Tricuspid Atresia

Tricuspid atresia is a heart defect present at birth (congenital) in which a valve (tricuspid
valve) between two of the heart's chambers isn't formed.
Instead, there's solid tissue between the chambers, which restricts blood flow and causes
the right lower heart chamber (ventricle) to be underdeveloped.
Symptoms
Tricuspid atresia symptoms become evident soon after birth, and can include:
• Blue tinge to the skin and lips (cyanosis)
• Difficulty breathing
• Tiring easily, especially during feedings
• Slow growth and poor weight gain
Causes
Tricuspid atresia occurs during fetal heart development. Some genetic factors, such as
Down syndrome, might increase your baby's risk of congenital heart defects such as
tricuspid atresia, but the cause of congenital heart disease is usually unknown.
Risk Factors
In most cases, the cause of a congenital heart defect, such as tricuspid atresia, is unknown.
However, several factors might increase the risk of a baby being born with a congenital heart defect,
including:
• A mother who had German measles (rubella) or another viral illness during early pregnancy
• A parent who has a congenital heart defect
• Older parental age at conception
• Mother's obesity
• Drinking alcohol during pregnancy
• Smoking before or during pregnancy
• A mother who has poorly controlled diabetes
• Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Claravis,
Amnesteem, others), some anti-seizure medications and some bipolar disorder medications
• The presence of Down syndrome, a genetic condition that results from an extra 21st chromosome
Complications
A life-threatening complication of tricuspid atresia is a lack of oxygen to
your baby's tissues (hypoxemia).
Complications later in life
• Although treatment greatly improves the outcome for babies with
tricuspid atresia, complications can develop later in life, including:
• Formation of blood clots that can lead to a clot blocking an artery in
the lungs (pulmonary embolism) or cause a stroke
• Easy tiring when participating in activity or exercise
• Heart rhythm abnormalities (arrhythmias)
• Kidney or liver disease
 Truncus arteriosus
Truncus arteriosus is a congenital (present at birth) defect that occurs
due to abnormal development of the fetal heart during the first 8 weeks of
pregnancy. The heart begins as a hollow tube, and the chambers, valves, and
great arteries develop early in pregnancy. The aorta and pulmonary artery
start as a single blood vessel, which eventually divides and becomes two
separate arteries. Truncus arteriosus occurs when the single great vessel fails
to separate completely, leaving a connection between the aorta and
pulmonary artery.
Another congenital heart defect that nearly always occurs with truncus
arteriosus is a ventricular septal defect (a hole in the dividing wall (septum)
between the two lower chambers of the heart known as the right and left
ventricles).
 The blood that passes through the common truncus has a lower
oxygen content than normal. Oxygen-poor (blue) blood from the right
ventricle and oxygen-rich (red) blood from the left ventricle mix together
before entering the common vessel. Some of this mixed blood will go into
the aorta and on to the body, producing some degree of cyanosis (blue color
of the skin, lips, and nailbeds).

The pulmonary artery section of the common vessel gets more blood
flow than the aorta does because the pressure is lower in the lungs than
in the body and it is easier for blood to travel in that direction. If not
repaired, the blood vessels in the lungs will become damaged by long-term
exposure to the extra blood flow. As the pressure in the blood vessels in the
lungs becomes higher, less blood goes to the lungs and more goes to the
body. Cyanosis can worsen as blood with lower amounts of oxygen travels to
the body.
The following are the most common symptoms of truncus arteriosus.
However, each child may experience symptoms differently. Symptoms may
include:
• Cyanosis
• Fatigue
• Sweating
• Pale skin
• Cool skin
• Rapid breathing
• Heavy breathing
• Rapid heart rate
• Congested breathing
• Disinterest in feeding, or tiring while feeding
• Poor weight gain
 Total Anomalous Pulmonary Venous Return
Total anomalous pulmonary venous return (TAPVR) is a rare congenital
malformation in which all four pulmonary veins do not connect
normally to the left atrium. Instead the four pulmonary veins drain
abnormally to the right atrium (right upper chamber) by way of an
abnormal (anomalous) connection.
Babies with this heart defect can't supply oxygen-rich (red) blood to the
body after birth. Without an additional heart defect that allows mixing
of oxygen-poor (blue) and oxygen-rich (red) blood, such as an atrial
septal defect, infants with TAPVR will have a mixture of oxygen-rich
(red) and oxygen-poor (blue) blood circulating through the right heart
and back to the lungs — a situation that is fatal.