Learn more about this life-threatening congenital heart condition that changes the way blood travels from the heart to the rest of the body.
Pulmonary atresia (uh-TREE-zhuh) is a heart defect present at birth (congenital) that's usually diagnosed soon after birth. In pulmonary atresia, the valve that lets blood out of the heart to go to the lungs (pulmonary valve) doesn't form correctly.
Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So, blood can't travel its usual route to pick up oxygen from the lungs. Instead, some blood travels to the lungs through other natural passages within the heart and its arteries.
These passages are necessary when a baby is developing in the womb, and they typically close soon after birth. Babies with pulmonary atresia typically have a bluish cast to their skin because they aren't getting enough oxygen.
Pulmonary atresia is a life-threatening situation. Procedures to correct a baby's heart condition and medications to help a baby's heart work more effectively are the first steps to treat pulmonary atresia.
In pulmonary atresia, the valve that lets blood flow from the heart to the lungs isn't formed properly. Instead, some blood may enter the lungs through a temporary connection (ductus arteriosus) between a baby's aorta and the pulmonary artery. Some babies born with pulmonary atresia may have a small right ventricle.
If a baby is born with pulmonary atresia, symptoms will be noticeable soon after birth. Signs and symptoms might include:
A baby with pulmonary atresia will most likely be diagnosed soon after birth. However, if your baby has symptoms of pulmonary atresia after you've returned home, seek emergency medical attention.
There's no known cause of pulmonary atresia. To understand how pulmonary atresia occurs, it may be helpful to know how the heart works.
The heart is divided into four hollow chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — the heart uses its left and right sides for different tasks.
The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart's left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body to supply the body with oxygen.
Blood moves through the heart in one direction through valves that open and close as the heart beats. The valve that allows blood out of the heart and into the lungs to pick up oxygen is called the pulmonary valve.
In pulmonary atresia, the pulmonary valve doesn't develop properly, preventing it from opening. Blood can't flow from the right ventricle to the lungs.
Before birth, the irregular valve isn't life-threatening, because the placenta provides oxygen for the baby instead of the lungs. Blood entering the right side of the baby's heart passes through a hole (foramen ovale) between the top chambers of the baby's heart, so the oxygen-rich blood can be pumped out to the rest of the baby's body through the aorta.
After birth, the lungs are supposed to provide oxygen to the body. In pulmonary atresia, without a working pulmonary valve, blood must find another route to reach the baby's lungs. The foramen ovale usually shuts soon after birth, but it may stay open in pulmonary atresia.
Newborn babies also have a temporary connection (ductus arteriosus) between the aorta and the pulmonary artery. This passage allows some of the oxygen-poor blood to travel to the lungs, where it can pick up oxygen to supply the baby's body. The ductus arteriosus typically closes soon after birth, but it can be kept open with medications.
Sometimes, there may be a second hole in the tissue that separates the main pumping chambers of the baby's heart. This hole is a ventricular septal defect (VSD).
The VSD allows a pathway for blood to pass through the right ventricle into the left ventricle. Children with pulmonary atresia and a VSD often have additional problems with the lungs and the arteries that bring blood to the lungs.
If there's no VSD, the right ventricle receives little blood flow before birth and often doesn't develop fully. This is a condition called pulmonary atresia with intact ventricular septum (PA/IVS).
A typical heart has two upper and two lower chambers. The upper chambers — the right and left atria — receive incoming blood. The lower chambers — the right and left ventricles — pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings (for the tricuspid and mitral valves) and exits (for the pulmonary and aortic valves).
The exact cause of a congenital heart defect, such as pulmonary atresia, is usually unknown. However, several things may increase a baby's risk of a congenital heart defect, including:
Without treatment, pulmonary atresia usually leads to death. Regular health checkups are needed after pulmonary atresia surgery to carefully monitor for complications.
Potential complications of pulmonary atresia and other structural heart problems include:
Because the exact cause of pulmonary atresia is unknown, it may not be possible to prevent it. However, some things can be done before or during pregnancy to help reduce a baby's overall risk of congenital heart defects, such as:
Tests to diagnose pulmonary atresia may include:
A baby will need urgent medical attention once pulmonary atresia symptoms develop. The choice of surgeries or procedures depends on the severity of the child's condition.
Medication may be given through an IV to help prevent the closure of the natural connection (ductus arteriosus) between the pulmonary artery and the aorta. This is not a permanent treatment for pulmonary atresia, but it gives health care providers more time to determine what type of surgery or procedure might be best for the child.
Sometimes, pulmonary atresia repairs can be done using a long, thin tube (catheter) inserted into a large vein in a baby's groin and threaded up to the heart. Catheter-based procedures for pulmonary atresia include:
Babies with pulmonary atresia often require a series of heart surgeries over time. The type of heart surgery needed will depend on the size of the child's right ventricle and pulmonary artery. Some examples include:
Here are some tips for caring for your child after coming home from the hospital:
Help your child stay active. Encourage as much play and activity as your child is able to tolerate, with plenty of time for rest and nap time. Staying active helps a child's heart stay fit.
As your child grows, talk with a heart doctor (cardiologist) about which activities are best for your child. If some are off-limits, such as competitive sports, encourage your child in other pursuits rather than focusing on what can't be done.
Talking with other parents who've been through a similar situation may bring comfort and encouragement. Ask your child's health care provider about local support groups.
It's most likely that your child would be diagnosed with pulmonary atresia soon after birth while still in the hospital. If your child is diagnosed with pulmonary atresia, you'll be referred to a heart specialist (cardiologist) for ongoing care.
Because appointments can be brief, and because there's often a lot to discuss, it's a good idea to be prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your health care provider.
For pulmonary atresia, some basic questions to ask your child's health care provider include:
Don't hesitate to ask other questions.
Your child's health care provider might ask: