Pulmonary Atresia
Before you begin reading about this heart defect, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
- What is pulmonary atresia?
- What are the symptoms?
- How is it diagnosed?
- What are the treatment options?
- What kind of follow-up care is required?
What is pulmonary atresia?
When the heart squeezes, the right ventricle (the lower right chamber) 
Tetralogy of Fallot with Pulmonary Atresia (TOF/PA)
Enlarge+contracts, 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 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:
- a smaller-than-normal right ventricle
- a defective tricuspid valve (between the upper and lower chambers on the right side of the heart)
What are the symptoms?
The symptoms include:
- blue or purple tint to lips, skin and nails (cyanosis)
- shortness of breath
- difficulty feeding
- heart murmur - the heart sounds abnormal when a doctor listens with a stethoscope
How is it diagnosed?
Pulmonary atresia may be diagnosed before birth, with 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 may require some or all of these tests:
- echocardiogram (also called "echo" or ultrasound) - sound waves create an image of the heart
- electrocardiogram (ECG) - a record of the electrical activity of the heart
- chest X-ray
- cardiac catheterization - a thin tube (catheter) is inserted into the heart through the large veins in the leg
- pulse oximetry - a painless way to monitor the oxygen content of the blood
What are the treatment options?
Your baby will be admitted to our Tabas Cardiac Intensive Care Unit (CICU) and may require a ventilator for help breathing.
The exact treatment required 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 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. Surgeons usually open the pulmonary valve by patching across it, and often also place a shunt (a narrow tube) to be sure there is enough blood flow to the lungs. Another operation or cardiac catheterization may be required when the baby is older.
What kind of follow-up care is required?
Through 18
Children who have had surgical repair of pulmonary atresia require life-long care by a cardiologist. Some receive medicines.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physicians.
Into adulthood
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 with heart conditions 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:
Jonathan J. Rome, MD
Gil Wernovsky, MD
Date: October 2008
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