Tricuspid atresia is a type of congenital heart disease in which the valve between the right atrium

and right ventricle fails to develop.

In a normal heart, oxygen-poor blood from the superior and inferior venae cavae goes into the
right atrium to the right ventricle through the tricuspid valve, the right atrioventricular valve,
Right Ventricle then pumps the it through the pulmonary arteries which carry the blood to the
lungs for oxygenation. Oxygen-rich blood returns to the left atrium of the heart via the
pulmonary veins. Left ventricle then sends oxygenated blood out of the body via the aorta to
begin the systemic circulation.
However, in tricuspid valve atresia, systemic venous blood cannot flow appropriately through the
heart and into the lungs to pick up oxygen because the tricuspid valve, the opening of between
the right atrium and right ventricle is not present. Instead of opening that helps prevent a
backflow, it is a solid tissue.
Right ventricle is also underdeveloped (Hypoplastic Right Ventricle). Since it does not get to
pump blood the muscles does not develop and the right ventricle is just a tiny little sac. It is not
functional and therefore theres no blood going to the lungs either.
Since the deoxygenated blood that returns to the right atrium cannot directly enter the right
ventricle, it flows through a hole in the atrial septum (atrial septal defect) into the left atrium.
Left atrium also receives oxygenated blood form the lungs through the pulmonary veins resulting
in a mixing of oxygenated and deoxygenated blood. The mixture of oxygenated blood and
deoxygenated blood then flows through the mitral valve into the left ventricle and from there it
goes to the aorta to the body. Aside from going to the aorta, the blood that entered the left
ventricle also crosses the ventricular septal defect (a hole in the ventricular septum) and enters
right ventricle in to the pulmonary trunk, goes to the lungs to pick up oxygen and comes back.
Tricuspid atresia is a congenital heart defect characterized by the absence of the right
atrioventricular valve (Tricuspid valve), hypoplasia of the right ventricular and atrial septum
defect. They usually also have a ventricular septal defect , a hole between the right ventricle
and left ventricle, which may obstruct pulmonary blood flow or result in functional subaortic
stenosis when the great arteries are transposed. . The aorta and pulmonary artery (great arteries)
are either normally related or transposed (aorta the large artery that carries blood to the body
arises from the small right ventricle, and the pulmonary artery (lung artery) arises from the
large left ventricle), or both arise from the left ventricle. Tricuspid atresia in children is often
associated with pulmonary stenosis or narrowing of the pulmonary valve

Tricuspid atresia can also be associated with transposition of the great arteries , where the.

Tricuspid atresia is a single-ventricle lesion, because the heart has only one functioning ventricle
(the left ventricle).

Symptoms
Cyanosis is the appearance of blue to purple coloration of the skin or mucous membranes due to
the tissue near the skin surface having low oxygen saturation. This cyanosis may be noted at
birth, or may only become evident after several days when the ductus arteriosus closes and little
blood flows to the lungs. As the ductus closes the cyanosis worsens. If the cyanosis is severe
enough, the baby will develop symptoms of poor oxygenation such as fast breathing or poor
feeding.
Occasionally, there is an increased amount of blood flow to the lungs. This baby may have little
to no cyanosis but will likely develop congestive heart failure because of this imbalance.
The increased workload on the left ventricle and easier path of blood flow to the lungs causes
them to become engorged with blood and causes fluid to leak from the bloodstream into the air
spaces of the lungs. This condition is called pulmonary edema and makes it harder for a baby
with this condition to breathe comfortably.
The combination of increased heart and lung work uses large amounts of calories and results in
the constellation of symptoms referred to as congestive heart failure (CHF).
The symptoms of congestive heart failure are fast breathing, fast heart rate, sweating with feeds
and poor weight gain.
If there is transposition of the great arteries, the presenting symptoms are related to low blood
flow through the aorta and out to the body. This results in low blood pressure, fast breathing,
poor feeding, cold, clammy hands and feet, and a pale, gray color.
Infants presenting with these symptoms are critically ill and require emergency medical

How is tricuspid atresia diagnosed?

A pediatric cardiologist and/or a neonatologist will be involved in your child's care. A
pediatric cardiologist specializes in the diagnosis and medical management of
congenital heart defects, as well as heart problems that may develop later in childhood.
A neonatologist specializes in illnesses affecting newborns, both premature and fullterm.
Cyanosis is the major indication that there is a problem with your newborn. Your
child's doctor may have also heard a heart murmur during a physical exam. In this case,
the heart murmur is a noise caused by the turbulence of blood flowing through the
openings that allow the blood to mix or across an obstructed pulmonary valve.
Diagnostic testing for congenital heart disease varies by the child's age, clinical
condition, and institutional preferences. Some tests that may be recommended include
the following:

Chest X-ray. A diagnostic test that uses invisible X-ray beams to produce images
of internal tissues, bones, and organs onto film.

Electrocardiogram (ECG). A test that records the electrical activity of the

heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart
muscle stress.

Echocardiogram (echo). A procedure that evaluates the structure and function

of the heart by using sound waves recorded on an electronic sensor that produce a
moving picture of the heart and heart valves.

Cardiac catheterization. A cardiac catheterization is an invasive procedure

that gives very detailed information about the structures inside the heart. Under
sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the
groin, and guided to the inside of the heart. Blood pressure and oxygen measurements
are taken in the chambers of the heart, as well as the pulmonary artery and aorta.
Contrast dye is also injected to more clearly visualize the structures inside the heart.

attention.

How is tricuspid atresia treated?

Your child will most likely be in the neonatal intensive care unit (NICU). Early medical treatment may include:

Supplemental oxygen or possibly a ventilator, to help with breathing.

IV medicines. Prostaglandin E1 may be given to keep the ductus arteriosus open. Other medicines may be
given to help the heart and lungs work better.
Other early treatment may include:

Cardiac cath. This may be used to treat some heart defects.

Balloon atrial septostomy. A special catheter with a balloon in the tip is used to create or enlarge an opening
in the wall between the left and right atria (atrial septum). This helps with the flow of blood.
Three surgeries, in stages, may be done. They are:

Blalock-Taussig shunt. This surgery is done on newborns. It varies, depending on the exact defect. A
connection (shunt) is created to allow blood to reach the lungs.

Glenn shunt. This surgery may be done on babies from age 3 to 6 months. A new connection (shunt)
replaces the first shunt. This shunt connects the large blood vessel from the top of the body to the heart (superior
vena cava) to the pulmonary artery. This allows blood to get to the lungs for oxygen.

Fontan procedure. This surgery may be done from age 2 to 3 years. A second connection (shunt) is
made. It allows blood from the large blood vessel from the lower part of the body to the heart to flow to the pulmonary
artery. This surgery varies depending on the exact defect, as well as other factors.
Heart transplant is also a choice, but it is not usually done because it is difficult to find a donor heart. Also the
surgeries to improve blood flow have become more and more successful.