• The tricuspid valve is found between the upper and lower chambers on the right side of the heart. Tricuspid atresia occurs when this valve is deformed, narrow, or missing.
• Babies born with tricuspid atresia are blue because they cannot get blood to the lungs to pick up oxygen.
• To get to the lungs, blood must cross an atrial septal defect (ASD), ventricular septal defect (VSD), or a patent ductus artery (PDA). (These conditions are described above.) This severely restricts blood flow to the lungs.
• Soon after birth, a medicine called prostaglandin E may be given. This medicine will help keep the patent ductus arteriosus open so that blood can continue to flow to the lungs. However, this will only work for a while. Surgery is needed.
• A series of shunts and surgeries may be necessary to correct this defect. The goal of this surgery is to allow blood from the body to flow into the lungs, and blood from the lungs to be pumped to the rest of the body.

Total anomalous pulmonary venous return (TAPVR) correction

• TAPVR occurs when the pulmonary veins bring oxygen-rich blood from the lungs back to the right side of the heart, instead of the left side of the heart, where this usually occurs in healthy people.
• This condition requires surgery to correct it. When the surgery is done will depend on how sick the baby is. The surgery may be done in the newborn period if the infant has severe symptoms. If it is not done right after birth, it is done in the first 6 months of the baby's life.
• TAPVR repair requires an open-heart surgery. The pulmonary veins are routed back to the left side of the heart, where they belong, and any abnormal connections are closed.
• If a PDA is present, it is tied off and divided.

Hypoplastic left heart repair

• This is a very severe heart defect that results from a severely underdeveloped left heart. If it is not treated, it causes death in most babies who are born with it. Operations to treat this defect are done at specialized medical centers. Usually surgery corrects this defect. A series of three heart operations is usually needed.
• The first operation is done in the first week of the baby's life. This is a complicated surgery where one blood vessel is created from the pulmonary artery and the aorta. This new vessel carries blood to the lungs and the rest of the body.
• The second operation is usually done when the baby is 4 to 6 months old.
• The third operation is done a year after the second operation.
• A heart transplant is another option for this condition. But finding a donor heart for an infant is very difficult. Infant heart transplants can be done only at a few medical centers.

Images

Ultrasound, normal fetus - heartbeat

Heart, front view

Ultrasound, normal fetus - heartbeat

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Review Date: 11/01/2010
Reviewed By: Shabir Bhimji, MD, PhD, Specializing in General Surgery, Cardiothoracic and Vascular Surgery, Midland, TX. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)