Pulmonary Hypertension (PPH & SPH)

Overview

What is pulmonary hypertension?

Pulmonary hypertension (PH) is high blood pressure in the blood vessels that line 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. Pulmonary blood pressure rises when the blood vessels in the lungs get narrow and stiff.

If the condition goes untreated, the heart cannot push hard enough against the lung pressures and not enough blood reaches the lungs, 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 are a common cause of hypertension in children.

Other causes include:

• Lung disease
• Sleep apnea
• Altitude effects
• Blood clotting disorders
• Autoimmune diseases
• Liver disease
• Familial disease

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 secondary pulmonary hypertension (SPH).

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

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Programs & Treatments

How is pulmonary hypertension treated?

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

If the hypertension is secondary (meaning it is the result of another condition), the best treatment plan is to repair the underlying heart condition when possible. Treatments for congenital heart defects range from careful monitoring by a doctor to surgery.

Pediatric cardiologists use a variety of medications to treat pulmonary hypertension. Select children’s hospitals participate in clinical trials to develop new medical treatments for kids with pulmonary hypertension.

Living with pulmonary hypertension

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

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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

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Diagnosis & Tests

How is pulmonary hypertension diagnosed?

If us 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

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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
• Pulmonary Hypertension Program at Children’s Hospital Colorado

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Reprinted with permission from Children's Hospital Colorado 2012 All rights reserved

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