Congenital Heart Defects

What Is a Heart Defect?

A heart defect is a problem in the heart's structure. Kids who have a heart defect were born with it. Heart defects are often called "congenital," which means "present at birth." Heart defects are also sometimes referred to as "congenital heart disease."

Heart defects can range from mild to severe.

What Are the Types of Heart Defects?

There are many different types of congenital heart defects. Some are commonly called a “hole in the heart” because they involve an abnormal connection between the heart's chambers. But other types of heart defects involve:

• abnormal heart valves
• blood flow that's blocked from entering or leaving the heart
• abnormal connections of the blood vessels to the heart
• parts of the heart that are too small to work properly

Types of congenital heart defects include:

• aortic stenosis
• atrial septal defect (ASD)
• atrioventricular canal defect
• bicuspid aortic valve
• coarctation of the aorta (COA)
• Ebstein anomaly
• hypoplastic left heart syndrome
• patent ductus arteriosus (PDA)
• patent foramen ovale (PFO)
• pulmonary atresia
• pulmonary stenosis
• tetralogy of Fallot (TOF)
• total anomalous pulmonary venous return
• transposition of the great arteries
• tricuspid atresia
• truncus arteriosus
• ventricular septal defect (VSD)

Sometimes, a child can be born with more than one congenital heart defect.

How Are Heart Defects Diagnosed?

Doctors can diagnose a congenital heart defect in several ways. Sometimes, they make a diagnosis before a child is born using a fetal echocardiogram (an ultrasound done before birth).

Other times, especially for more serious conditions, a baby may show a bluish color of the skin called “cyanosis,” which is a sign of a low oxygen level in the blood. Doctors can use pulse oximeter to check a baby for this. All newborns are screened for critical congenital heart disease before they go home. This can help doctors find some (but not all) forms of congenital heart disease.

Some babies or children might have a heart murmur. But most heart murmurs are harmless (called innocent murmurs).

Signs of a possible congenital heart problem include fast breathing, slower growth, and in older kids, shortness of breath or heart palpitations (feeling their heart beating fast or differently).

How Are Heart Defects Treated?

Children with minor heart defects may not need any treatment. But some babies have serious symptoms that need medical or surgical treatment within the first year of life. They'll be cared for by:

• pediatric cardiologists: doctors who specialize in treating children's heart problems
  or
• pediatric heart surgeons: doctors who specialize in children's heart surgery

Procedures done through cardiac catheterization — such as balloon angioplasty or valvuloplasty — can widen an obstructed blood vessel or valve. Another procedure, transcatheter device occlusion, can close abnormal openings or holes within the heart or blood vessels without surgery.

Some problems, such as small- or moderate-sized ventricular septal defects, may close or get smaller as a child grows. While waiting for the hole to close, the child might have to take medicines.

Complex defects found early might need a series of operations that are finished when a child is about 3 years old.

What Happens After Treatment?

Kids treated for a defect (surgically or medically) will need regular visits with a pediatric cardiologist. At first, these visits might happen often — perhaps every month or two. Later, they might be cut back, sometimes to just once a year.

The cardiologist may use tools like X-rays, electrocardiograms (ECGs), or echocardiograms to watch the defect and the effects of treatment.

Some physical activities might be limited, but kids can still play and explore with friends. Always check with the cardiologist about which activities are OK for your child and which to avoid. Some competitive sports could be off limits, for example.

Preventing Infection

Infective (or bacterial) endocarditis is an infection of the tissue that lines the heart and blood vessels. Kids with heart defects used to get before procedures that could let get into the bloodstream, such as:

• dental work
• surgery in body areas where bacteria tend to grow, such as the mouth or gastrointestinal tract

But now, preventive antibiotics are given only to some children with heart defects. This includes those who:

• have a type of congenital heart disease that causes cyanosis (bluish color of the skin)
• have had infective endocarditis before
• had their defect repaired with prosthetic material (like an artificial heart valve) or device

The cardiologist will know the latest guidelines, and can advise you based on your child's diagnosis.

Kids with heart defects should take good care of their teeth. They should brush and floss daily, and have regular dental visits and cleanings as often as the dentist recommends.

Looking Ahead

Most heart defects are now treated during infancy. So when your child is old enough to understand, explain what happened. Talk about why your child:

• has a surgical scar
• needs to take medicine
• has to visit the pediatric cardiologist

Describe the treatment in a way your child can understand.

It can be tempting to be very protective. But help your child lead as normal a life as possible. Talk with your cardiologist or the care team about safe ways to do this. They are there to support your child and the whole family.

It also can help to look for local and online support groups. This can connect you to other families who can share what works for them.

What Else Should I Know?

As kids get older, it's important to help them learn how to take charge of their medical care. A younger teen could fill a prescription or schedule an appointment. Older teens should know their heart diagnosis, and what surgery or procedures they have had. As they get older, they should understand health insurance coverage and know how to access their medical records.

Help an older teen move from a pediatric cardiologist to one who cares for adults. The cardiologist should play an active role in choosing the new doctor. Encourage your child to make appointments, ask questions and take notes, and set aside time to speak with the doctor alone.

To prepare for adulthood and manage their health care, teens should know:

• about their heart condition
• when to get care
• the names of all medicines, their dosages and when to take them, common side effects, and interactions with other medicines
• if they have allergies to food or medicine
• the answers to most questions about their health and medical history
• how to:
  • schedule appointments
  • order prescription refills
  • contact the care team
  • manage medical tasks outside of home
• what problems can happen if they don't follow the treatment plan
• about their insurance coverage
• to always carry their insurance information with them