Pulmonary Atresia With Intact Ventricular Septum (PA/IVS)

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with pulmonary atresia with intact ventricular septum (or “PA/IVS”), we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

With this relatively rare abnormality (seen in 1 to 1.5 percent of children with congenital heart disease, or about 1 in 10,000), the blood vessel going to the lungs, and the ventricle underneath it, don’t develop properly, which results in multiple problems of varying severity. Depending on the size of the right ventricle (the ventricle that pumps through the pulmonary artery) and the associated tricuspid valve (the valve that connects the atrium to the right ventricle), children can have a right ventricle large enough that it can support pumping blood to the lungs, or it can be so small that it will never be able to perform this job.

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

Pulmonary atresia with intact ventricular septum (PA/IVS) is detected at birth or within the first few days after birth. It’s also often diagnosed prenatally (during pregnancy).

 
How & When Is It Treated?

Babies will need some sort of procedure before leaving the hospital. In the milder forms, it may be treatable with interventional catheterization rather than open-heart surgery. (In a catheterization, a Nemours Cardiac Center cardiac catheterization specialist inserts a thin plastic, flexible tube, called a “catheter,” into an artery and vein that lead to the heart.) But the more severe forms require multiple surgeries or, sometimes, a heart transplant.

In cases where there isn’t enough of the right ventricle to pump blood to the lungs, the primary decision is between a:

At the Nemours Cardiac Center, our heart surgeons decide which option is better to treat pulmonary atresia with intact ventricular septum (PA/IVS) based on the exact characteristics in each individual child such as:

  • the size and function of the tricuspid valve and right ventricle
  • how the coronary blood flow interacts with the small right ventricle
  • whether the connections between the coronary arteries (which feed the heart its blood) and the right ventricle will result in problems delivering blood and oxygen to the heart muscle itself

Learn More
 
How Will It Affect My Child Long Term?

The long-term consequences of pulmonary atresia with intact ventricular septum (PA/IVS) vary greatly depending on the severity of the condition. Some children may not need additional procedures after treatment as a newborn. Most will require procedures (either cardiac catheterizations or surgeries) to maintain unobstructed blood flow to the lungs.

Children with the most severe form will need at least three operations (often more) in the first few years of life. The Nemours Cardiac Center surgeons and care team will be there for your child, from start to finish, answering your questions, addressing your concerns, and offering top-quality medical and surgical care.

If your child has pulmonary atresia and intact ventricular septum, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

Make an Appointment

(800) 416-4441

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


International
Patients

+1 (302) 651-4993

Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »