What Is Tricuspid Atresia?
Tricuspid atresia is a condition seen in newborns in which they’re
born without an important part of the heart called “the tricuspid valve.” This
valve plays a part in the heart’s essential function, which is to pump blood
between the lungs and body.
In some newborns, the tricuspid valve is missing or hasn’t
properly developed. Tricuspid atresia is a rare heart disorder that’s
congenital, or present at birth. It’s a serious condition, and it can be
How Does Tricuspid Atresia Affect the Heart?
In a properly functioning heart, blood flows from the body
to the heart and then to the lungs, where each blood cell will be loaded up
with oxygen. This process is known as oxygenation. After oxygenation, the blood
returns to the heart and is pumped out to the body.
Four different heart valves control the flow of blood in
your heart. The valves allow the blood to flow between the four different
chambers that make up your heart. These four chambers are the right and the
left atria and ventricles. A wall known as the septum separates them.
The tricuspid valve is one of the four valves that allow
blood to flow through your heart. It’s on the right side of your heart. When a
newborn is missing their tricuspid valve, their heart has to circulate blood
through the heart using two holes in the septum instead. One hole is between
the left and the right atria. The other is between the left and the right
ventricles. All unborn babies have a hole between the atria known as the
foramen ovale, but it usually closes up before birth, as part of the
development of the fetus.
In newborns with tricuspid atresia, the missing tricuspid
valve causes the heart to circulate blood using the hole between the atria
instead. Instead of closing up, the hole stays open, or in some cases gets even
larger. The hole stays open or enlarges between the two atria to allow blood to
circulate from the body into the heart. When this happens, the hole is called an
atrial septal defect (ASD).
For the heart to be able to then pump blood out to the body
and the lungs, a baby with tricuspid atresia has to develop another hole as
well. This hole is known as a ventricular septal defect (VSD). A baby has to be
born with a VSD when their tricuspid valve is missing so that their heart can effectively
pump blood. The VSD is located between the left and the right ventricles. This
allows blood to flow from the left ventricle to the pulmonary artery in the
In some newborns with tricuspid atresia, a fetal vessel
called the ductus arteriousus stays open to allow blood to circulate to the
lungs from the aorta.
What Are the Types of Tricuspid Atresia?
There are three forms of tricuspid atresia. The type is based
on how large the VSD is:
If the hole in your newborn’s heart is large, too much blood
may go to the lungs. This can result in a condition known as congestive
heart failure (CHF).
If your newborn has a moderate VSD, the hole is
medium-sized. This hole allows some blood to pump to the lungs. Newborns with
this condition will experience less distress than those with large or small
If the hole is small, not enough blood pumps to the lungs to
pick up oxygen. A lack of oxygen to vital organs can cause your newborn’s skin
to turn blue. This is called cyanosis.
What Are the Symptoms of Tricuspid Atresia?
The symptoms of tricuspid atresia depend on the specific heart
defect that’s present. Babies that are born with tricuspid atresia usually show
signs of distress within the first few hours of life. However, some babies may
be healthy at birth and only begin to show symptoms in the first two months of
Common symptoms associated with tricuspid atresia include:
- rapid breathing
- shortness of breath, or dyspnea
- a bluish skin color, or cyanosis
- fatigue that occurs during feeding
- slow growth
If the hole in the newborn’s heart is big enough to cause CHF,
there may be other symptoms associated with this condition. These include
coughing and swelling in the legs and feet, which is known as “edema.”
What Causes Tricuspid Atresia?
The heart begins to form when a fetus is just 8 weeks old.
Tricuspid atresia is a result of the tricuspid valve failing to develop during
this period of growth. Doctors don’t know why this happens. Tricuspid atresia
is often common in families. It also occurs with certain genetic or chromosomal
abnormalities, including Down syndrome.
How Is Tricuspid Atresia
Your doctor may identify tricuspid atresia while your baby
is still in the womb. Routine prenatal ultrasounds that monitor the growth of
your baby may detect abnormalities in the heart of the fetus.
After your baby is born, your doctor will be able to
diagnose tricuspid atresia by performing a physical exam. Your doctor may find a
heart murmur and may notice a bluish tone to your baby’s skin. Your doctor may
also perform several tests to confirm the diagnosis. Tests may include:
- an ultrasound of the heart
- an electrocardiogram
- an echocardiogram
- a chest X-ray
- a cardiac catheterization
- an MRI of the heart
How Is Tricuspid Atresia Treated?
If your baby is diagnosed with tricuspid atresia, they’ll
enter a neonatal intensive care unit (NICU). In the NICU, your baby will
receive specialized care to treat the condition. If your baby can’t breathe,
they may go on a breathing machine, or ventilator. Your baby may also receive
medication to maintain their heart function.
After your baby’s condition stabilizes, they’ll need surgery
to correct the heart defect. For infants who have tricuspid atresia, three
surgeries are typically necessary as the child’s heart and body grow and
In most infants with this condition, the first heart surgery
happens in the first few days of life. This procedure involves placing
a shunt in the heart to keep blood flowing to the lungs.
Following the surgery, your baby can go home. They’ll need
medications and monitoring to detect complications. A pediatric cardiologist
will oversee your baby’s care. This doctor will determine when your baby is ready
for a second surgery.
The second surgery typically happens when your baby is
between 3 and 6 months old. This operation is known as the Glenn
shunt or hemi-Fontan procedure.
The vein that carries blood into your heart after it has
delivered oxygen throughout your body is called the superior vena cava. The
artery that funnels blood from your heart to your lungs is called the right
pulmonary artery. This procedure is done to connect the superior vena cava to
the right pulmonary artery.
Following that surgery, your baby will require one more
procedure that will take place when your baby is between 18 months and 5 years
of age. This procedure is known as the Fontan procedure. In
this final surgery, your child’s surgeon will create a path for oxygen-depleted
blood returning to the heart to flow into the arteries that will take it
directly to the lungs.
In the rare instance that your child isn’t a candidate for
these surgical procedures, your child’s doctor will discuss the possibility of
a heart transplant.
What Is the Long-Term Outlook?
If your baby has tricuspid atresia, they’ll need continued
care for years after successful treatment. Open-heart surgery will be
necessary. This surgery carries with it a number of potential complications,
including heart failure.
After the surgeries to correct tricuspid atresia are done,
your baby will require ongoing care and monitoring into adulthood. Children
with tricuspid atresia are at increased risk for the following across their
- a stroke
They may need to take medications to help prevent these
conditions. Your healthcare team will work with you to figure out a plan of
care for your baby.