Pulmonary Hypertension Clinic

What is Pulmonary Hypertension?

Pulmonary hypertension is high blood pressure in the lung arteries. These arteries carry blood from the right side of the heart to the lungs. In a healthy system, blood pressure in the lungs is low. However, when the pulmonary arteries become narrowed or blocked, pressure builds up, and the heart has to work harder to pump blood to the lungs.

This can result in less oxygen in the blood as well as increased strain on the heart. Eventually the heart can become enlarged, less flexible and less able to pump blood to the rest of the body. Without proper treatment, pulmonary hypertension can lead to right heart failure, which can be fatal.

What Causes Pulmonary Hypertension?

A number of conditions can lead to pulmonary hypertension. Some babies are born with narrowed pulmonary arteries. In other children and adults, pulmonary hypertension develops over time as a result of other health problems, such as:

• Congenital (present at birth) heart defects
• Left-sided heart disease such as mitral valve disease
• Lung disease such as bronchopulmonary dysplasia
• Abnormal lung development such as congenital diaphragmatic hernia
• HIV
• Connective tissue diseases
• Autoimmune diseases such as lupus or scleroderma
• Sleep apnea and other breathing disorders
• Blood diseases such as sickle cell anemia or other clotting disorders
• Liver disease
• Certain drugs or toxins

In rare cases, pulmonary hypertension is an inherited condition. Sometimes the cause is unknown. This is called idiopathic pulmonary hypertension.

Pulmonary hypertension can’t be cured, but it can be treated. The earlier treatment starts, the better the outcomes will be.

What Are the Symptoms of Pulmonary Hypertension?

Symptoms of pulmonary hypertension are often similar those of other conditions. This makes it very important for children and adults to be seen by experts to get a correct diagnosis quickly.

Symptoms include:

• Shortness of breath
• Chest pain
• Dizziness
• Fainting
• Loss of energy
• Swelling of the arms, legs, ankles or abdomen
• Dry cough
• Enlarged liver
• Headache
• Blue or grayish skin from low levels of oxygen in the blood

How Is Pulmonary Hypertension Diagnosed?

Your doctor at Phoenix Children’s will ask about your child’s symptoms and do a complete physical examination. This can provide information such as abnormal heart sounds or other signs of pulmonary hypertension.

If your doctor suspects that your child may have this condition, you will be referred to a cardiologist. The specialists at Phoenix Children’s have seen many adults and children with pulmonary hypertension, and they are skilled at knowing the most effective tests to use to diagnose this condition.

In most cases, a number of tests are needed to make the diagnosis. Your child may have some of these tests:

• Pulse oximetry. This uses a small probe to check the amount of oxygen in the blood.
• Chest X-ray. This shows the heart and lungs and may reveal new or chronic changes.
• Electrocardiogram (ECG). An ECG records the electrical activity of the heart as well as abnormal rhythms. It can show stress to the heart muscle.
• Echocardiogram, or echo. An echo test uses sound waves to create a moving picture of the heart and its valves. It can show the direction of blood flow and the size of any defects. A number of types of echoes may be done.
• Pulmonary (lung) function testing. These tests check how well your child’s lung are working.
• Cardiac catheterization, or heart cath. This test provides very detailed information about the structures inside the heart. The doctor threads a small flexible tube called a catheter through a blood vessel in the groin and guides it to the heart. They can then measure blood pressure and oxygen in the heart. In some cases, contrast dye is used to show blood flow and structures inside the heart more clearly.
• Cardiovascular magnetic resonance imaging, or CMRI. This test uses a powerful magnetic field and radio waves to make detailed images of the heart and blood vessels.
• Computed tomography (CT) scans and CT angiography. These tests may be used to check for blood clots in the lung arteries and in the lung.
• Stress test. This uses an ECG test while the patient is exercising.
• Exercise tolerance test. This uses an ECG and other technologies to monitor a patient’s heart rate, blood pressure and breathing during exercise. It can show if the blood supply is reduced in the heart’s arteries
• Blood tests. A number of blood tests may be used to check the oxygen levels in the blood, to observe liver and kidney function, and to identify diseases such as thyroid problems, infection or collagen vascular disease. A blood test called brain natriuretic peptide helps doctors assess the strain on the heart. This test may also be used to monitor response to treatment.

How Is Pulmonary Hypertension Treated?

Each person with pulmonary hypertension is different, and each one requires personalized care. While this condition can’t be cured, there are many treatments that can improve your child’s quality of life. If an underlying cause is identified, some of the treatments may be aimed at improving symptoms and increasing the ability to participate in school, sports and other activities.

Treatments include oral, inhaled and intravenous (IV) medications as well as medications that are infused under the skin. These medications are used to relax the blood vessels in the lungs, to reduce clotting, to remove excess fluid from body, to reduce the heart’s workload and for other goals. Your doctor may also recommend a heart-healthy diet and limited exercise, depending on the severity of your child’s condition.

For some children and adults with pulmonary hypertension, surgery may help. At Phoenix Children’s, heart transplantation is also an option.

Living with Pulmonary Hypertension

If your child’s doctor suspects pulmonary hypertension and you would like your child to be seen by our team, or for a second opinion about a diagnosis, please contact our team.

A diagnosis of pulmonary hypertension can be discouraging. While this is a challenging condition, your team at Phoenix Children’s will do everything possible to help your child live well with pulmonary hypertension.

Our child life specialists can help you explain this condition to your child and help them deal with symptoms. And your treatment team is always here to answer questions and recommend the right treatments at the right time.

You can also find support from the Pulmonary Hypertension Association, which has helpful information about this condition, medical therapies, and specific support for kids, teens and parents.