Hypoplastic left heart syndrome

Diagnosis

To diagnose hypoplastic left heart syndrome (HLHS), a healthcare professional examines the baby and listens to the baby's heart. The healthcare professional may hear a sound called a heart murmur. Rushing blood flow causes this sound.

Tests

Tests used to find hypoplastic left heart syndrome (HLHS) in the baby before or after birth may include:

• Pregnancy ultrasound. A routine ultrasound exam during the second trimester of pregnancy usually can tell if the baby has HLHS.
• Echocardiogram. This test uses sound waves to make pictures of the heart. It shows how blood flows through the heart. It can be used after a baby is born to diagnose hypoplastic left heart syndrome. If a baby has HLHS, the test might find that the lower left heart chamber and heart valves are small. The body's main artery, called the aorta, also may be small.

Treatment

A baby born with hypoplastic left heart syndrome (HLHS) needs urgent treatment. Treatment can include many surgeries or a heart transplant. Medicines and other therapies are used to manage symptoms before heart surgery.

Talk with your child's healthcare professional about treatment options for your child.

If hypoplastic left heart syndrome is found before birth, healthcare professionals usually recommend giving birth at a hospital with a cardiac surgery center.

Medications

The medicine alprostadil (Prostin VR Pediatric) is used to keep the ductus arteriosus open. It typically closes in all babies soon after birth. But in babies with hypoplastic left heart syndrome, the ductus needs to stay open so that blood can go to the rest of the body.

Therapies

While waiting for surgery or a heart transplant, a baby with hypoplastic left heart syndrome may be given medicine and have these treatments:

• Breathing help. Babies who have trouble breathing may need help from a breathing machine called a ventilator.
• Fluids through a vein. A baby might receive fluids through a tube inserted into a vein. These are called intravenous (IV) fluids.
• Feeding tube. Babies who have trouble feeding or who tire while feeding can be fed through a feeding tube.

Surgery or other procedures

Most children with hypoplastic left heart syndrome need several surgeries.

• Atrial septostomy. This treatment uses tubes called catheters and a balloon to make or widen an opening between the heart's upper chambers. It lets more blood flow between the upper two chambers of the heart. This treatment is done if the foramen ovale closes or is too small. Babies born with a hole in the heart, called an atrial septal defect, might not need atrial septostomy.

Other surgeries can make separate pathways to get the correct blood flow to the body and lungs. The surgeries are done in three stages.

• Norwood procedure. This surgery is often done within the first two weeks of life. There are various ways to do this treatment.

  Surgeons rebuild the aorta and connect it to the heart's lower right chamber. Then they add a tube called a shunt to provide the lungs with blood. The surgeons may use one of two types of tubes. One type of tube connects the body's main artery to the arteries leading to the lungs. Those are the pulmonary arteries. The other type of tube goes from the right lower heart chamber to the pulmonary arteries. This treatment lets the right lower heart chamber pump blood to both the lungs and the body.

  Sometimes, a mixed, also called hybrid, procedure is done. Heart surgeons place a stent in the ductus arteriosus to maintain the opening between the pulmonary artery and the aorta. Then they place bands around the pulmonary arteries to reduce blood flow to the lungs. They also make an opening between the upper chambers of the heart.

  After the Norwood procedure, a baby's skin often is still slightly still slightly blue or gray. This is because oxygen-rich and oxygen-poor blood continue to mix within the heart. The blue or gray color may be easier or harder to see in some babies. Once a baby has this treatment, the odds of survival can go up.

• Bidirectional Glenn procedure. This tends to be the second surgery. It's usually done when a child is between 4 and 6 months of age. It involves removing the first shunt and connecting the large vein that drains blood from the head and arms to the pulmonary artery. Now the lungs receive blood from the vein instead of the shunt. The large vein is called the superior vena cava.

  This surgery lessens the work of the right lower heart chamber by letting it pump blood mainly to the aorta. It also lets most of the oxygen-poor blood returning from the body flow directly into the lungs. After this treatment, all the blood returning from the upper body flows to the lungs. So blood with more oxygen is pumped to the aorta to supply organs and tissues throughout the body.

• Fontan procedure. This surgery usually is done when a child is between 3 and 4 years of age. The surgeon creates a path for the blood from the lower legs to flow directly into the pulmonary arteries. The vessel involved is called the inferior vena cava. The pulmonary arteries then send the blood into the lungs.

  The Fontan procedure lets the rest of the oxygen-poor blood returning from the body flow to the lungs. After this surgery, there's little mixing of oxygen-rich and oxygen-poor blood in the heart. So the skin should no longer look blue or gray.

• Heart transplant. Many babies with hypoplastic left heart syndrome need a heart transplant. (8) Children who have heart transplants need to take medicines for life to so that their bodies don't reject the donor heart.

Follow-up care

After surgery or a transplant, a baby needs lifelong care with a heart doctor trained in congenital heart disease to watch for complications. Your child may need further treatment or other medicines for these complications.

Some children may need to take antibiotics before certain dental or other procedures to help prevent infections. Ask your child's healthcare professional if your child needs preventive antibiotics. Some children also may need to limit physical activity.

Follow-up care for adults

Adults who were born with hypoplastic left heart syndrome (HLHS) need to see a heart doctor trained in congenital heart disease in adults. Recent advances in surgical care have helped children with HLHS grow into adulthood. So it's not yet clear what challenges an adult with the heart condition might have. Adults need regular, lifelong follow-up care to watch for changes in the condition.

People thinking about becoming pregnant should talk with their healthcare professionals about pregnancy risks and birth control options. Having hypoplastic left heart syndrome raises the risk of:

• Heart and blood vessel problems during pregnancy.
• Miscarriage.
• A baby being born with a congenital heart defect.

Coping and support

It can be challenging to live with hypoplastic left heart syndrome (HLHS) or to care for a baby with the condition. These tips may help:

• Seek support. Ask family members and friends for help. Caregivers need breaks. Talk with your child's heart doctor, also called cardiologist, about support groups and other types of help. And think about meeting with a counselor or therapist if you often feel anxious, sad or too stressed.

  If you're a teen or an adult with HLHS, ask your healthcare team if there are support groups for people your age with congenital heart disease. It can be helpful to talk to other people who share your challenges.

• Keep health records. Write down your or your baby's diagnosis, medicines, surgery and other treatments. Include the dates of treatment and the name and phone number of the doctor or surgeon. Also note emergency contact numbers for healthcare professionals and hospitals. Include a copy of surgery reports in your records.

  This information helps you keep track of the care received. It is helpful for any new healthcare professionals who don't know your or your child's health history. This information also is helpful when your child moves from pediatric care to adult cardiology care.

• Talk about your concerns. Talk with your child's healthcare team about which activities are safest for your child. If some are off-limits, encourage your child in other hobbies. If you are an adult with hypoplastic left heart syndrome, ask your healthcare professional about activities you can do