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Hypoplastic Left Heart Syndrome by Deanna M. Neff, MPH

Definition

Hypoplastic left heart syndrome is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood then moves out to the rest of the body.

With this syndrome, structures on the left side of the heart (aorta, aortic valve, left ventricle, and mitral valve) may be:

Too small
Absent
Abnormally developed

Since the heart cannot function properly, oxygen-rich blood flow to the body is limited. This condition requires immediate care from a doctor.

Heart Chambers and Valves

Blood Flow Through the Heart

Causes

This is a congenital heart defect. This means that the heart forms incorrectly when the baby is developing in the womb. The baby is born with the condition. It is not known why the heart forms this way.

Risk Factors

These factors increase your chance of having a child with hypoplastic left heart syndrome:

Previous pregnancy with fetal heart abnormalities or fetal loss
Family history of congenital heart defect

Symptoms

Symptoms usually appear within days after birth. Tell the doctor if you notice the following in your infant or child:

Blue/gray skin color
Cool skin
Rapid or difficult breathing
High heart rate
Sweaty, clammy skin
Poor feeding

These symptoms may be caused by other conditions. If your child has any of these, tell the doctor right away.

Diagnosis

Your doctor will:

Ask about your child’s symptoms and medical history
Do a physical exam

Tests may include:

Echocardiogram —a test that uses high-frequency sound waves (ultrasound) to examine the size, shape, and motion of the heart
Chest x-ray —imaging test that uses low amounts of radiation to get a picture of the chest
Electrocardiogram (ECG, EKG)—a test that records the heart’s activity by measuring electrical currents through the heart muscle
MRI scan —a test that uses magnetic waves to make pictures of structures inside the body
CT scan —a type of x-ray that uses a computer to make pictures of structures inside the body
Cardiac catheterization —a thin tube is threaded through a vein to the heart where blood samples and blood pressure are measured

Treatment

Talk with your doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:

Medications

Medicines, such as Prostaglandin E1, may be given. This medicine helps to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and aorta (largest artery in the body). It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medicines may be used, as well.

Surgery

Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done to improve blood flow. Treatment will include more surgeries, which are usually done in stages:

After birth
4-6 months of age
2-4 years of age

In addition to the shunt procedure, the initial surgery may also create a connection between the aorta and the right ventricle. This is done so that the right ventricle pushes blood to both the lungs and the rest of the body. This is called a Norwood Procedure. As the child ages, more surgeries will be needed to create other connections with the heart, lungs, and body. In some cases, a heart transplant may be needed.

Lifelong Monitoring

Your child will need to see a heart specialist regularly. Heart medicine will be needed throughout your child's life.

Prevention

There is no way to prevent this condition. Getting appropriate prenatal care is always important.

RESOURCES:

American Family Physician http://www.aafp.org/
American Heart Association http://www.americanheart.org/

CANADIAN RESOURCES:

Canadian Cardiovascular Society http://www.ccs.ca/
Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

References:

American Heart Association. Hypoplastic left heart syndrome. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=1353 . Updated August 18, 2010. Accessed July 15, 2010.
Children’s Hospital Boston. Hypoplastic left heart syndrome. Children’s Hospital Boston website. Available at: http://www.childrenshospital.org/az/Site502/mainpageS502P0.html . Accessed July 15, 2010.
DynaMed Editorial Team. Hypoplastic left heart syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 10, 2010. Accessed July 15, 2010.
Johns Hopkins University, Cove Point Foundation. Hypoplastic left heart syndrome. Johns Hopkins University, Cove Point Foundation website. Available at: http://www.pted.org/?id=hypoplasticleft4 . Updated April 3, 2009. Accessed July 15, 2010.
Mayo Clinic. Hypoplastic left heart syndrome. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/hypoplastic-left-heart-syndrome/DS00744 . Updated May 27, 2010. Accessed August 2, 2010.

Last reviewed September 2012 by Michael Woods, MD

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