Tricuspid Atresia

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with tricuspid atresia, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

The tricuspid valve is the valve that separates the right atrium (the upper chamber of the heart that receives deoxygenated blood from the body) and the right ventricle (the lower chamber that pumps that blood out to the lungs). When a valve either doesn’t form properly or narrows so much that there’s no longer any opening, it’s called “atresia.” So “tricuspid atresia” means that the tricuspid valve has no opening into the right ventricle.

Babies with tricuspid atresia have no opening in their tricuspid valve — and because there’s no blood moving through that valve into the right ventricle, the right ventricle is also small and underdeveloped (this is called “hypoplastic”). Instead of entering the right ventricle, blood moves across the atrial septum through an atrial septal defect (or “ASD,” a hole in the wall between the right and left atria — the top two chambers of the heart). This deoxygenated blood then mixes with the oxygen-rich blood from the lungs and is usually pumped out to the body through the aorta.

Children with tricuspid atresia always have either of these other heart defects:
  • a ventricular septal defect (“VSD,” an abnormal opening in the wall between the right and left ventricles)
  • the ductus arteriosus remaining open after birth in order for blood to reach the lungs. (The ductus arteriosus is a blood vessel present in all infants that usually closes shortly after birth and connects the aorta to the pulmonary artery — the large artery that usually receives blood from the right ventricle and carries it to the lungs.)

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

These days, tricuspid atresia is usually diagnosed by a fetal echocardiogram (“echo”) during pregnancy. When babies who haven’t been previously diagnosed are born with tricuspid atresia, they may get sick very rapidly with both:

  • hypoxia (low levels of oxygen in the blood, which makes the skin look bluish — this is called “cyanosis”)
  • poor blood flow to the body
 
How & When Is It Treated?

Because babies with tricuspid atresia have only one functional ventricle (the right ventricle is usually too small to pump effectively), the management strategy involves a series of procedures in three “stages,” like with most children with single ventricle physiology (a group of congenital heart defects in which there’s only one, rather than two, pumping chambers):

  • A “shunt operation” (the first stage) is performed in the first days after birth. During this procedure, our Nemours Cardiac Center heart surgeons place a small tube (called a “shunt”) between the aorta (or one of its branches) and the pulmonary artery. This helps increase blood flow to the lungs so that an appropriate amount of oxygen can be delivered to the body.
  • The superior cavopulmonary connection (the second stage) consists of either one of two types:
    • the hemi-Fontan procedure
    • the bidirectional Glenn procedure

This stage is usually performed between 4 and 6 months old (for more details, see “Stage 2” in Single Ventricle Physiology & Palliation. The shunt placed during the first stage operation is also closed at this time.

  • The Fontan procedure (the third and final stage) is performed between 16 and 36 months of age (for more details, see “Stage #3” in Single Ventricle Physiology & Palliation. At the end of this procedure, the blood flows from the heart and out to the body, it then travels to the lungs without first passing through the heart as it normally would. This way, a child with only one ventricle can effectively and efficiently deliver blood to both the lungs and the body.

Learn More About Heart Surgery »

 
How Will It Affect My Child Long Term?

Children who’ve had a Fontan procedure may be somewhat less able to exercise as much or as vigorously, on average, as children who haven’t had heart surgery. But some may be able to exercise normally with their peers.

Children may need additional procedures or operations to make sure the heart and its valves continue to function well as they grow. Most children with single ventricle physiology, including those with tricuspid atresia, will need at least one or two medications for the rest of their life.

Children with tricuspid atresia generally have better outcomes following the Fontan than those with other diagnoses resulting in a single ventricle. This may be because with tricuspid atresia, it’s the left ventricle that’s functionally normally — and the left ventricle is designed to pump into the higher pressures of the body.

If your child has tricuspid atresia, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

Make an Appointment

(800) 416-4441

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


International
Patients

+1 (302) 651-4993

Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »