Are you familiar with Tricuspid Atresia?

Question

My 2 year old son has tricuspid atresia and I just wanted to know like how well is your child developing, what obstacles do you have to go through, and can he/she still play like other children.

Answer 1

Tricuspid Atresia

  

(It will be helpful to review normal heart function before reading this.)

In this condition, there's no tricuspid valve so no blood can flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. The child's survival depends on there being an opening in the wall between the atria (atrial septal defect) and usually an opening in the wall between the two ventricles (ventricular septal defect). As a result, the venous (bluish) blood that returns to the right atrium flows through the atrial septal defect and into the left atrium. There it mixes with oxygen-rich (red) blood from the lungs. Most of this poorly oxygenated mixture goes from the left ventricle into the aorta and on to the body. The rest flows through the ventricular septal defect into the small right ventricle, through the pulmonary artery and back to the lungs. Because of this abnormal circulation, the child looks blue.

Often in these children it's necessary to do a surgical shunting procedure to increase blood flow to the lungs. This reduces the cyanosis. Some children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery banding) to decrease blood flow to the lungs.

Other children with tricuspid atresia may have a more functional repair (Fontan procedure). In this, a connection is created between the right atrium and pulmonary artery. The atrial defect is also closed. This eliminates the cyanosis but, without a right ventricle that works normally, the heart can't work totally as it should.

Children with tricuspid atresia require lifelong follow-up by a cardiologist for repeated checks of how their heart is working. They also risk infection in the heart's valves (endocarditis) before and after treatment. Please see our page on endocarditis to determine whether or not your child will need to take antibiotics before certain dental procedures to help prevent endocarditis. Good dental hygiene also lowers the risk of endocarditis. For more information about dental hygiene and preventing endocarditis, ask your pediatric cardiologist.

Answer 2

Hi! I have a two year old son also with tricuspid atresia. He is getting ready to have a Fontan surgery in a couple of weeks that I am extremely anxious about.

So far his development has been great - he is happy and able to do everything a normal two year old does. He does get winded when walking up stairs and I know his exercise capacity is not perfect.  When checked his oxygen saturations are usually around 84%. I hope the Fontan increases them.

For his future, right now I am so worried about his upcoming surgery. After that, I don't know what to expect for him and that is the hardest part.  The doctors have said a Fontan circulation is his best chance for a near normal life, so that is all I can hope for.  The other thing I hope for is the rapidly advancing cardiac research and development for more solutions for adults with these kinds of congenital heart defects.

Best of luck of for you and your little guy! When my son was born and in the hospital so much, it seems like many people were telling us they knew of older kids and teenagers with the same defects that were happy and that always gave us so much hope.