Pulmonary Hypertension

Overview

What is pulmonary hypertension?

Pulmonary hypertension (PH) 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 reasons a child could have pulmonary hypertension. Congenital heart defects and lung disease are common causes of hypertension in children.

Other causes include:

• Sleep apnea
• Altitude effects
• Blood clotting disorders
• Autoimmune diseases
• Liver disease
• Familial disease
• Persistent Pulmonary Hypertension of the Newborn (PPHN)
  

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.

Programs & Treatments

How is pulmonary hypertension treated?

The Pulmonary Hypertension Program at Children's Hospital's Colorado has been treating children with the condition for more than 25 years. 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. 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 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.

Signs & Symptoms

What are the signs and symptoms of pulmonary hypertension?

Children with pulmonary hypertension (PH) feel short of breath and tired, especially after activity. Symptoms of pulmonary hypertension 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
  

Diagnosis & Tests

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:

• Blood tests
• Chest x-ray
• EKG
• ECHO
• Exercise or stress test
• Diagnostic cardiac catheterization
• Lung CT scan
• Abdominal ultrasound
• Polysomnogram (a sleep study)
• Ventilation/perfusion scan
• Modified barium swallow study (aspiration study)
• pH/impedence study (reflux study)
  

Helpful resources:

To learn more about pulmonary hypertension, visit the following websites:

Pulmonary Hypertension Association
The American Heart Association 
The U.S. Library of Medicine