What is hypoplastic left heart syndrome?
Sometimes during pregnancy,
the left side of the baby's heart does not grow properly. This causes a rare
anomaly called hypoplastic left heart syndrome.
Normally, the right side of
the heart pumps blood from the heart to the lungs, where it receives oxygen.
Returning to the heart, the left side pumps oxygen-rich blood to the rest of
the body.
The heart of a child with
hypoplastic left heart syndrome cannot do this. The lower left chamber may be
smaller than normal or absent altogether. The valves on the left side may not
be working properly, or the main artery from the heart may not be as large as
it should be.
A child with hypoplastic left
heart syndrome may also have a hole between the left and right upper chambers
of the heart. This is called an atrial septal defect and can result in too much
blood flow to the lungs.
Signs of hypoplastic left
heart syndrome may not appear until a few days after birth. During the first
two days, the baby's heart can adjust to the standard state. If the left side
of the heart cannot pump blood well to the rest of the body, the right side
will do more work. But adaptation lasts only a few days.
A newborn baby has a blood
vessel that connects the two sides of its heart. This is called a persistent
ductus arteriosus, and it only stays open for a few days after birth. After
that, it naturally closes. It is then that most children with a defect begin to
show symptoms of a lack of oxygen-rich blood and overwork of the heart.
Cause
Doctors don't know why
hypoplastic left heart syndrome occurs, but it's a hereditary condition. Some
experts believe that what a mother eats, drinks, or comes into contact with
during pregnancy may also increase the risk of her baby developing hypoplastic
left heart syndrome. This may include smoking or drinking alcohol, or not
taking prenatal vitamins with folic acid.
Symptoms and diagnosis
Heart defects can be detected
on ultrasound in the second trimester of pregnancy. Thus, some parents discover
it even before the baby is born.
In other cases, hypoplastic
left heart syndrome is detected a few days after the birth of the child. Signs
include:
- Drowsiness or lack of desire to move
- Cold hands and feet
- Fast breathing or difficulty breathing
- Gray or blue skin
- Poor eating
When your doctor listens to
your child's heartbeat, they may hear a heart murmur that sounds like a
whistle. This is due to unusual causes of hypoplastic left heart syndrome.
If your doctor thinks your
child may have hypoplastic left heart syndrome, they will order an
echocardiogram. In this test, sound waves are used to create images of her
heart on a video screen. It can show the chambers of the heart and track blood
flow.
Treatment
It is important that
hypoplastic left heart syndrome be diagnosed and treated immediately.
Otherwise, your baby's organs won't get enough blood. This may shock him.
Treatment includes:
- Medication: Your child may be given a medicine called alprostadil to keep the ductus arteriosus open. He may also need medication to strengthen his heart muscle, lower his blood pressure, and help the body get rid of excess fluid.
- Feeding and breathing assistance: Your baby will be weak and will likely need fluids
through a drip or feeding tube. A breathing device called a ventilator can also
be used to make sure your child is getting enough oxygen.
- Atrial septostomy. If
your child does not have an atrial septal defect, your doctor may recommend
this surgery. It creates an opening between the upper chambers of the heart to
allow more blood to pass through.
A child with hypoplastic left
heart syndrome may also have a series of heart repair surgeries. The first,
called the Norwood procedure, is performed shortly after birth. This is a
complex operation that creates a new aorta for your child and allows the right
ventricle of the heart to pump all the blood into the body (after the canal is
closed).
Two more operations usually
follow to restore the heart and maintain normal blood flow. Timing depends on
your baby's condition, but the next surgery, known as Glenn's surgery, is
usually done when your baby is a few months old. The third operation is called
the Fontan operation and is performed at the age of 3 or 4 years.
In some cases, a doctor may
suggest a heart transplant. This will give your child a healthy heart, but it
may take time to find a donor. She will also have to take medication for the
rest of her life so that her body does not reject them.
Your baby will need lifelong
care from a cardiologist who specializes in birth defects. Your
child is also likely to need other surgeries and is more likely to have other
heart problems such as irregular heartbeats and blood clots.
An infant who has undergone heart repair surgery may grow up physically weaker than other children and have developmental problems. He may need additional support at home and at school.