About Pediatric Pulmonary Hypertension

Pediatric Pulmonary Hypertension Clinic

About Pediatric Pulmonary Hypertension

Pulmonary hypertension is a rare disorder that causes high blood pressure in the lungs. Pulmonary blood pressure rises when the blood vessels in the lungs narrow or are blocked or destroyed, which makes it difficult for blood to flow through the lungs. 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 pressure, which can lead to heart failure.

What causes pulmonary hypertension in children?

Congenital heart defects and lung disease are common causes of pulmonary hypertension in children. Other causes include:

  • Sleep apnea
  • Altitude effects
  • Blood clotting disorders
  • Autoimmune diseases
  • Liver disease
  • Familial (genetic) disease
  • Persistent Pulmonary Hypertension of the Newborn (when a baby's body doesn't make that transition from fetal circulation to newborn circulation)
  • Idiopathic pulmonary arterial hypertension (IPAH)

No cause has been found for the disease.

What are the symptoms of pulmonary hypertension in children?

Children with pulmonary hypertension 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 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:

Treating of Pediatric Pulmonary Hypertension

If the hypertension is secondary (meaning it is the result of another condition), the best treatment plan is exploration and treatment of those conditions. This may include repair of the underlying congenital heart defects or treatment of lung disease, sleep apnea or severe reflux or aspiration.

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

  • Inhaled nitric oxide
  • Calcium channel blockers
  • Intravenous of inhaled epoprostenol (Flolan)
  • Intravenous or subcutaneous treprostinil (Remodulin)
  • Oral medications including Bosentan (Tracleer), Abrisentan (Letairis), Sildenafil (Revatio) and Tadalafil (Adcirca)

Living with Pediatric Pulmonary Hypertension

There is currently no cure for many forms of pulmonary hypertension but close follow-up with doctors that have experience in treating pulmonary hypertension can help your child live as normal a life as possible.

The Pulmonary Hypertension Association has excellent resources for patients and parents of children with pulmonary hypertension, including emotional support, school planning and tips, letters for school and medication information.

More resources:

Video Resources