Overview

Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). In hypoplastic left heart syndrome, the left side of the heart is critically underdeveloped.

If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body, so the right side of the heart must pump blood to the lungs and to the rest of the body.

Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past.

Source: http://www.mayoclinic.com

Symptoms

Babies born with hypoplastic left heart syndrome usually are seriously ill soon after birth. Hypoplastic left heart syndrome symptoms include:

• Grayish-blue skin color (cyanosis)
• Rapid, difficult breathing
• Poor feeding
• Cold hands and feet
• Being unusually drowsy or inactive

In a baby with hypoplastic left heart syndrome, if the natural connections between the heart's left and right sides (foramen ovale and ductus arteriosus) are allowed to close, he or she may go into shock and may die. Signs of shock include:

• Cool, clammy skin that may be pale or gray
• A weak and rapid pulse
• Abnormal breathing that may be either slow and shallow or very rapid
• Dilated pupils
• Lackluster eyes that seem to stare

A baby who is in shock may be conscious or unconscious. If you suspect your baby is in shock, immediately call 911 or your local emergency number.

When to see a doctor

It's more likely that your baby would be diagnosed with hypoplastic left heart syndrome either before birth or soon after. However, you should seek medical help if you notice that your baby has the following symptoms:

• Grayish-blue skin color
• Rapid, difficult breathing
• Poor feeding
• Cold hands and feet
• Being unusually drowsy or inactive

If your baby has any of the following signs of shock, call 911 or your local emergency number right away:

• Cool, clammy skin that may be pale or gray
• A weak and rapid pulse
• Abnormal breathing that may be either slow and shallow or very rapid
• Dilated pupils
• Lackluster eyes that seem to stare

Source: http://www.mayoclinic.com

Causes

Hypoplastic left heart syndrome occurs during fetal growth when the baby's heart is developing. The cause is unknown. However, if your family has one child with hypoplastic left heart syndrome, the risk of having another with a similar condition is increased.

A normal heart has four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — the heart uses its left and right sides for different tasks. The right side moves blood to the lungs. In the lungs, oxygen enriches the blood, which then circulates to the heart's left side. The left side of the heart pumps blood into a large vessel called the aorta, which circulates the oxygen-rich blood to the rest of the body.

What happens in hypoplastic left heart syndrome

In hypoplastic left heart syndrome, the left side of the heart can't properly supply blood to the body because the lower left chamber (left ventricle) is too small or in some cases it may not even exist. In addition, the valves on the left side of the heart (aortic valve and mitral valve) don't work properly, and the main artery leaving the heart (aorta) is smaller than normal.

For the first several days of life, the right side of the heart can pump blood both to the lungs and to the rest of the body through a blood vessel that connects the pulmonary artery directly to the aorta (ductus arteriosus). The oxygen-rich blood returns to the right side of the heart through a natural opening (foramen ovale) between the right chambers of the heart (atria). When the foramen ovale and the ductus arteriosus are open, they are referred to as being "patent."

If the ductus arteriosus and the foramen ovale close — which they normally do after the first day or two of life — the right side of the heart has no way to pump blood out to the body. Keeping these connections open is necessary for survival in the first few days of life in babies with hypoplastic left heart syndrome. This will keep blood flowing to the body until the first surgical procedure is performed.

Source: http://www.mayoclinic.com

Diagnosis

Before birth

It's possible for a baby to be diagnosed with hypoplastic left heart syndrome while it's still in the womb. Your doctor may be able to identify the condition on a routine ultrasound exam during the second trimester of pregnancy.

After birth

After your baby is born, his or her doctor may suspect a heart defect, such as hypoplastic left heart syndrome, if your baby has grayish-blue skin or has trouble breathing. Your baby's doctor may also suspect a heart defect if he or she hears a heart murmur — an abnormal whooshing sound caused by turbulent blood flow.

Doctors usually use an echocardiogram to diagnose hypoplastic left heart syndrome. This test uses high-pitched sound waves that bounce off your baby's heart to produce moving images that can be viewed on a video screen. In a baby with hypoplastic left heart syndrome, the echocardiogram reveals a smaller than normal left ventricle and aorta, and it may also show abnormal mitral or aortic valves.

Because this test can track blood flow, it also shows blood moving from the right ventricle into the aorta. In addition, an echocardiogram can identify associated heart defects, such as an atrial septal defect.

Source: http://www.mayoclinic.com

Complications

Without surgery, hypoplastic left heart syndrome is fatal, usually within the first few weeks of life.

With treatment, many babies survive, although most will have complications later in life. Some of the complications may include:

• Tiring easily when participating in sports or other exercise
• Heart rhythm abnormalities (arrhythmias)
• Fluid buildup in the lungs, abdomen, legs and feet (edema)
• Formation of blood clots that may lead to a pulmonary embolism or stroke
• Developmental problems related to the brain and nervous system
• Need for additional heart surgery or transplantation

Source: http://www.mayoclinic.com

Prevention

There's no known way to prevent hypoplastic left heart syndrome. If you have a family history of heart defects, or if you already have a child with a congenital heart defect, before getting pregnant consider talking with a genetic counselor and a cardiologist experienced in congenital heart defects.

Coping and support

Caring for a baby with a serious heart problem, such as hypoplastic left heart syndrome, can be challenging. Here are some strategies that may help make it easier:

• Seek support. Ask for help from family members and friends. Talk with your child's cardiologist about support groups and other types of assistance that are available near you.

• Record your baby's health history. It will be important to write down your baby's diagnosis, medications, surgery and other procedures and the dates they were performed, as well as the name and phone number of your child's cardiologist, emergency contact numbers for your child's doctors and hospital, and any other important information about your baby's care. It's also important to include a copy of the operative report from your child's surgeon in your records.

  This information will help you recall the care your child has received, and it will be useful for doctors who are unfamiliar with your baby to review his or her complex health history.

• Talk about your concerns. As your child grows, you may worry about activities in which he or she can safely participate. Talk with the cardiologist about which activities are best for your child. If some are off-limits, encourage your child in other pursuits rather than focusing on what he or she can't do. If other issues about your child's health concern you, discuss them with your child's cardiologist, too.

Although every circumstance is different, remember that many children with congenital heart defects grow up to lead healthy lives.