Hypoplastic left heart syndrome is a type of congenital heart defect. Congenital heart defects are heart problems a baby is born with. These heart problems are usually diagnosed at or before birth.
"Hypoplastic" means "not developed." A "syndrome" is a group of problems. So hypoplastic left heart syndrome is a group of problems caused when the baby's heart hasn't grown the way it should. The main problem is that the structures on the left side of the heart are much too small.
In a normal heart, the left ventricle takes in oxygen-rich blood (red blood) from the lungs and pumps it out through the large blood vessel called the aorta and out to the body. The right ventricle takes in oxygen-poor blood (blue blood) from the body and pumps it to the lungs to get oxygen.
In hypoplastic left heart syndrome, the left ventricle and the aorta are both too small. So the right ventricle has to take over and do both pumping jobs.
Most babies with this problem also have an atrial septal defect. This is a hole in the wall between the left and right sides of the heart. Some of the blood flows through the hole. So too much blood goes to the lungs and not enough red blood goes to the body.
The baby's body is still able to get some oxygen because of a small blood vessel that connects the left and right sides of the heart. This blood vessel is called the ductus arteriosus. It is part of the circulation system before birth. It usually closes shortly after birth. Medicine can keep it open for a while. The opening lets red blood mix with blue blood so that at least some oxygen goes out to the baby's body.
Your baby will need special care, such as being in the neonatal intensive care unit (NICU). This may be scary for you. But the hospital staff understands this. They will explain what happens and will answer your questions.
Your doctor may hear abnormal heart sounds, such as a heart murmur, when he or she examines your newborn.
Your doctor will order tests to find the cause of abnormal sounds or of symptoms. The most common test used to identify this defect is called an echocardiogram, or "echo" for short. It uses sound waves to make an image of your baby's heart.
Other tests, such as an EKG (electrocardiogram), chest X-ray, and checking the amount of oxygen in the blood, also help identify the problem.
A fetal ultrasound, which looks at the baby's heart, may find this defect before birth.
The heart is not pumping well, so most babies get very sick soon after birth because there is not enough blood being pumped out to the body. Symptoms include fast breathing and a blue tint to the skin.
Some babies may not have symptoms until 12 to 24 hours after birth.
Deciding what treatment is right for your baby can be very hard. Your doctor will help you understand your choices and what to expect from each choice.
Your baby may get medicine to keep the opening between the left and right sides of the heart open. This helps keep red blood flowing to the body and helps prepare your baby for surgery. The medicine may be given through a blood vessel. It is often given through the belly button.
If there is no opening between the left and right sides of the heart, your doctor may do a treatment to create one. This treatment is called catheterization. The doctor puts a thin tube into a blood vessel in your baby's groin. This tube is called a catheter. The doctor moves the catheter through the blood vessel to the heart. He or she moves special tools through the catheter to the heart to create the hole. Your baby will be asleep during this treatment.
Sometimes a series of surgeries is done to help the baby's heart work better. The first surgery is usually done shortly after birth. A second surgery may be done a few months later. If a third surgery is planned, it is done when the baby is about 18 to 30 months old.
The main goal of the surgeries is to reroute some of the blood vessels around the heart. That will make the right ventricle the main pumping chamber of the heart, instead of the left ventricle. This will not make the heart work like a normal heart. But it will help the heart get red blood to the body.
If you and your doctor decide not to do surgery, your doctor may suggest other treatment to help your baby's symptoms and keep your baby comfortable.
Follow-up care is a key part of your child's treatment and safety. Be sure to make and go to all appointments, and call your doctor or nurse call line if your child is having problems. It's also a good idea to know your child's test results and keep a list of the medicines your child takes.
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Current as of: December 6, 2017
Adam Husney, MD - Family Medicine
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