Tricuspid atresia is a type of heart disease that is present at birth (
Causes, incidence, and risk factors
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).
- Bluish color to the skin
- Fast breathing
- Poor growth
- Shortness of breath
Signs and tests
This condition may be discovered during routine
Tests may include the following:
- Chest x-ray
- Cardiac catheterization
- MRI of the heart
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 circulatingto 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 tokeep 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.
During stage I and II, the child may stilllook blue (cyanotic).
Stage III, the final step, is called the Fontan procedure. The rest of the veins carrying blue blood from the bodyare connected directly to the pulmonary artery leading to the lungs. The left ventricle now only has to pump to the body, not the lungs. This surgery is usually performed when the child is 18 monthsto 3 years old. After this final step, the baby is no longer blue.
Usually surgery will improve the condition.
- Irregular, fast heart rhythms (arrhythmias)
- Chronic diarrhea (from a disease called protein loosing enteropathy)
- Heart failure
- Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
- Blockage of the artificial shunt
- Strokes and othernervous systemcomplications
- Sudden death
Calling your health care provider
Contact your health care provider right away if your infant has:
- New changes in breathing patterns
- Problems eating
- Skin that is turning blue
There is no known way to prevent tricuspid atresia.
Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In:Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia, Pa:Saunders Elsevier; 2011:chap 65.