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Alternative Names Return to topTri atresia
Definition Return to top
Tricuspid atresia is a type of congenital heart disease in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle.
Causes Return to top
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 into the right atrium, then through the tricuspid valve to the right ventricle and on to the lungs. If the tricuspid valve is missing or broken, the blood cannot flow from the right atrium to the left ventricle. Blood ultimately cannot enter the lungs, where it must go to pick up oxygen (become oxygenated).
Infants with tricuspid atresia generally are cyanotic (bluish discoloration of skin) and easily become short of breath.
Symptoms Return to top
Exams and Tests Return to top
This condition may be discovered during routine prenatal ultrasound imaging or when the baby is examined shortly after birth. Cyanosis may be present from birth. A heart murmur is generally present at birth and may increase in loudness over several months.
Tests may include the following:
Treatment Return to top
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 maintain circulation of blood to the body.
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 maintain blood flow to the lungs.
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 determine 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 body directly to blood vessels to the lungs that get oxygen. The surgery is usually done when the child is between 4 - 6 months of age.
During stage I and II, the child may still appear somewhat blue (cyanotic).
Stage III, the final step, is called the Fontan procedure. The remainder of the veins carrying blue blood from the body is connected directly to blood vessels leading to the lungs, and the right ventricle now serves as the only pumping chamber for the heart. This surgery is usually performed between the ages of 18 months and 3 years of age. After this final step, the baby is no longer blue (cyanotic).
Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure.
In some hospitals, heart transplantation is considered to be a better alternative to the 3-step surgery process, but there are few donated hearts available for small infants.
Outlook (Prognosis) Return to top
Improvement can be expected with most surgical procedures. Unexpected death may occur with heart arrhythmias.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
If your infant has new changes in breathing patterns, eats less (decreased feeing), or has skin or mucous membranes that are turning blue (cyanotic), contact your health care provider immediately.
Prevention Return to top
There is no known way to prevent tricuspid atresia.
References Return to top
Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.Update Date: 12/10/2007 Updated by: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.