Without treatment, HLHS is fatal, but with intervention within a few days after birth, an infant may survive. A pediatric cardiac surgeon
may perform a series of operations or a full heart transplant. In the meantime, the ductus may be kept open to allow blood flow using medication containing prostaglandin
. Because these operations are complex and need to be individualized for each patient, a cardiologist must assess all medical and surgical option on a case-by-case basis.
The two methods for treatment of HLHS are transplantation
and surgical correction. If corrective surgery is performed instead of a transplant, it may involve the 3-step-Norwood procedure. In the first step (switching the right ventricle
in functional position of the absent left ventricle) it allows the right ventricle to continue to pump blood to the whole body, followed by the next two steps (which are nearly identical with the Fontan procedure) to connect the veins which return blood to the heart and pulmonary artery
. The Norwood procedure is generally performed within a week of birth, with the next two steps generally being performed within the first two years of life.