Treatment for tricuspid atresia

If your baby is born with tricuspid atresia, they will be admitted to the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), where they will receive round-the-clock attention from a team of dedicated cardiac critical care medicine specialists. Your baby may require oxygen and a medication called prostaglandin, to keep enough oxygen in their blood. 

Prostaglandin is an IV (intravenous) medication that keeps the connection between the pulmonary artery and the aorta open. This connection, called the patent ductus arteriosus (PDA), is open in the fetus, and closes soon after birth. When the PDA closes, some babies with tricuspid atresia turn blue. An infusion of prostaglandin is a lifesaving intervention that can re-open the PDA. Not all babies with tricuspid atresia require prostaglandin.

It is not unusual for babies to have trouble breathing or stop breathing for short periods (apnea) while on prostaglandin infusion. If your baby struggles to breathe, they may need to be placed on a breathing machine or ventilator. 

To repair many types of single ventricle defects, including tricuspid atresia, surgeons perform a series of open-heart procedures over a period of years. This is called “staged reconstruction.”

Blalock-Taussig-Thomas shunt or Ductal Stent

Babies who need prostaglandin to keep their oxygen levels stable will need surgery shortly after birth. The first surgery involves placing a “shunt,” which is a tube that connects a branch of the aorta to the pulmonary artery, taking over the job of the PDA. This surgery is called the Blalock-Taussig-Thomas (or BTT) shunt. 

Many babies with tricuspid atresia are healthy enough to go home soon after birth. However, some may need the BTT shunt a few weeks later if their oxygen levels drop.

Instead of having a surgical stunt, some babies may have a ductal stunt placed. This means putting a small tube (stent) into the ductus arteriosus, the blood vessel that connects the pulmonary artery to the aorta. The vessel is open in the fetus but closes after birth. The stent keeps it open, allowing more blood to flow to the lungs. 

Some babies with tricuspid atresia are too “pink” (meaning their oxygen levels are too high because of too much blood flow to the lungs). These babies need an operation called “pulmonary artery banding” to narrow the pulmonary artery and control blood flow to the lungs. 

Babies who have tricuspid atresia and transposition of the great arteries may need the Norwood procedure.

Glenn

The second operation, called the Glenn operation, usually happens within six months of a child’s birth. During this surgery, the superior vena cava — the large vein attached to the heart that returns deoxygenated blood from the upper half of the body — is closed off or disconnected from the heart and instead attached to the pulmonary artery. The surgeon will also close the BTT shunt or remove the ductal stent. After the Glenn, deoxygenated blood from the upper body goes directly to the lungs without passing through the heart.

Fontan

The third operation, called the Fontan, usually happens when a child is 18 months to 3 years old. During this surgery, blood from the inferior vena cava — the large vein that returns deoxygenated blood to the heart from the lower half of the body — is diverted directly to the pulmonary artery. This means that deoxygenated blood from the whole body now goes to the lungs without passing through the heart. This is called Fontan circulation.

Your Cardiac Center team will explain staged reconstruction to you in greater detail based on your child’s individual anatomy.

Outlook for tricuspid atresia

Because of enormous strides in medicine and technology, today many children born with tricuspid atresia go on to lead healthy, productive lives as adults.

Follow-up care

Through age 18

Though early outcomes for children with single ventricle heart defects after staged reconstruction have greatly improved over the years, the time between the Norwood procedure and the Glenn operation is a very vulnerable time for infants. Our Infant Single Ventricle Monitoring and Management Program provides care and monitoring for babies with single ventricle heart defects between two necessary heart repair procedures. 

Children with tricuspid atresia require life-long care by a cardiologist. Many remain on medications for life. Additional surgeries may be required.

In addition, children with complex congenital heart defects who have had open heart surgery as infants are at a higher risk for neurodevelopmental issues when compared to children without congenital heart defects. CHOP’s Cardiac Kids Development Follow-up Program provides evaluation, screening and clinical care for children with complex congenital heart disease who are at risk for neurodevelopmental problems. 

 As patients with Fontan circulation get older, doctors are recognizing that, while some do fine, many experience complications. These complications can include lung, liver and gastrointestinal diseases. Our Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program specializes in the care of these patients and is researching improved treatments for them. 

Into adulthood

Our pediatric cardiologists follow patients until they are young adults. The Philadelphia Adult Congenital Heart Center, a joint program of CHOP and Penn Medicine, meets the unique needs of adults who were born with heart defects. At CHOP’s Cardiac Center, we support our adolescent and young adult patients through a smooth and seamless transition to adult cardiology care at the Philadelphia Adult Congenital Heart Center or, if desired, to a cardiologist with congenital heart disease expertise in another location.