Pulmonary Atresia (Tetralogy of Fallot)

Pulmonary atresia affects the pulmonary valve, a part of the heart that controls how blood is pumped to the lungs.

Normally, blood flows through the pulmonary valve on its way from the heart to the lungs, where it receives oxygen before it’s pumped to the rest of the body.

But in pulmonary atresia, the pulmonary valve does not form at all. Blood can’t flow from the heart to the lungs, and the body can’t get the oxygen-rich blood it needs.

This condition occurs as part of a group of heart defects called tetralogy of Fallot, including a ventricular septal defect (VSD) and, often, an abnormal pulmonary artery.

As with all congenital heart defects, it’s important for a patient with pulmonary atresia to have lots of different specialists working together to guide their care. Connecticut Children’s Heart Center is known for this multidisciplinary approach, including experts in advanced cardiac imaging, cardiac catheterization and congenital cardiac surgery.

Would you like to schedule an appointment with Cardiology & Cardiac Surgery?

Schedule

What Are the Signs and Symptoms of Pulmonary Atresia?

Pulmonary atresia is usually detected before birth, or very soon after a child is born. Here are signs that a newborn may have pulmonary atresia.

  • Bluish or greyish lips, skin and nails
  • Heart murmur
  • Trouble breathing
  • Sweaty or clammy skin
  • Feeding problems
  • 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 Pulmonary Atresia?

Pulmonary atresia occurs before birth, when the fetal heart doesn’t develop properly during pregnancy.

In some babies, it may be linked to one of these risks.

  • Genetic condition like DiGeorge syndrome
  • Family history of congenital heart problems
  • Mother had an infection like rubella (German measles) during pregnancy
  • Mother used certain harmful medications during pregnancy

But pulmonary atresia also occurs in babies without any of the above risk factors, and there may not be any known cause.

How is Pulmonary Atresia Treated?

Pulmonary atresia must be addressed immediately when a child is born. The treatment plan depends on how severe the child’s case is, and if they have other health risks. Doctors may use medication and minimally invasive procedures at first, delaying surgery until the child is older and strong enough.

  • Medication to help a newborn’s blood flow to their lungs
  • Balloon atrial septostomy: Uses a long, thin tube (catheter) to place a balloon in a natural hole in between the upper chambers of the heart. This enlarges the hole and allows blood from both sides of the heart to mix, which results in more oxygen-rich blood going to the body
  • Stent placement: Uses a catheter to place a rigid tube called a stent into the ductus arteriosus, an opening between the aorta and pulmonary artery. The stent keeps the ductus open, allowing more blood to travel to the lungs
  • RFA (radio frequency ablation): Uses a catheter to create a hole between the right ventricle and the pulmonary artery
  • Shunting: Open-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 valve and artery replacement: Open-heart surgery to put in a donor pulmonary valve and artery
  • Staged reconstruction: Between birth and age 3, a series of surgeries or catheter interventions to rehabilitate the pulmonary artery

Patients born with pulmonary atresia need care that continues throughout their life, often including additional surgeries or catheter procedures to replace the donor valve and artery. 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.