Description

Hypoplastic left heart syndrome (HLHS) is a rare heart condition that a child is born. That means that it is a congenital defect of the heart. In this condition, the left side of the heart are not fully developed and it is too small. So it can't pump blood well. In contrast, the right side of the heart pumps blood to the lungs and to the rest of the body.

Treatment for hypoplastic left heart syndrome may include medication, surgery on the heart or a heart transplant. Advances in care have improved the outlook for babies born with HLHS.

Symptoms

Babies born with hypoplastic left heart syndrome (HLHS) are usually very ill shortly after birth. The symptoms of HLHS include:

  • The blue or the gray skin, lips or nails. Depending on the color of the skin, these changes may be more difficult or more easy to see.
  • Fast, trouble breathing.
  • A bad diet.
  • Cold hands and feet.
  • Weak pulse.
  • It is more drowsy, or less active than is typical for most babies.

Without treatment, a baby with this condition may go into shock. Symptoms of shock include:

  • Cold, cold and clammy skin that may be pale or lips that can be blue or gray.
  • A weak and rapid pulse.
  • Breathing may be slow and shallow, or very fast.
  • Dull eyes that seem to stare.

When to see a doctor

Get emergency medical help if your baby has:

  • Changes in the skin or the color of the nails.
  • Difficulty breathing or rapid breathing.
  • Pulse weak or rapid pulse.
  • Chill the wet skin.

Causes

Hypoplastic left heart syndrome (HLHS) occurs in the womb when a baby's heart develops. The cause is not known. The changes in genes can play a role.

In the hypoplastic left heart syndrome, the left side of the heart has not grown enough so that it does not develop fully. Cannot correctly send the blood to the body. In HLHS, the following areas of the heart are too small:

  • The lower left chamber of the heart called the left ventricle.
  • The body's main artery, called the aorta.
  • The valves of the heart on the left side of the heart, the flame of the mitral and aortic valves.

After birth, the right side of the heart of a baby is usually pumps the blood to the lungs and to the rest of the body. The blood passes through an opening, called the ductus arteriosus. This opening, also called a vessel connecting the pulmonary artery directly to the aorta. The oxygenated blood returns to the right side of the heart through a natural opening between the upper chambers of the heart. The opening is called the foramen ovale.

The ductus arteriosus usually closes after the first day or two of life. When that happens, the right side of the heart can't pump blood to the body. The left side of the heart carries out this work.

But in babies with hypoplastic left heart syndrome, the left can't pump blood well. So that the need for medicine to keep these connections open and maintain the flow of blood to the body until they have heart surgery.

Risk factors

People who have a child with hypoplastic left heart syndrome (HLHS) are at a higher risk of having another baby with this or a similar condition.

There are No other risk factors for the hypoplastic left heart syndrome.

Complications

With proper treatment, many infants with hypoplastic left heart syndrome (HLHS) to survive. But they have many surgeries and may have less energy, and other challenges. Complications of HLHS may include:

  • To tire more easily during the practice of sports or other exercises.
  • Irregular heartbeats, called arrhythmias.
  • The buildup of fluid called edema in the lungs, in the area of the stomach, legs, and feet.
  • It does not grow well.
  • The development conditions related to the brain and the nervous system.
  • Need more of a heart surgery or a heart transplant.

Prevention

There is no way to prevent the hypoplastic left heart syndrome. If you were born with a heart condition, talk to a heart doctor and genetic counselor before becoming pregnant.

Diagnosis

To diagnose the hypoplastic left heart syndrome (HLHS), a health care professional examines the baby and hear the baby's heart. The health care provider may hear a sound called a heart murmur. Running blood flow to the cause of this sound.

Tests

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

  • Pregnancy ultrasound. A routine ultrasound examination during the second trimester of pregnancy is usually possible to tell if the baby has HLHS.
  • The echocardiogram. This test uses sound waves to create images of the heart. Shows how blood flows through the heart. Can be used after the birth of the baby to diagnose the hypoplastic left heart syndrome. If a baby has HLHS, the proof can be found that the lower left chamber of the heart and the heart valves are small. The body's main artery, called the aorta, can also be small.

Treatment

A baby born with hypoplastic left heart syndrome (HLHS) needs an urgent treatment. The treatment can include many surgeries and a heart transplant. Medications and other therapies that are used to control the symptoms before heart surgery.

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

If the hypoplastic left heart syndrome can be found before birth, the health care professionals often recommend to give birth in a hospital with a cardiac surgery center.

Drugs

Alprostadil (Prostin VR Pediatric) is used to keep the ductus arteriosus open. Normally closes in all infants soon after birth. But in babies with hypoplastic left heart syndrome, the conduit must be open so that it can pass the blood to the rest of the body.

Therapies

While waiting for surgery or a heart transplant, a baby with hypoplastic left heart syndrome, may self-administer the medication, and of these treatments:

  • Help with breathing. Babies who have breathing problems may need the help of a breathing machine called a ventilator.
  • Fluids through a vein. A baby you may receive fluids through a tube inserted in a vein. These are the so-called intravenous (IV) fluids.
  • Feeding tube. Infants who have feeding problems or that tire, while power can be fed through a feeding tube.

Surgery or other procedures

The majority of children with hypoplastic left heart syndrome require multiple surgeries.

  • Septostomy headset. This treatment uses tubes called catheters and a balloon to make or enlarge an opening between the upper chambers of the heart. This allows more blood to flow between the two upper chambers of the heart. This treatment is done if the foramen ovale is closed or it is too small. Babies who are born with a hole in the heart, called an atrial septal defect, you may not need septostomy headset.

Other surgeries can be done separately from the ways of achieving the proper flow of the blood to the body and lungs. The surgeries were carried out in three stages.

  • Norwood procedure.This surgery is often done in the first two weeks of life. There are several ways to do this treatment. The surgeons of the reconstruction of the aorta and connect with the heart in the bottom right of the camera. Then added a tube called a shunt to provide blood to the lungs. Surgeons can use one of two types of tubes. A type of tube that connects the main artery of the body the arteries that carry blood to the lungs. These are the pulmonary arteries. The other type of tube that goes from the right lower chamber of the heart to the pulmonary arteries. This treatment allows the right lower chamber of the heart to pump blood to both the lungs and the body. Sometimes, a joint, also called hybrid, the procedure is performed. The cardiovascular surgeons to place a stent in the ductus arteriosus to maintain the opening between the pulmonary artery and the aorta. Then place the bands around the pulmonary arteries to reduce the flow of blood to the lungs. They also make an opening between the upper chambers of the heart. After the Norwood procedure, the skin of a baby is often still a little still slightly blue or gray. This is due to the oxygen-rich and oxygen-poor blood to continue to mix within the heart. The blue or the grey color can be easier or harder to see in some babies. When a baby has this treatment, the survival rate can go up.
  • The bi-directional Glenn procedure.This tends to be the second surgery. This is usually done when the child is between 4 and 6 months of age. It is the removal of the first shunt and connection of the large vein that drains blood from the head and arms to the pulmonary artery. Now, the lungs receive blood from the vein in place of the shunt. The large vein called the superior vena cava. This surgery reduces the work of the right lower chamber of the heart let the blood pump mainly to the aorta. Also allows the most oxygen-poor blood returning from the body to flow directly to the lungs. After this treatment, all the blood that returns to the upper part of the body flows into the lungs. So that the blood with more oxygen is pumped into the aorta to supply of organs and tissues throughout the body.
  • The Fontan procedure.This surgery is usually done when the child is between 3 and 4 years of age. The surgeon creates a way for the blood from the lower part of the legs to flow directly into the pulmonary arteries. The vessel involved is called the inferior vena cava. The pulmonary arteries, then send the blood to the lungs. The Fontan procedure allows the rest of the oxygen-poor blood returning from the body to flow to the lungs. After this surgery, there is some mixing of oxygen-rich and oxygen-poor blood in the heart. So that the skin should no longer look blue or gray.
  • Heart transplant. Many infants with hypoplastic left heart syndrome needs a heart transplant. (8) Children who have heart transplants have to take medicine for life so that your body does not reject the donor heart.

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

The surgeons of the reconstruction of the aorta and connect with the heart in the bottom right of the camera. Then added a tube called a shunt to provide blood to the lungs. Surgeons can use one of two types of tubes. A type of tube that connects the main artery of the body the arteries that carry blood to the lungs. These are the pulmonary arteries. The other type of tube that goes from the right lower chamber of the heart to the pulmonary arteries. This treatment allows the right lower chamber of the heart to pump blood to both the lungs and the body.

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

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

The bi-directional Glenn procedure. This tends to be the second surgery. This is usually done when the child is between 4 and 6 months of age. It is the removal of the first shunt and connection of the large vein that drains blood from the head and arms to the pulmonary artery. Now, the lungs receive blood from the vein in place of the shunt. The large vein called the superior vena cava.

This surgery reduces the work of the right lower chamber of the heart let the blood pump mainly to the aorta. Also allows the most oxygen-poor blood returning from the body to flow directly to the lungs. After this treatment, all the blood that returns to the upper part of the body flows into the lungs. So that the blood with more oxygen is pumped into the aorta to supply of organs and tissues throughout the body.

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

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

The follow-up care

After surgery or a transplant, a baby needs care for life with a heart doctor trained in congenital heart disease to see of 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 provider if your child needs to take preventive antibiotics. Some children may also need to limit physical activity.

Follow-up care of adults

Adults who were born with hypoplastic left heart syndrome (HLHS) you need to see a heart doctor trained in congenital heart disease in adults. Recent advances in surgical care have helped the children with HLHS grow up to adulthood. So that it is not yet clear what the challenges are of an adult with the condition of the heart that you might have. Adults need regular, ongoing follow-up care to monitor changes in the condition.

The people thinking of becoming pregnant should talk with their health care professionals about the risks of pregnancy and birth control options. Have hypoplastic left heart syndrome increases the risk of:

  • Heart and blood vessel problems during pregnancy.
  • Spontaneous abortion.
  • The birth of a baby with a congenital heart defect.

Coping and support

It can be difficult to live with the hypoplastic left heart syndrome (HLHS) or to care for a baby with the disease. 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 a cardiologist, about support groups and other types of assistance. And think about the meeting with a counselor or therapist if you often feel anxious, sad, or too stressed. If you are a teen or an adult with HLHS, ask your health care team if there are support groups for people of your age with congenital heart disease. It can be helpful to talk with other people who share your challenges.
  • Maintain health records.Write your or your baby's diagnosis, medications, surgery, and other treatments. Include the dates of treatment and the name and telephone number of the physician or surgeon. Also note the emergency contact numbers for health care professionals and hospitals. Include a copy of the reports of surgery in their records. This information helps to keep a record of the care received. It is useful for new health professionals who do not know you or your child's health history. This information is also useful when the child moves from pediatric care to adult cardiology care.
  • Talk about your concerns. Talk with your child's health care team about the activities that are safer for your child. If some are off-limits, and encourage your child in other hobbies. If you're an adult with hypoplastic left heart syndrome, ask your health care professional about the activities that you can do

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

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

Maintain health records. Write your or your baby's diagnosis, medications, surgery, and other treatments. Include the dates of treatment and the name and telephone number of the physician or surgeon. Also note the emergency contact numbers for health care professionals and hospitals. Include a copy of the reports of surgery in their records.

This information helps to keep a record of the care received. It is useful for new health professionals who do not know you or your child's health history. This information is also useful when the child moves from pediatric care to adult cardiology care.

Symptoms and treatment of Hypoplastic left heart syndrome