Hypoplastic Left Heart Syndrome

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, which includes the 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

Copyright © Nucleus Medical Media, Inc.

Blood Flow Through the Heart

Copyright © Nucleus Medical Media, Inc.

Causes

Hypoplastic left heart syndrome is present at birth. It is not known exactly why the heart does not develop normally.

Risk Factors

Factors that may increase your chance of having a child with hypoplastic left heart syndrome include:

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

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

Diagnosis

You will be asked about your child's symptoms and medical history. A physical exam will be done.

Images may be taken of your child's chest. This can be done with:

• Echocardiogram
• X-ray
• MRI scan
• CT scan
• Cardiac catheterization

Electrocardiogram (EKG) can monitor the heart's electrical activity.

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

Medications are necessary to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta, which is the largest artery in the body. It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medications may be used as well.

Surgery

Surgery may be done to improve blood flow. This can be done through a variety of reconstructive and shunting procedures. Surgeries are usually done in stages:

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

Lifelong Monitoring

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

Prevention

There are no current guidelines to prevent hypoplastic left heart syndrome because the cause is unknown. Getting proper prenatal care is always important.