There are essentially three treatment options that have been proposed for children with Hypoplastic Left heart syndrome:
The most common treatment for HLHS is ‘staged reconstruction’ in which a series of operations are performed to reconfigure the cardiovascular system to be as efficient as possible despite the lack of an adequate left ventricle. These surgeries do not correct the lesion and are considered to be palliative.
- The first surgery is known as the Norwood operation and is usually performed in the first week of life. The Norwood operation turns the right ventricle into the systemic or main ventricle pumping blood to the body.
- The second surgery is the Bi-directional Cavo-pulmonary shunt (or Glenn procedure). This is typically carried out at around 3 months of age. This procedure connects the Superior vena cava to the pulmonary arteries so that blood can be shunted directly into the lungs to be oxygenated.
- The final surgery is the Fontan operation and can be done in number of different ways. It is usually completed when the child is three to four years old but may be done later. The aim of the operation is to improve oxygen saturation levels by directing venous (blue) blood directly to the lungs.
Please note that some of these surgeries are known by different names. There may also be variations on how they are completed depending on the individual anatomy of the patient and on the hospital at which they are carried out. Additionally, there are related surgeries that may be used in conjunction with, or instead of those described above. More information about this can be found in our links pages.
The survival rates for Heart Transplantation and the Norwood procedure are about the same. However, in most cases the Norwood procedure is used because of the shortage of donor hearts for transplantation. If any of the staged palliation surgeries fail, heart transplant may be an “acceptable strategy” for HLHS patients.
In the past, due to poor outcomes with available treatments at that time, no treatment was often recommended. Compassionate care may still be advised today in cases when the infant is unable to be satisfactorily stabilized.