Before you begin reading about pulmonary atresia, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
When the heart squeezes, the right ventricle (the lower right pumping chamber)
View Large Tetralogy
of Fallot with
Pulmonary Atresia (TOF/PA)Illustrationcontracts, pushing the blood out to the lungs. The pulmonary valve sits on the way out of the heart to the lungs, to prevent blood from leaking back into the heart between beats. In pulmonary atresia, the pulmonary valve has not formed correctly, is sealed and cannot open.
Many children with pulmonary atresia also have a ventricular septal defect, a hole in the tissue between the lower chambers of the heart. This combination is often called tetralogy of Fallot with pulmonary atresia.
When there is not a ventricular septal defect, children with pulmonary atresia may also have:
Pulmonary atresia symptoms include:
Pulmonary atresia may be diagnosed before birth, with a fetal echocardiogram (ultrasound). Our Fetal Heart Program will prepare a plan for delivery and care immediately after birth.
Sometimes pulmonary atresia is diagnosed a few hours or days after birth. Pediatricians from other hospitals refer newborns to the Cardiac Center when they notice symptoms.
Diagnosis for pulmonary atresia may require some or all of these tests:
Your baby will be admitted to our Tabas Cardiac Intensive Care Unit (CICU) and may require a ventilator for help breathing.
The exact treatment for pulmonary atresia depends on each child's heart anatomy. Many cases can be treated with a cardiac catheterization technique called balloon valvuloplasty. Doctors advance a thin tube (catheter) to the heart through a vein in the leg. A special catheter is used to create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is advanced across the valve. The balloon is briefly inflated to open up the valve, and then deflated and withdrawn.
In other cases, open heart surgery is required to repair pulmonary atresia. Surgeons may open the pulmonary valve by patching across it, or they may place a shunt (a narrow tube) from the aorta to the pulmonary artery (vessel to the lung) to be sure there is enough blood flow to the lungs. Another operation or cardiac catheterization may be required when the baby is older.
In some cases of pulmonary atresia, the right ventricle is poorly developed and cannot function adequately as a pump. In such cases, a “single ventricle” strategy may be necessary.
In children with pulmonary atresia and ventricular septal defect (VSD), surgery will be necessary to close the VSD. Sometimes the pulmonary arteries are very small. In this case, multiple surgeries and cardiac catheterization may be needed.
Children who have had surgical repair of pulmonary atresia require life-long care by a cardiologist. Some receive medicines.
As “single ventricle survivors” get older, doctors are recognizing that, while some do fine, many experience complications, including lung, liver and gastrointestinal complications. The Cardiac Center at CHOP created the Single Ventricle Survivorship Program to bring together doctors from different specialties to care for single ventricle survivors.
In addition, as a group, 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. The Cardiac Center at CHOP created the NeuroCardiac Care Program (NCCP) to provide evaluation, screening and clinical care for children with complex congenital heart disease who are at risk for neurodevelopmental problems.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physicians.
We will help older patients with the transition to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Because of enormous strides in medicine and technology, today most children born with pulmonary atresia go on to lead healthy, productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Jack Rychik, MD
Date: November 2013
For a second opinion, cardiac referral, or for more information.
To schedule an outpatient appointment.