Treatment:

Once the diagnosis of hypoplastic left heart is made, the baby will be admitted to the neonatal intensive care unit. A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to keep blood circulating to the body by keeping the ductus arteriosus open.

These measures do not solve the problem. The condition always requires surgery.

The first surgery, called the Norwood operation, occurs within the baby's first few days of life. Stage I of the Norwood procedure consists of building a new aorta by:

• Using the pulmonary valve and artery
• Connecting the hypoplastic old aorta and coronary arteries to the new aorta
• Removing the wall between the atria (atrial septum)
• Making an artificial connection from either the right ventricle or a body-wide artery to the pulmonary artery to maintain blood flow to the lungs (called a shunt)

Afterwards, the baby usually goes home. The child will need to take daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done.

Stage II of the operation is called the Glenn shunt or hemi-Fontan procedure. This procedure connects the major vein carrying blue blood from the top half of the body (the superior vena cava) directly to blood vessels to the lungs (pulmonary arteries) to get oxygen. The surgery is usually done when the child is 4 to 6 months of age.

During stages I and II, the child may still appear somewhat blue (cyanotic).

Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body (the inferior vena cava) are connected directly to the blood vessels to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually performed when the baby is 18 months - 3 years old. After this final step, the baby is no longer blue.

Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure.

In some hospitals, heart transplantation is considered a better choice than the three-step surgery process. However, there are few donated hearts available for small infants.

Expectations (prognosis):

If left untreated, hypoplastic left heart syndrome is fatal. Survival rates for the staged repair continue to rise as surgical techniques and postoperative management improve. Survival after the first stage is more than 75%.

The size and function of the right ventricle are important in determining the child's outcome after surgery.

Complications:

Complications include:

• Blockage of the artificial shunt
• Chronic diarrhea (from a disease called protein losing enteropathy)
• Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
• Heart failure
• Irregular, fast heart rhythms (arrhythmias)
• Strokes and other neurological complications
• Sudden death

Calling your health care provider:

Contact your health care provider immediately if your infant:

• Eats less (decreased feeding)
• Has blue (cyanotic) skin or mucus membranes
• Has new changes in breathing patterns