With this serious but rare condition, no blood can flow from the right atrium to the right ventricle, because the tricuspid
valve has failed to develop. With this defect, the right ventricle also does not form fully and is too small. As a result
of the defect, blood passes through a hole in the atrial septum from the right atrium to the left, and mixes with blood coming
back from the lungs.
Tricuspid atresia is rare, making up only about 3% of heart defects. In about 20% of cases it is associated with other cardiac
abnormalities.
| Tricuspid Atresia |
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| With this condition, no blood can flow from the right atrium to the right ventricle, because the tricuspid valve has failed
to develop. An atrial septal defect and ventricular septal defect (VSD) are usually present, and the VSD enables blood to
reach the pulmonary artery and lungs.
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What are the symptoms of tricuspid atresia?
Children with tricuspid atresia are usually cyanotic at birth or shortly after, and have congestive heart failure. The diagnosis
will be made by way of a chest X-ray, electrocardiogram, or echocardiogram.
How is tricuspid atresia treated?
Surgery is usually needed early in life. A shunt is placed between the aorta and pulmonary artery to provide blood flow to
the lungs. Months later, a Glenn procedure is done to attach the superior vena cava to the pulmonary arteries to provide more
blood flow to the lungs, and the shunt is removed. When the child is about two or three, a Fontan procedure is done.
What is the long-term outlook for children with tricuspid atresia?
For children with this condition, outcomes are quite good through the staged repair, with survival at 75% to 95%. In some
cases, arrhythmias can develop later in life, as can lung problems.
