Pulmonary Hypertension in Children

What Is Pediatric Pulmonary Hypertension (PPH)? 

Pediatric pulmonary hypertension is a rare disease that causes high blood pressure in the arteries of the lungs in children.

PPH means:

Pediatric: children
Pulmonary: lungs
Hypertension: high blood pressure

What Are the Signs and Symptoms of Pulmonary Hypertension?

Signs and symptoms are often similar to other childhood conditions that are less severe such as asthma. As a result, diagnosing PPH can take longer. Signs and symptoms may be more noticeable with activity, such as playing with siblings or friends. They may include:

• Tiredness
• Shortness of breath
• Fainting or near fainting
• Breathing harder or faster

Less common signs/symptoms include:

• Chest pain
• Pale or blue color of the lips, hands or feet
• Swelling in the legs or ankles

If your child is being treated for another more common condition and is not responding to that treatment, talk to your healthcare provider about testing for pulmonary hypertension.

What Causes PPH?

There are many different causes of PPH.

• Other lung diseases such as cystic fibrosis or interstitial lung disease
• Congenital (present at birth) heart defects
• Chronic blood clots in the blood vessels of the lung
• Rheumatologic disease
• Inherited from family (heritable)
• Blood disorders such as sickle cell disease
• Abnormalities of the chromosomes, such as Downs syndrome
• HIV
• Idiopathic (no known cause)- less frequent in children

Types of Pulmonary Hypertension

Ther are five different types of PH that are categorized into groups based on what is causing the PH

• Group I: Pulmonary arterial hypertension or PAH
• Group 2: Caused by left side heart disease
• Group 3: Caused by lung disease
• Group 4: Caused by blood clots in the blood vessels of the lungs
• Group 5: Caused by a combination of diseases

How Pulmonary Hypertension Is Diagnosed

Your child’s healthcare provider may order several tests to help confirm or rule out a diagnosis. When you are referred to a specialist, some of these tests may be repeated to make sure the results are the same and to determine the exact type of PH your child has.

• BNP blood test checks to see if the heart is working harder than it should have to
• Chest x-ray examines the structure of the heart and lungs
• Exercise testing to estimate how much a child is limited in function
• Pulmonary function tests to measure lung capacity 
• Electrocardiograms assess the heart rate and rhythm
• Echocardiography examines the structure and function of the heart
• Pulmonary perfusion scan to evaluate for chronic blood clots in the lung
• Cardiac catheterization measures pressure on the right side of the heart

How Pulmonary Hypertension is Treated

There are many treatment options available to help relieve PH symptoms and slow the progression of this condition. It is important to get a customized treatment plan started for your child as soon as possible. Knowing what type of PH your child has will help your healthcare provider know what treatment plan will best meet your child’s needs.

These medications can help manage PH. You can learn more about PH medications on this downloadable medication guide.

• Phosphodiesterase -5 (PDE5) inhibitors
• Endothelin receptor antagonists (ERA)
• Prostacyclin pathway agonists
• Calcium channel blockers (CCB)
• Diuretics
• Anticoagulant therapies
• Oxygen

PH is a rare disease, so it is vital to see a provider that specializes in this condition to ensure your child is getting the most up-to-date treatment. You can ask your primary healthcare provider to recommend a specialist. You can learn more about finding a specialist and look for a PH accredited center for pediatric care where providers have experience in meeting the complex needs of children living with PH.

How to Talk to Others About PPH

Sometimes it can be challenging to know how to talk to family and friends about your child’s chronic lung disease. It is up to you, and your child, regarding how much you are comfortable sharing. Here are some tips that may be helpful. You can:

• Provide basic information about the condition and how it impacts your child’s daily life
• Explain some of the challenges that the condition presents, such as any physical activity limitations and the importance of following a regular schedule for medications
• Provide age-appropriate explanations to siblings and friends of your child, using simple language and if appropriate visuals, for example showing what an inhaler looks like
• Let family and friends know the best way that they can provide support

Tips For Parents

Take care of yourself. It is important for you to prioritize your mental and physical well-being.

Connect with other parents of children living with PAH. Talking to other parents who have experienced similar challenges can help you feel less isolated.

Creating a routine can be helpful for both your child and family.

Empower your child by helping them learn self-management skills. Talk with your child’s specialist about setting specific goals that are right for them for their age.