Definition

Pulmonary hypertension is an abnormal increase in the pressure of blood flow in the arteries of the lungs. 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 fail.

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Causes

This problem may be caused by:

• Birth defects that affect the heart, such as septal defects and leaky heart valves
• A 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 people, the cause is not known.

Risk Factors

Things that may raise the risk of this problem are:

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

Symptoms

Problems may be:

• Shortness of breath
• Blue coloring of skin around mouth, hands, and feet
• Chest pain or pressure
• Fast heartbeat
• Cough
• Lack of energy
• Fainting and lightheadedness
• Ankle or leg swelling

Diagnosis

The doctor will ask about your child’s symptoms and health history. A physical exam will be done.

Blood tests may be done.

Images may be taken of your child's body. 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

Underlying causes will be treated. In others, symptoms will be managed with medicines, such as:

• Medicine to lower blood pressure in the lungs
• Blood thinners
• Diuretics to remove excess fluids
• Nitric oxide to relax the muscles in the arteries of the lungs

Oxygen therapy may also be needed to raise the level of oxygen in the blood.

Children who are not helped by these methods may need a lung transplant.

Prevention

There are no known guidelines to prevent this health problem.