Pulmonary Hypertension

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

1600 Rockland Road, Wilmington, DE 19803 | Get Map & Directions »

Pediatric pulmonary hypertension can’t be prevented, but a lot can be done to treat it and improve heart function and a child’s quality of life. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), children with pulmonary hypertension can receive the specialized care they need to have the very best possible chance for a healthy future.

When your child has a heart condition, we want to help you learn as much as you can about the problem. Understanding what’s going on inside your child’s heart can make things a little less stressful and scary for you, your child and your whole family. Plus, the more you know, the more you can be an advocate throughout your child’s care.

What Is It?

Idiopathic pulmonary arterial hypertension (or “IPAH,” for short) is a rare childhood disease (occurring in less than 1 in 100,000) in which the blood vessels in the lung become narrowed more and more over time. This forces the right ventricle (the heart chamber that pumps blood to the lungs) to work much harder than it was designed to — so the right ventricle will commonly fail early in life without aggressive medical treatment.

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?
Pediatric pulmonary hypertension can cause:
  • breathing difficulties
  • chest pain
  • fatigue
  • loss of consciousness

Because these symptoms are relatively vague and not specific to pulmonary hypertension, many children aren’t diagnosed for years.

Over time, pulmonary hypertension can lead to:
  • right-sided heart failure
  • blood clots
  • irregular heartbeats (“arrhythmia”)
  • bleeding in the lungs

Sometimes, pediatric pulmonary hypertension is caused by another medical problem such as lung disease, a heart defect, autoimmune diseases or blood clotting disorders. In these cases, your child’s Nemours Cardiac Center cardiologist will work with other specialists to treat the additional problem(s) in order to help improve the pulmonary hypertension and your child’s quality of life.

How & When Is It Treated?

At the Nemours Cardiac Center, our specialists typically treat pediatric pulmonary hypertension with medications that help improve blood flow in the pulmonary arteries. Depending on the medication, the child may receive treatment orally, via inhalation or by continuous infusions through a surgically placed IV. Each child is different — your doctor will create an individualized treatment plan specifically designed for your child.

Although there’s no cure for pediatric pulmonary hypertension, medications have significantly improved the quality and duration of life for children with this disease. But many children must go on to have lung or heart-lung transplants.

If your child has pediatric pulmonary hypertension, 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 disease journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More

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

Insurance We Accept »

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


+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 »