Pulmonary Hypertension Clinic

Pulmonary hypertension is high blood pressure in the arteries supplying the lungs. These arteries carry blood from the right side of the heart to the lungs. In normal physiology, the pressure in the vessels supplying the lungs is much lower than the vessels supplying the rest of the body. However, when the pulmonary arteries become narrowed, pressure builds up and the right side of the heart has to work harder to pump blood to the lungs.

This can result in increased work on the right side of 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 life threatening.

Symptoms of pulmonary hypertension are often similar to those of other common 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

Your team at Phoenix Children’s will ask about symptoms and do a comprehensive work up, including a complete physical examination, laboratory data, diagnostic testing and potentially genetic testing. This workup can help determine the root cause of a patient’s pulmonary hypertension.

If a patient is diagnosed with this condition, they will be referred to the pulmonary hypertension team. The specialists at Phoenix Children’s have seen many adults and children with pulmonary hypertension, and they are experts in providing a comprehensive workup and treatment plan.

In most cases, a number of tests may be required to understand the diagnosis and severity of the disease. Additional tests may include: 

• Pulse oximetry uses a small probe to check the amount of oxygen in the blood.
• Chest X-ray shows the heart and lungs and may reveal new or chronic changes.
• Electrocardiogram (ECG) records the electrical activity of the heart as well as abnormal rhythms. It can show stress to the heart muscle.
• Echocardiogram, or echo 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 checks how well the patient’s lungs are working.
• Cardiac catheterization is a test that 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, is a test that 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 tests may be used to check for blood clots in the lung arteries and in the lung.
• Stress test uses an ECG test while the patient is exercising.
• Exercise tolerance test 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.
• Genetic testing includes a comprehensive pulmonary arterial hypertension panel and can be very helpful in the management and treatment of pulmonary hypertension. 
• 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.

Each patient with pulmonary hypertension is different and warrants individualized and personalized care. Some but not all conditions may be cured or managed with the appropriate evaluation and treatment. Many patients with pulmonary hypertension enjoy a good quality of life aimed at improving symptoms and increasing the ability to participate in school, sports and other activities.

There are many medications to treat pulmonary hypertension. These medications can be delivered orally, inhaled, intravenous (IV) or subcutaneous (SQ). These medications are used to relax the blood vessels in the lungs, encourage increased blood flow to the lungs, reduce the risk for developing any blood clots and remove excess fluid from the body. This may reduce the strain on the right side of the heart. Additionally, each patient may receive recommendations on following a heart-healthy diet.