Once the diagnosis of hypoplastic left heart is made, a nunber of things are done. First, the patient is put on a ventilator to assist with breathing if necessary. A medicine called prostaglandin E1 is started to prevent a shunt called the ductus arteriosus from closing. Intravenous fluids are started and medicine to help the heart beat stronger is often begun.
These measures are only supportive; they do not solve the problem. The next step is surgery.
There are two surgical approaches to hypoplastic left heart syndrome. One is to do a heart transplant early in life. This means that a small donor heart, which is very rare, must be available. It also means that the patient will have to take a number of medicines to prevent rejection of the new heart.
The other option is a series of operations done over the first 3 years of life; this option is called the Norwood procedure. Stage I of the Norwood procedure is done within the first few days of life and consists of attaching the functioning right ventricle to the aorta (vessel that delivers blood to the body).
Because the lungs must still receive blood, a surgically created conduit (tunnel) is created to attach the a large artery in the chest to the pulmonary arteries (the vessels that lead to the lungs). This conduit is called a Blalock-Taussig (BT) shunt.
In the stage II operation, called the Glenn or Hemi-Fontan procedure, the superior vena cava (the vessel that removes deoxygenated blood from the head and arms) is attached to the pulmonary arteries and the BT shunt is removed.
During stage III, called the Fontan procedure, the inferior vena cava (the vessel that returns deoxyganted blood from the lower half of the body) is also attached to the pulmonary arteries. This is usually performed between the ages of 2-3 years. After the third stage the patient is no longer blue (cyanotic).
The Norwood procedure is relatively new, but outcomes have been good so far. While it is possible that these patients' hearts eventually will fail and they will need a heart transplant, it is much easier to transplant an older patient than an infant.
