In this rare disorder, the tricuspid valve – which is supposed to connect the heart’s right chambers – does not form in a baby during pregnancy.

Normally, blood flows from the heart’s upper right chamber, through the tricuspid valve, and into the heart’s lower right chamber. Then it’s pumped to the lungs, where it picks up oxygen. But in babies with tricuspid atresia, the tricuspid valve hasn’t formed. Blood can’t flow through the right side of the heart the way it’s supposed to, and the body doesn’t get the oxygen-rich blood it needs.

Usually, children with tricuspid atresia are also born with an atrial septal defect (ASD) and a ventricular septal defect (VSD), holes in the wall between the left and right sides of the heart. Sometimes they also have other heart problems like transposition of the great arteries (TGA) or patent ductus arteriosus (PDA).

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What Are the Signs and Symptoms of Tricuspid Atresia?

Because this condition is so serious, it’s usually detected during pregnancy or within the first few days after a baby is born.

Here are signs that a newborn may have tricuspid atresia.

  • Bluish or grayish lips, skin and nails
  • Heart murmur
  • Trouble breathing
  • Trouble feeding
  • Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What Causes Tricuspid Atresia?

Tricuspid atresia occurs before birth, when the fetal heart doesn’t develop properly during the first eight weeks of pregnancy. Doctors don’t know exactly why this happens.­­

While tricuspid atresia can occur just by chance, it’s a bit more common in babies with:

  • Genetic disorders like Down syndrome
  • Family history of congenital heart problems
  • Mother infected with rubella (German measles) during pregnancy
  • Mother with poorly controlled diabetes or lupus (an autoimmune disease)
  • Mother who used certain anti-acne or anti-seizure medicines during pregnancy

How is Tricuspid Atresia Treated?

Babies born with tricuspid atresia usually need “staged reconstruction,” a series of up to three surgeries to redirect how blood flows through the heart and lungs. These surgeries are spaced out over the first years of their life.

Connecticut Children’s cardiac surgeons specialize in these complex surgeries, with outstanding outcomes. Our cardiac catheterization experts play an important role before and between surgeries, using minimally invasive techniques to help the baby’s heart stay as healthy as possible.

  • Medication to open the ductus arteriosus, the blood vessel connecting the heart’s two major arteries, improving blood flow to the lungs
  • Medication to strengthen the heart muscle, lower blood pressure, and get rid of extra fluid
  • Supplemental oxygen or a machine to help the baby breathe
  • Balloon atrial septostomy: Uses a long, thin tube (catheter) to make or enlarge a hole between the upper chambers of the heart. This allows blood from both sides of the heart to mix, which results in more oxygen-rich blood going to the body
  • Cardiac catheterization: Uses a catheter to place a mesh insert in the ductus arteriosus to keep it open
  • Blalock-Taussig (BT) shunt: Closed-heart surgery to place a tube, or shunt, between the aorta and pulmonary artery, which allows more blood to flow to the lungs. Babies usually outgrow this within a few years
  • Pulmonary artery band placement: If blood flow to the lungs is too high, places a band around the pulmonary artery to lessen blood flow
  • Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
  • Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen

Patients born with tricuspid atresia need lifelong care from congenital heart experts who understand their heart’s unique anatomy. Connecticut Children’s Heart Center is there every step of the way, with care that transitions into adulthood with our nationally accredited Adult Congenital Heart Disease program.