Children's Hospital Colorado

Pulmonary Hypertension

What is pediatric pulmonary hypertension?

Pediatric pulmonary hypertension (PH) is a rare disorder of high blood pressure in the blood vessels of the lungs. Pulmonary blood pressure rises when arteries and capillaries in the lungs become narrowed, blocked or destroyed, making it harder for blood to flow through the lungs. Because the vessels in the lungs and heart are connected, this makes blood pressure in the heart rise and forces the heart to work harder than normal.

If the condition goes untreated, the heart has difficulty pushing against the pressure from the lungs, which may eventually lead to heart failure.

What causes pediatric PH?

In some cases, there is no underlying cause of the high blood pressure in the lungs. This type of PH is called idiopathic pulmonary arterial hypertension (IPAH). There are also genetic forms of PH, called heritable pulmonary arterial hypertension (HPAH). In some cases, PH is the result of other conditions, which is known as associated pulmonary hypertension.

Causes of associated pulmonary hypertension include:

Some forms of PH will improve or resolve with proper treatment. Most forms of PH require lifelong treatment.

What are the signs and symptoms of pulmonary hypertension?

Children with pulmonary hypertension (PH) can experience shortness of breath and fatigue, especially after activity. Signs of PH can be confused with other conditions like asthma, sometimes leading to a delay in diagnosis and treatment.

Other pediatric pulmonary hypertension symptoms include:

  • Chest pain
  • Exercise intolerance
  • Shortness of breath
  • Dizziness
  • Fainting
  • Blue tint to the skin, also called cyanosis
  • Swelling of the feet and ankles
  • Recurrent nausea
  • Poor growth
  • Recurrent respiratory infections

What tests are used to diagnose pulmonary hypertension?

If your doctor suspects that your child has pulmonary hypertension (PH), they will likely order more tests to confirm the diagnosis. Common tests include:

How is pulmonary hypertension treated?

There are many treatment options for kids and young adults with pulmonary hypertension (PH). If the PH is associated with another condition, it is important to first treat the underlying cause such as repair of congenital heart defects or treatment of lung disease.

PH is a disease that can progress over time, especially if not properly diagnosed, monitored and treated. Symptoms and severity of the disease vary among children with PH.

The goal of treatment is to decrease the high blood pressure in the lungs, by relaxing the blood vessels. This improves symptoms and quality of life. Treatment for PH may include oxygen or medicines that are taken by mouth, inhaled or injected.

The Breathing Institute participates in various clinical trials. Please reach out to our program to get more information.

Living with pediatric PH

There is currently no cure for many forms of PH. However, having a specialist with experience in treating PH monitor your child's pulmonary pressures and response to medications can help greatly improve their quality of life.

Why choose us for treatment of pulmonary hypertension?

The Pulmonary Hypertension Program at Children's Colorado has been treating children with PH since 1995. We provide comprehensive care for your child, with a multidisciplinary team approach. Our multidisciplinary team includes leading experts in cardiology and pulmonology, along with nurse practitioners, nurses, respiratory therapists, child life therapists, palliative care and psychologists. We currently manage more than 200 patients with severe pulmonary hypertension. Our program offers state-of-the-art diagnosis, consultation and long-term follow-up care for kids with pulmonary hypertension.

The Pulmonary Hypertension Association recognizes our program as an accredited comprehensive care center, one of only eight pediatric programs in the U.S. This designation recognizes our excellence in clinical care and adherence to evidence-based guidelines.

  • The Pulmonary Hypertension Association is a nonprofit organization that provides education, support and resources for patients and families affected by pulmonary hypertension.
  • The American Heart Association is a voluntary organization dedicated to fighting heart disease and stroke. They support research and provide educational resources on a variety of heart conditions.
  • The Pediatric Pulmonary Hypertension Network is a network of clinical specialists, researchers and centers bringing a collaborative and multidisciplinary approach to improving care for children with pulmonary vascular disease.

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