Pulmonary Hypertension—Child

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.

Pressure may rise for any number of reasons. It creates a potentially serious condition. 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.

Heart and Lungs

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Causes

The blood vessels in the lungs are very 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 (eg, septal defects, leaky heart valves)
• Deformity of the chest wall (pectus excavatum)
• Conditions like scleroderma, sickle cell disease, lupus, HIV
• Chronic lung disease
• Blood clots in the lungs

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

Risk Factors

Risk factors include:

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

Symptoms

Symptoms 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 dizziness
• 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

The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include the following:

• Blood tests
• Blood gas determination —to check the concentration of gases in the blood
• Chest x-ray —a test that uses radiation to take a picture of structures inside the chest
• CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
• Cardiac catheterization — a test that uses a catheter and an x-ray machine to assess the heart and its blood supply
• Pulmonary function tests —a group of breathing tests to see how well the lungs are working
• Echocardiogram —a test that uses sound waves to look at the size, shape, and motion of the heart

Treatment

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

If the doctor cannot determine the cause, your child may be treated with medicine, such as:

• Medicines to lower blood pressure in the lungs
• Anticoagulants to prevent blood clots from forming
• Diuretics to help the body eliminate extra fluid
• Oxygen therapy to increase the oxygen getting into the blood stream
• Nitric oxide to relax the muscles in the arteries of the lungs

Your child may also need oxygen therapy. In severe cases, a lung transplant may be needed.

Prevention

To help reduce your child’s chance of developing pulmonary hypertension, take these steps:

• If your child has a condition that can lead to pulmonary hypertension, get proper treatment.
• In adults, smoking is a risk factor for pulmonary hypertension. Encourage your child to avoid smoking.