Hypoplastic left heart

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Heart, front view

Heart, section through the middle

Hypoplastic left heart syndrome; HLHS

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 maintain circulation of blood to the body.

These measures are only supportive; they do not solve the problem. The condition always requires surgery.

The first surgery, called the Norwood operation, occurs within the first few days of life. Stage I of the Norwood procedure consists of building a new aorta, and an artificial shunt is inserted to maintain blood flow to the lungs.

Afterwards, the baby usually goes home. The child will need to take one or more 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 Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the body directly to the lungs that get oxygen. The surgery is usually done when the child is between 4 and 6 months of age.

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

Stage III, the final step, is called the Fontan procedure. The remainder of the veins carrying blue blood from the body is connected directly to the lungs, and the right ventricle now serves as the only pumping chamber for the heart. This surgery is usually performed between the ages of 18 months and 3 years of age. After this final step, the baby is no longer blue (cyanotic).

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 to be a better alternative to the 3-step surgery process, but there are few donated hearts available for small infants.

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