Tricuspid Atresia

Definition

Definition of Tricuspid Atresia
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the heart to be unable to properly oxygenate the rest of the blood in the body. Because of this, the body does not have enough oxygen to live, and steps must be taken to keep the child alive. Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to maintain blood flow. Also, since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD).

Symptoms

Symptoms of Tricuspid Atresia

1. Bluish color to the skin
2. Fast breathing
3. Fatigue
4. Poor growth
5. Shortness of breath

Causes

Causes of Tricuspid Atresia
Tricuspid atresia is an uncommon form of congenital heart disease that affects about 5 in every 100,000 live births. Twenty percent of patients with this condition will also have have other heart problems.

Normally, blood flows from the body into the right atrium, then through the tricuspid valve to the right ventricle and on to the lungs. If the tricuspid valve does not open, the blood cannot flow from the right atrium to the right ventricle. Blood ultimately cannot enter the lungs, where it must go to pick up oxygen (become oxygenated).

Diagnosis

Diagnosis of Tricuspid Atresia
This condition may be discovered during routine prenatal ultrasound imaging or when the baby is examined shortly after birth. Bluish skin is present from birth. A heart murmur is often present at birth and may increase in loudness over several months.

Tests may include the following:

1. ECG
2. Echocardiogram
3. Chest x-ray
4. Cardiac catheterization
5. MRI of the heart

Treatment

Treatment of Tricuspid Atresia
Once the diagnosis is made, the baby will be admitted to the neonatal intensive care unit (NICU). A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to keep the blood circulating to the lungs.

The condition always requires surgery. If the heart is unable to pump enough blood out to the lungs and rest of the body, the first surgery usually occurs within the first few days of life. In this procedure, an artificial shunt is inserted to keep blood flowing to the lungs. In some cases, this first surgery is not needed.

Afterwards, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will decide when the second stage of surgery should be done.

Stage II of the operation is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the upper half of the body directly to the pulmonary artery. The surgery is usually done when the child is between 4 - 6 months old.

Prognosis

Prognosis of Tricuspid Atresia
Usually surgery will improve the condition.

Prevention

Prevention of Tricuspid Atresia
There is no known way to prevent tricuspid atresia.