In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle through the tricuspid valve. The blood travels to the lungs through the pulmonary valve to pick up fresh oxygen. Next, the blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body.
Tricuspid atresia is when the tricuspid valve (located on the right side of the heart) does not develop. Also, the right ventricle is abnormally small. When this occurs, blood cannot move from the right atrium to the right ventricle. The defect can be mild to severe. There may also be other defects, like holes in the walls of the heart chambers that allow blood to move back and forth.
This condition is a congenital defect. This means that the heart forms incorrectly while the baby is in the womb. It is not known exactly why some babies’ hearts develop this way.
Risk factors for congenital heart defects like tricuspid atresia may include:
Symptoms may include:
The doctor may also detect a heart murmur during the exam.
This condition can lead to heart failure. If your child has any of these symptoms, get medical care right away.
Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:
Talk with your doctor about the best treatment plan for your child. Often, surgery is needed right away with follow-up surgeries at ages 3-6 months and 2-5 years. Treatment options include:
Newborn babies are often given a medicine called prostaglandin. This medicine helps keep pathways open so blood can get to the lungs for oxygenation.
The goal of surgery is to:
Depending on the severity of the defect, there are several options. For example, a shunt may be placed to increase blood flow to the lungs. A Fontan procedure is another type of surgery. This involves a series of surgeries with a goal to reroute blood travel through the heart, lungs and body.
Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent a heart infection.
Preventing fetal heart defects may not always be possible, but you can reduce your risk by:
American Family Physician
American Heart Association
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
American Heart Association. How your cardiologist diagnoses heart defects. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=152 . Accessed July 6, 2010.
American Heart Association. Tricuspid atresia. American Heart Association website. Available at: http://americanheart.org/presenter.jhtml?identifier=1310 . Accessed July 6, 2010.
Cincinnati Children’s Hospital. Tricuspid Atresia. Cincinnati Children’s Hospital website. Available at: http://www.cincinn... . Accessed July 6, 2010.
DynaMed Editorial Team. Tricuspid atresia. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated February 11, 2010. Accessed July 6, 2010.
Mayo Clinic. Atrioventricular canal defect. Mayo Clinic website. Available at: http://www.mayocli... . Accessed July 7, 2010.
Last reviewed June 2012 by Kari Kassir, MD
Last Updated: 06/07/2012