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.
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%.
It is important to note that the size and function of the right ventricle are important determinants of outcome after surgery.
Complications:
If untreated, heart failure continues to worsen and will result in death.
Calling your health care provider:
If your infant shows any of the symptoms of hypoplastic left heart syndrome, contact your health care provider immediately.
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