have the only dedicated cardiac intensive care in the region.

For more information regarding pediatric congenital heart disease services in St. Louis or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.

Treatment of Congenital Heart Disease in Adults

A new subspecialty within cardiology is emerging, as the number of adults with congenital heart disease is now greater than the number of babies born with congenital heart disease. This improved survival is a result of advances in diagnostic procedures and treatment interventions.

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

For adults with congenital heart disease, 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 their young adult children. This will help ensure the transition to adult specialty care and will optimize the health status of the young adult with congenital heart disease.

For more information regarding pediatric congenital heart disease services in St. Louis or to make an appointment with a pediatric cardiologist, please call 314.454.5437 or 800.678.5437 or email us.