Tricuspid Atresia

Tricuspid atresia is a congenital (present at birth) disorder in which there is a solid tissue between the right atrium and right ventricle instead of valves (tricuspid valve).

Due to this disorder, blood cannot flow properly to the lungs to pick up oxygen as normally it should, as a result, the lungs cannot give oxygen supply to the rest of the body giving the body a blue tinge (cyanosis).

Multiple surgeries are required to correct it, and the person may lead a normal life in adulthood.

Tricuspid atresia symptoms become evident soon after birth and can include:

• Blue tinge to the skin and lips.
• Difficulty in breathing.
• Tiring easily.
• Especially during feedings.
• Fatigue or weakness.
• Edema in lower extremities.
• Swelling of abdomen.
• Fluid retention and poor weight gain.

Tricuspid atresia is caused during fetal heat development. Some factors like hereditary factors or down syndrome may increase baby’s risk of having tricuspid atresia, but the exact cause is unknown. 

Under normally circumstances (without defect), blood from all parts of the body come to right atrium- through the tricuspid valve- goes to right ventricle- through semilunar valve- to the pulmonary artery.

This artery gives deoxygenated blood to the lungs to pick up oxygen and comes back via pulmonary veins to right atrium of the heart. The blood through bicuspid valve- goes to the left ventricle- through aortic valve- to the aorta.

This artery gives oxygenated blood to rest of the body. Now, due to the defect, the blood cannot pass through the tricuspid valve and cannot be supplied to the lungs.

The blood will not be able to get oxygen from lungs and will not be supplied to the rest of the body. The right ventricle often hypoplastic.

This defect is often accompanied with hole in the wall between the right atrium and left atrium (atrial septal defect) or enlarged foramen ovale, ductus arteriosus and ventricular septal defect.

Tricuspid atresia can be diagnosed before birth during routine ultrasound investigation. After birth the following diagnostic tools are used:

• Physical examination: the blue tinge of body, trouble in breathing, poor weight gain, edema of legs and listening to heart murmurs while auscultation with stethoscope leads to the suspicion of congenital heart defects.
• Echocardiogram: this is used to rule out other disorders of heart. Sound waves are used that bounce off the heart giving the image on the screen. This test reveals absence of tricuspid valve and smaller right ventricle.
• ECG (Electrocardiogram): It records the electrical activity of the heart.
• Pulse oximetry: This test measures the amount of oxygen in the blood.
• Chest X-ray: this help to show enlargement of heart chambers and blood flow to the lungs.
• Cardiac catheterization: In this test, a catheter is inserted into a blood vessel at the child’s groin and is moved up to the heart.

The treatment goal for tricuspid atresia is to ensure proper blood flow through the heart and into the lungs. Quite often, more than one surgical procedures are required. The following surgical procedures may be done:

• Atrial Septostomy- Using this procedure, the opening between the upper heart chambers is created or enlarged to allow more blood flow from right atrium to the left atrium.
• Shunting- creating a bypass between the main arteries allows adequate blood flow to the lungs. This bypass is created in the first 4-8 weeks of life.
• Pulmonary Artery Band Replacement- If excessive amount of blood is delivered to the lungs then a band is placed around the pulmonary artery to reduce the flow.
• Glenn Procedure- A procedure that makes way for fontan procedure. This is done when babies outgrow their first shunt.
• Fontan Procedure- This is a standard procedure in which the surgeon created a path for the oxygen-poor blood in a blood vessel that returns blood to the heart.

Some medications like prostaglandins are recommended to dilate the ductus arteriosus. Life-long follow-up care is required to monitor health conditions.

Hospital care is needed but certain home remedies can be used:

• Good Nutrition- high calorie foods should be avoided since more energy is required to be digested. Some nutritional supplements may be recommended.
• Preventive Antibiotics- taking antibiotics before dental or other procedures may help to reduce the risk of infective endocarditis.
• Helping Child to Stay Active- normal playing and activity along with ample rest and breaks helps the child to stay healthy.
• Good Child Care- all immunizations should be done routinely and vaccinations against flu, pneumonia and others.
• Regular follow-up Appointments- annual appointments are necessary with the child’s doctor to evaluate the condition. For adults with tricuspid atresia, regular follow-up appointments, taking preventive antibiotics and doing limited activities or choosing occupation according to the condition should be kept in mind.

In case of a woman with such defect who wants to become pregnant, talking to the doctor will be necessary.

Coping up with tricuspid atresia can be depressive and challenging. Here are some ways which can help to manage the situation well:

• Bonding between the family is a very important for such children and spending as much time as possible with the child.
• Keeping the child happy and relaxed is the key to better healing.
• Seeking support from friends and families is good way to know about the situation more closely.
• Taking record of all events from diagnosis to treatment including follow-up will be helpful.
• Taking to the child’s doctor about the risks and concerns of a parent is also very helpful.

The following factors may pose a risk for getting this disorder:

• A mother with TORCH infection (toxoplasma- rubella- cytomegalovirus- herpes).
• A parent with congenital heart defect.
• Drinking alcohol during pregnancy.
• Smoking during pregnancy.
• Poorly controlled diabetes in mother.
• Mother with autoimmune disorders.
• Use of certain medications during pregnancy and chromosomal defects in the baby.

Surgery generally, improves the condition of the baby but some complications may still appear, which include:

• formation of blood clots blocking important vessels (e.g.-pulmonary embolism),
• getting tired easily,
• arrhythmias (heart rhythm problems),
• certain complications in the lymphatic system.