Without medicines and a series of three surgeries to rebuild the heart,
babies with hypoplastic
left heart syndrome (HLHS) won't survive. The left side of the heart can't be
fixed, so the goal of the surgeries is to rebuild parts of the heart and "redirect"
the way blood flows.
The first surgery is called the Norwood procedure. It’s usually done when
a baby is around 1–2 weeks old.
Make the right ventricle pump blood to the lungs
through a new path to the pulmonary artery (the main path
from the heart to the lungs).
What Happens During the Norwood Procedure?
The main steps of the Norwood procedure are:
Building a new, larger aorta. The bottom part of the pulmonary
artery is joined with the baby's weak, undeveloped aorta. This new aorta is now the
path from the right ventricle to the body.
Because the bottom of the pulmonary artery is used to make the new aorta, a new
path from the heart to the lungs has to be built by:
Using a shunt
to get blood to the lungs. A round tube or "shunt" routes blood either from
the aorta (with a Blaylock-Taussig or BT shunt) or directly from the right ventricle
(with a Sano shunt) to the pulmonary artery. The care team will decide which shunt
is best for the baby.
The shunt is a temporary fix. It helps the baby get blood to the lungs until the
next surgery, which creates a more permanent solution.
Closing the patent
ductus arteriosus (PDA). Until now, the PDA was the only way blood could
flow to the body. Now that the right ventricle has taken over pumping to the body,
the PDA isn't needed anymore.
Opening the atrial
septal defect. To make sure blood with oxygen gets back to the right
ventricle, the atrial septal defect is made bigger.
What Happens After the Norwood Procedure?
Babies who have the Norwood procedure usually spend 3 to 4 weeks in the hospital
to recover. They get-around-the-clock care and monitoring. They also get medicines
to help the heart and improve blood flow.
During this time, the care team teaches parents how to care for their baby at home.
Babies usually can go home when they are feeding well, growing well, and gaining weight.
At home, the baby needs to be watched closely. Parents will be in close contact
with the care team, and should keep an eye on:
weight gain and growth
Parents should call the care team right away if their baby:
has feeding problems
has breathing problems
seems very irritable
just doesn't seem quite right
What's the Next Procedure?
The second surgery is called the Glenn
procedure. It's usually done when a baby is 4 to 6 months old.