Children's Hospital Colorado

Pulmonary Hypertension

What is pulmonary hypertension (PH)?

Pulmonary hypertension in kids is a rare disorder of high blood pressure in the blood vessels that line the lungs. Pulmonary blood pressure rises when arteries and capillaries in the lungs become narrowed, blocked or destroyed, making it hard for blood to flow through the lungs. Because the vessels of the lung and the heart are physically 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 cannot push hard enough against the lung pressures, which may eventually lead to heart failure.

What causes pulmonary hypertension in children?

There are several causes of pulmonary hypertension in kids. Congenital heart defects and lung disease are common causes of hypertension in children.

Other causes include:

In the above cases, the pulmonary hypertension is secondary because the rise in blood pressure was the result of another condition. This is known as associated pulmonary arterial hypertension (APAH), previously known as secondary pulmonary hypertension.

Other times, there is no underlying reason causing the blood pressure increase. This is called idiopathic pulmonary arterial hypertension (IPAH), previously known as primary pulmonary hypertension (PPH). Idiopathic pulmonary hypertension tends to affect girls more than boys. Children of any age can develop the condition.

What are some pediatric pulmonary hypertension symptoms?

Children with pulmonary hypertension (PH) feel short of breath and tired, especially after activity. Pulmonary hypertension signs can be confused with other conditions like asthma, sometimes leading to a delay in diagnosis and treatment.

Other pediatric pulmonary hypertension symptoms include:

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

How is pulmonary hypertension diagnosed?

If your doctor suspects that your child has pulmonary hypertension, he or she will likely order more tests to confirm the diagnosis. Common tests include:

About pediatric pulmonary hypertension treatment at Children's Hospital Colorado

The Pulmonary Hypertension Program at Children's Colorado has been treating children with the condition for more than 25 years. The Pulmonary Hypertension Association recently awarded our Program with a prominent accreditation for the ability to diagnose and care for medically complex patients. This accreditation makes our program one of only three pediatric programs in the U.S. to receive this distinction.

There are many treatment options for kids and young adults with pulmonary hypertension (sometimes called "PH" for short).

If the hypertension is secondary (meaning it is the result of another condition), the best treatment plan is exploration and treatment of contributing conditions. This may include repair of the underlying congenital heart defects, treatment of lung disease, as well as addressing less common secondary causes.

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

Although there is no cure for many types of pulmonary hypertension, treatments are available that can help lessen symptoms and improve your child's everyday life.

Pediatric pulmonary hypertension treatment options include:

  • Inhaled nitric oxide
  • Calcium channel blockers
  • Intravenous or inhaled epoprostenol (Flolan)
  • Intravenous and subcutaneous treprostinil (Remodulin)
  • Bosentan (Tracleer)
  • Ambrisentan (Letairis)
  • Sildenafil (Revatio)
  • Tadalafil (Adcirca)
  • Participation in clinical trial studies

Living with pediatric pulmonary hypertension

There is currently no cure for many forms of pulmonary hypertension, although close follow-up by a specialist with experience in treating PH can help your child live as normal a life as possible by monitoring your child's pulmonary pressures and response to medications.

Learn more about our Pulmonary Hypertension Program.

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