Definition

Pressure helps blood flow through blood vessels. Hypertension occurs when this pressure gets too high. When this happens in the blood vessels in the lungs, it is called pulmonary hypertension.

As a result, the right side of the heart needs to pump harder to move blood against the pressure. If left untreated, the right side of the heart can eventually fail.

Causes

The blood vessels in the lungs are sensitive to oxygen. The lower the level of oxygen, the narrower the vessels become. Higher pressure will be needed to push blood through the narrow blood vessels. Pressure will also increase when there is more blood in the lungs than normal.

There are many different causes of pulmonary hypertension in children, such as:

• Birth defects that affect the heart, such as septal defects and leaky heart valves
• Deformity of the chest wall (pectus excavatum)
• Conditions like scleroderma, sickle cell disease, lupus, and HIV
• Chronic lung disease
• Blood clots in the lungs

Pulmonary hypertension can also be inherited. This is called familial pulmonary hypertension. In other cases, the cause is unknown.

Risk Factors

Factors that may increase your risk of pulmonary hypertension include:

• Being born with a heart defect or a deformity of the chest wall
• Having certain conditions, such as scleroderma, sickle cell disease, lupus, and HIV
• Having heart or lung disease
• Having a family member with pulmonary hypertension
• Living in a high altitude

Symptoms

Symptoms may include:

• Progressive shortness of breath
• Blue coloring of skin around mouth, hands, and feet
• Chest pain or pressure
• Fast heart rate
• Chronic cough
• Fatigue
• Fainting and lightheadedness
• Ankle or leg swelling from fluid retention

If your child has any of these symptoms, talk to the doctor right away. These symptoms may be caused by other conditions.

Diagnosis

You will be asked about your child’s symptoms and medical history. A physical exam will be done.

Your child’s bodily fluids may be tested. This can be done with blood tests.

Images may be taken of your child’s bodily structures. This can be done with:

• Chest x-ray
• CT scan
• Cardiac catheterization
• Echocardiogram

Your child’s lung function may be tested. This can be done with pulmonary function tests.

Treatment

Most cases of pulmonary hypertension are due to another condition. In this case, the underlying cause will be treated.

If the cause cannot be determined, your child may be treated with medication, such as:

• Medication to lower blood pressure in the lungs
• Anticoagulants to prevent blood clots from forming
• Diuretics to help the body eliminate extra fluid
• Nitric oxide to relax the muscles in the arteries of the lungs

Your child may also need oxygen therapy to increase the oxygen getting into the blood stream.

In severe cases, a lung transplant may be needed.

Prevention

To help reduce your child’s chance of developing pulmonary hypertension, get your child proper treatment for conditions that may lead to pulmonary hypertension.

Pulmonary hypertension. Cincinnati Children’s website. Available at:
http://www.cincinnatichildrens.org/health/info/heart/diagnose/ph.htm
Updated June 2014. Accessed November 7, 2014.