The management of the newborn with hypoplastic left heart syndrome can be divided into the initial stabilization period and the operative / post-operative period.
If the fetus has been diagnosed before delivery, stabilization measures are started immediately so the newborn does not become unstable. In newborns that are delivered and then suspected of having hypoplastic left heart syndrome, stabilization begins even while diagnostic tests are going on. The rapid stabilization of these infants must begin as soon as the diagnosis is suspected.
Catheters are placed, usually in the umbilical blood vessels, which allow medications to be given and blood to be obtained for testing. An infusion of prostaglandin, a medication that prevents the patent ductus arteriosus from closing, is begun, thus maintaining the pathway for blood to reach the body from the right ventricle.
Even though the infant may have low oxygen saturations, supplemental oxygen is avoided since it tends to promote more blood flow to the lungs, which may steal blood flow from the body and place excessive demands on the already stressed single right ventricle.
Manipulations of medications and respiratory treatments (including possible mechanical ventilation) are performed to optimally balance the flow of blood to the body and the flow of blood to the lungs.
Close monitoring is essential to detect any organ dysfunction and maintain cardiopulmonary stability because infants with this anomaly may be very unpredictable and undergo quite sudden changes.
There are essentially three treatment options that have been proposed for children with hypoplastic left heart syndrome.
In the past, due to poor outcomes with available treatments at that time, no treatment was often recommended. Today it is rare that a family may choose not to treat a child with HLHS, though in cases when the infant is unable to be satisfactorily stabilized, no treatment may be advised.
Cardiac transplantation in the newborn period is performed as primary treatment for hypoplastic left heart syndrome at some centers in this country. While transplantation has the advantage of replacing the very abnormal heart of a child with HLHS with one of normal structure, this treatment is limited by the scarcity of newborn organs available for transplantation and the life-long need for anti-rejection therapy. Additionally, although outcomes for transplantation continue to improve, and the incidence of rejection is lowest in patients transplanted as newborns, the average life span of the transplanted heart is limited (currently less than 15 years).
The most commonly pursued treatment for hypoplastic left heart syndrome is "staged reconstruction" in which a series of operations, usually three, are performed to reconfigure the child's 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 instead considered "palliative."
The first operation in the staged approach is known as the Norwood operation and is typically performed in the first week of life. With the Norwood operation, the right ventricle becomes the systemic or main ventricle pumping to the body. A "new" or "neo" aorta is made from part of the pulmonary artery and the original, tiny aorta, which is reconstructed / enlarged to provide blood flow to the body. Finally, to provide blood flow to the lungs, a small tube graft is placed either from an artery to the lung vessels (called a modified Blalock-Taussig shunt) or from the right ventricle to the lung vessels (called a Sano modification). Because of the extensive reconstruction of the aorta that must be done, this operation is one of the most challenging heart surgeries in pediatrics.
The subsequent operations in the staged reconstruction plan are:
- The bi-directional Glenn procedure, typically done at 3 to 6 months of age
- The Fontan operation, typically done in children older than 2 or 3 years.
These operations are described in more detail in the Heart Encyclopedia chapter on single ventricle cardiac anomalies.