With this serious but rare condition, the tricuspid valve has failed to develop, so blood cannot flow from the right atrium to the right ventricle. 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.
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.